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Executive Chair's Statement
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Welcome to the 8th Annual Children's National Medical Center Research Day. Over the last 10 years we
have witnessed unprecedented growth in research at Children’s as a result of the hard work and creativity of
our faculty, staff, and students. This Research Day is a celebration of the success of our academic program
and serves to recognize all individuals that have made this possible. The CRI Board and leadership would
like to thank all the presenters for their hard work and dedication to find better ways to prevent, diagnose,
and treat childhood diseases. This year we are pleased to present 188 projects, the largest number ever
presented, and a sample of Children’s best academic accomplishments that includes basic, translational,
clinical, and community research as well as educational projects performed by faculty, fellows, trainees and
staff from our diverse programs.
The Children’s Research Institute is in an expansion mode with the recruitment of new research leaders
and building of new research space and facilities. This parallels the inauguration of the new hospital tower
and the increase in the quality and quantity of our clinical care programs, making Children’s one of the
premier pediatric hospitals in the United States.
We are looking forward to another productive year of innovative research and education which we hope to
share during the next Research Day. Thank you for your dedication to the mission of Children’s.
Sincerely,
Mendel Tuchman, MD
Executive Chair, 8th Annual
Children’s National Medical Center Research Day
Vice Chair, Research
Scientific Director, Children’s Research Institute
Mark L. Batshaw, MD
Chief Academic Officer
Children’s National Medical Center
Director, Children’s Research Institute
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Table of Contents
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Executive Chair’s Statement ……………………………………………..……..1
Schedule of Events ……………………………………………..………….……4
Project Descriptions …………………..………………………………………... 5
Basic/Translational Research …………………………………………... 5
Clinical Research………………………………………… ……………..29
Community-Based Research…………………………………………….60
Educaiton, Training, and Program Development Research……………..68
Indices
Projects by Keyword..…………………………………………………………..88
Projects by Program……………………………………………………….…...92
Projects by Department………………………………………………………...93
Projects by First Author…………………………………….………………….95
Acknowledgements
The leadership of the Children’s Research Institute would like to acknowledge the work and dedication of
the 8th Annual Children’s National Medical Center Research Day faculty, staff, and support departments for
without their planning and assistance this event would not be possible.
Event Faculty: Mark Batshaw, MD; Terrence Fry, MD; Cathie E. Guzzetta, RN, PhD; Gaetano R.
Lotrecchiano, PhD; Mary Rose, PhD; Randi Streisand, PhD; Stephen J. Teach, MD, MPH; Mendel
Tuchman, MD; Stanislav Vukmanovic, PhD; and Bernard Wiedermann, MD
Event Staff: Nikkie Adesida; Ruby Daniels; Gerry Gebbia; Dawn Griffiths; Jennifer Leischer; Kara Lord;
Melva McGlen; Marie Pichaske; Susan Pfennig, and Lisa Sheehy.
Support Departments: CNMC Volunteer Services, Environmental Services, Public Relations and
Marketing, Nutrition Services, Research Administration, Office of Academic Services and the Print Shop.
Research Day Companion Book: Arlene Gendron, Office of Academic Services
WebPortal Support: Saied Goudarzi, Biostatistics and Informatics Department
Special Recognition
On behalf of all the participants of the 8th Annual Children’s National Medical Center Research Day,
special recognition is extended to Annie Fulton, Office of Academic Services, for all of her hard work,
coordination, and leadership as the key coordinator of this event. Your dedication to this event has ensured
its integrity and continued success. We are sincerely grateful.
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Schedule of Events
CNMC MAIN ATRIUM
8:00am-9:00am
Mounting of Posters
9:00am-11:30am
Adjudication
Mary Rose, PhD, Stanislav Vukmanovic, PhD, Randi Streisand, PhD, Terrence Fry ,MD,
Stephen J. Teach, MD, MPH, Cathie E. Guzzetta, RN, PhD,
Bud Wiedermann, MD, Mendel Tuchman MD
11:30am-1:00pm
Attended Public Viewing
All Presenting authors will be stationed by their poster during this time.
1:00pm-1:15pm
Awards Ceremony
Mendel Tuchman, MD, CNMC 8th Annual Research Day Executive Chair
1:15pm-4:00pm
Unattended Public Viewing
CRI ATRIUM (Floor 5)
1:15pm-
Exhibitors’ Luncheon
Hosted by Mark L. Batshaw, MD. Chief Academic Officer, CNMC
All authors are invited to lunch.
CNMC MAIN ATRIUM
4:00pm-5:00pm
Breakdown of Poster Area
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TIMP-1-NUCLEATES A CRITICAL BRONCHIAL EPITHELIAL
RESPONSE NETWORK TO TOBACCO SMOKE IN ASTHMA
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RJ Freishtat AS Benton Z Wang MC Rose A Watson EP Hoffman
We used unsupervised clustering analysis to identify four publicly-available human and murine bronchial
epithelial (BE) mRNA expression profile datasets (n=136 microarrays) with high signal-to-noise ratios
relevant to asthma and/or tobacco smoke (TS). These microarrays were processed and normalized with
two probe set algorithms, dChip difference model and PLIER. Using a two-way clustering approach, we
identified 60 probe sets coordinately-regulated in asthma and tobacco-exposed BE. This gene cluster was
then overlaid on a phosgene model of direct BE oxidative stress (murine; 9 time points; n=104
microarrays), where 39 out of 60 probe sets showed significant (p=0.01) expression patterns, representing
a core set of BE genes involved in the response to asthma, TS, and oxidative stress. This gene group was
imported into Ingenuity Pathways Analysis software and corresponded highly to a gene/protein network
centered on tissue inhibitor of metalloproteinase (TIMP)-1. ELISA of cultured primary normal human BE
confirmed the increased intracellular BE TIMP-1 upon oxidative stress with H2O2 (p=0.017). By
comparison, asthmatic BE expressed less intracellular TIMP-1 upon oxidative stress (p=0.001) as well as
under TS extract-exposed (p=0.003) and control (p=0.003) conditions. As apical, basal, and intracellular
MMP-9 expression were unaffected, MMP-9:TIMP-1 ratios changed under several conditions. In
particular, the basally-secreted MMP-9:TIMP-1 ratio was significantly lower in TS extract-exposed
asthmatic BE (0.013+/-0.007 vs. 0.066+/-0.016; p=0.035) than untreated asthmatic BE, a milieu known to
favor subepithelial airway remodelling in chronic asthma. Therefore, we argue that this TIMP-1 network
is an oxidative stress pathway critical for the BE response to TS in asthma. Similar to steroid-resistant
asthma, where children show a blunted TIMP-1 response, certain TS-exposed children with asthma could
be more susceptible to metalloproteinase-mediated airway remodelling.
A MOUSE MODEL OF PRIMARY CILIARY DYSKINESIA REVEALS
HIGH FREQUENCIES OF HETEROTAXY AND COMPLEX CONGENITAL
HEART DEFECTS
L Leatherbury S Tan J Rosenthal X Zhao R Francis B Chatterjee P Connelly C Lo
Specification of left-right asymmetry is essential for formation of the four chamber heart and separate
systemic and pulmonary circulation. Previous studies suggest monocilia at the embryonic node is required
for left-right patterning. This patterning is perturbed in primary ciliary dyskinesia (PCD) where situs
defects and bronchiectasis are observed, often due to ciliary dysfunction arising from dynein mutations.
Most PCD patients exhibit situs solitus or situs inversus totalis, but heterotaxy with complex congenital
heart disease (CHD) appears to be rare, reported as 6%. We recovered a mouse mutation in dynein
Mdnah5 that disrupts ciliary function. Homozygous mutants exhibit situs phenotypes consistent with PCD
in humans. To assess the frequency of CHD associated with PCD, we harvested16 litters of embryos. All
wildtype and heterozygous offspring (89) showed normal body situs. Of the 21 (19%) homozygous
mutants obtained, 6 had situs solitus, 7 situs inversus and 8 heterotaxy, with heterotaxy being any situs
deviation in the cardiac, pulmonary or visceral anatomy. Of the heterotaxic embryos, 3 had levo and 5
dextrocardia. Histology and 3D reconstruction showed 7 of the heterotaxy embryos had complex CHD,
which included atrial isomerism, superior-inferior ventricles (Figure), malposition of the great arteries,
AV cushion defects, and azygous continuation of the inferior vena cava. These results show a much
higher frequency of heterotaxy and complex CHD than previously reported for PCD (38% vs. 6%),
suggesting PCD patients should be screened for CHD. The high incidence of CHD associated with PCD
indicates ciliary function may have other roles in cardiovascular patterning.
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CRYSTAL STRUCTURES OF N-ACETYLGLUTAMATE SYNTHASE
PROVIDE INSIGHTS INTO CATALYTIC AND REGULATORY
MECHANISMS
D Shi V Sagar Z Jin X Yu L Caldovic H Morizono N Allewell M Tuchman
N-acetylglutamate synthase (NAGS) catalyzes the first committed step of the arginine biosynthetic
pathway. In mammals, NAGS produces N-acetyl-L-glutamate (NAG), an obligatory allosteric activator of
carbamyl phosphate synthase I (CPSI) in the urea cycle. NAGS deficiency in human leads to
hyperammonemia owing to the decreased activity of CPSI deprived of its cofactor NAG. We report the
crystal structures of NAGS from Neisseria gonorrhoeae in the inactive T-state bound with its allosteric
inhibitor L-arginine and in the active R-state complexed with acetyl-co-enzyme A (AcCoA), or CoA and
NAG. These structures reveal that NAGS consists of two separately folded domains, an amino acid kinase
domain (AAK) and an N-acetyltransferase (NAT) domain. The kinase domain has a fold similar to other
members of the AAK family while the NAT domain has a fold similar to GCN5-related NAT proteins.
The monomers form a hexameric ring that consists of a trimer of dimers with inner and outer ring
diameters of ~20 Г… and ~100 Г…, respectively, and a height of 110 Г…. The catalytic sites are located within
the NAT domains and the mode of binding of AcCoA and CoA is similar to other NAT family proteins.
NAG interacts with two arginines (Arg316 and Arg425), to anchor the substrate in its proper position.
Comparison of active R- and inactive T- state structures indicates that binding of arginine to the AAK
domain induces a large global conformational changes in the enzyme. The hexameric structure becomes
~20 Г… shorter and ~10 Г… larger in diameter. Specifically, the NAT domain rotates ~109Вє to rearrange the
interaction between AAK and NAT domains. This rearrangement disrupts the interactions of the adjacent
AAK domain with AcCoA hampering its binding and thus inhibiting catalytic activity. These findings
provide new insights into the catalytic and inhibition mechanism of NAGS.
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GLYCOSYLATION DEFECTS IN MUSCULAR DYSTROPHY
SE Sparks A Kesari EP Hoffman
Abnormal glycosylation of alpha-dystroglycan underlies the pathology of a group of muscular
dystrophies known as the dystroglycanopathies. The clinical phenotype ranges from congenital onset of
muscular dystrophy with CNS and eye involvement (CMD), to a later onset form of limb girdle muscular
dystrophy (LGMD), without any CNS or eye involvement. To date, six genes have been identified which
alter the glycosylation pattern of alpha-dystroglycan, all of which are known or putative
glycosyltransferases. However, with the anticipated 10-15 steps in the glycosylation of alphadystroglycan, there are more to be identified. The muscle biopsy database of Dr. Eric Hoffman contains
over 5000 samples that were referred for diagnostic testing of muscular dystrophy. This database was
utilized to screen for patients that potentially have a defect a dystroglycanopathy. Once the initial screen
was completed, samples that clinically fit for CMD were subjected to immunohistochemical
determination of glycosylation of alpha-dystroglycan. In those with a defect, molecular and biochemical
characterization has been pursued. In contrast, in samples that fulfilled a LGMD clinical phenotype, a
molecular screen for FKRP mutations was performed. A two tiered approach by first screening for the
common c.826C>A (p.L276I) mutation, followed by full sequencing of the FKRP gene in those
demonstrating a mutation was utilized. In those samples with two FKRP mutations, glycosylation status
of alpha-dystroglycan has been analyzed. The results of this study show that different screening
approaches are necessary to identify dystroglycanopathies in patients with unknown muscular dystrophy
depending on the presenting clinical symptoms.
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P38 MAP KINASE REGULATES OLIGODENDROCYTE DEVELOPMENT
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LJ Chew W Coley V Gallo
Activation of the p38 mitogen activated protein kinase (p38MAPK) signal transduction pathway is known
to mediate inflammation and injury in many cell types, and p38MAPK is being targeted in antiinflammatory therapies, but its role(s) in CNS white matter formation is not well characterized. In this
study we examined how p38MAPK participates in the development of CNS myelin-forming
oligodendrocytes from oligodendrocyte progenitor cells (OPCs), and identified molecular mediators of
p38MAPK activity in oligodendrocyte maturation. Adult mouse brain expressed high levels of p38MAPK
in the myelin-containing structures of the subcortical white matter, corpus callosum and caudate putamen.
White matter CC1+ or CNP+ mature oligodendrocytes also expressed high levels of p38alpha MAPK,
dual-phosphorylated p38MAPK (P-p38) and its phosphorylated substrate, P-ATF2. In adult corpus
callosum tissue, high P-p38 levels were coincident with decreased phosphorylated extracellular signalregulated kinase (P-ERK) expression in CC1+ cells. In cultured OPCs, p38MAPK phosphorylation is
elevated by differentiation paradigms, and the inhibition of p38MAPK activity reduced myelin gene RNA
levels and the percentages of differentiated O1/GalC+ cells without detectable change in cell viability or
proliferation. In reporter assays, dominant negative p38alphaMAPK reduced, while its upstream kinase
MKK6 stimulated MBP promoter activity. MKK6 also enhanced activity of a reporter construct bearing
SOX-transcription factor binding sites. P38MAPK inhibition lowered Sox10 RNA levels, and reduced
Sox10 binding of the MBP promoter in electrophoretic mobility shift assays. The decline in ERK
phosphorylation associated with OPC differentiation was prevented by p38 inhibition, and co-incubation
with a MEK/ERK inhibitor partially restored myelin gene expression and OPC lineage progression,
without significantly restoring Sox10 RNA. Our findings indicate that p38MAPK expression in the brain
is associated with myelin synthesis during white matter development, and that its activity promotes OPC
lineage progression both through modulating Sox10 expression and through cross-talk with the ERK
cascade.
CIGARETTE SMOKE ACTIVATES NFОєB- MEDIATED TNF A RELEASE
FROM MOUSE MIDDLE EAR CELLS
DA Preciado E Kuo S Ashktorab M Rose
Objectives: Cigarette smoke exposure is a significant risk factor in the development of otitis media (OM).
NF-ОєB is a ubiquitous transcription factor known to mediate cigarette smoke effects on gene regulation in
multiple cell types. Sustained inflammation in the middle ear is critical in the progression of acute to
chronic OM. We hypothesized that in vitro stimulation of a, murine middle ear epithelial cells (MEEC),
with cigarette smoke condensate (CSC) activates NF-ОєB and induces pro-inflammatory cytokine release.
Methods: Time course CSC stimulation of immortalized murine MEEC was performed. Antibody array
technology was utilized to simultaneously measure 40 different inflammatory cytokines after CSC
exposure. Enzyme linked immunosorbent assays (ELISA) and quantitative real time reverse transcriptase
polymerase chain reaction RT-PCR were performed to further evaluate the cytokines that changed the
most on the array results. Luciferase reporter assays, and electromobility shift assays (EMSA) and were
performed to evaluate NF-ОєB activation with CSC in the cells. Chromatin immunoprecipitation (ChIP)
assays and luciferase reporter assays were performed to determine whether CSC activates the TNFa
promoter through NF-ОєB interaction with canonical ОєB sites. Results: Of the 40 pro-inflammatory
cytokines sampled there was a significant increase in TNFa, IL6, IL13, KC, and IFNОµ with CSC
exposure. ELISA studies demonstrated that TNFa secretion increased the most after CSC stimulation.
Reporter assays and EMSA demonstrated 3-5 fold dose dependent activation of NF-ОєB with CSC. CSC
stimulation likewise increased TNFa mRNA abundance and induced reporter activity 1.8-4.8 fold in
luciferase plasmids. ChIP assays demonstrated NF-ОєB binding to canonical ОєB sites in the TNFa
promoter with CSC stimulation. Conclusions: CSC activates NF-ОєB in immortalized MEEC.
Furthermore, this activation results in CSC induced TNFa promoter activation, gene expression, and cell
secretion.
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DETECTION OF HUMAN HERPESVIRUS 6 (HHV6) IN CNS TUMORS:
PREDOMINANCE OF ACTIVE VIRAL REPLICATION IN GLIAL TUMORS
J Crawford MR Santi R Cornelison SL Salinnen H Haapasalo S Jacobson T MacDonald
Human Herpesvirus 6 (HHV6) infection has been associated with central nervous system (CNS) diseases
of encephalitis, multiple sclerosis, and epilepsy. HHV6 has been detected by polymerase chain reaction
(PCR) in 6-37% of adult CNS tumors. However, the percentage of active HHV6, as determined by viral
protein expression, is not known. To address this issue, we screened a series of CNS tumor tissue
microarrays containing a variety of adult CNS tumors for the presence of HHV6 nucleic acid and protein.
One hundred six of 224 (47%) CNS tumors were positive for HHV6 by in situ hybridization (ISH) using
probes specific for Major Capsid Protein with no difference in frequency between glial and non glial
tumor subtypes. (p=0.069). Nested PCR using HHV6 Major Capsid Protein primers was performed on a
subset of ISH positive and negative paraffin embedded tumors as validation. Fourteen of 29 (48%) tumors
were HHV6 positive by nested PCR. HHV6 sequence analysis revealed HHV6 Variant A in 6 of 14
samples. Using two HHV6A/B non-specific monoclonal antibodies gp116/54/64 and p41, active viral
replication was observed by immunohistochemistry (IHC) in 84 of 282 (35%) and 66 of 277 (24%) of
tumors respectively. Interestingly, glial tumors showed a three fold higher IHC positivity compared to
non glial tumors for both HHV6 gp116/54/64 (p=0.0001) and HHV6p41 (p=0.007) antibodies. Kaplan
Meier analysis revealed no difference in survival between ISH (p=0.9711) or IHC positivity using either
HHV6 gp116/54/64 (p=0.8522) or HHV6 p41 (p= 0.8170) antibody. We conclude that active HHV6 is
detected in adult CNS tumors at a higher frequency in tumors of glial origin. While the presence of HHV6
was not associated with a significant difference in overall survival in our series, we hypothesize that the
glial-tropic features of HHV6 may play an important modifying role in tumor biology that warrants
further investigation.
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URINE SAMPLES FROM HIV- INFECTED CHILDRENS WITH RENAL
DISEASE INCREASE THE PERMEABILITY OF CULTURED HUMAN
RENAL GLOMERULAR ENDOTHELIAL CELLS
M Jerebtsova AA Soler-Garcia PE Ray
HIV-infected children are at high risk of developing several types of HIV-1 associated renal diseases
(HIVARD). These diseases are characterized by the presence of proteinuria and rapid progression to
chronic renal failure. We have found high levels of angiogenic factors and inflammatory cytokines in the
urine of children with HIVARD. Here, we hypothesize that the angiogenic-inflammatory cytokine milieu
in children with HIVARD, changes the permeability and/or structure of glomerular endothelial cells
(HGEC) leading to alterations in the glomerular filtration barrier. Urine samples were collected from
HIV-infected children with chronic renal disease and normal controls. HGEC immortalized with the SV40 large T antigen were cultured on trans-well permeable filters. Permeability of the cells monolayer was
characterized by trans-endothelial electrical resistance (TEER) and permeability of FITC-dextran. Light
microscopy and fluorescent staining for F-actin was used to evaluate the changes in cell shape. In contrast
to the control samples, HIVARD samples induced a significant decrease in TEER and increased the
permeability to FITC-dextran in culture HGEC without causing cell detachment or other cytotoxic effects.
These changes were associated with the rearrangement of stress fibers and increase size of intracellular
gaps in HGEC exposed to samples from HIVARD. Thus, angiogenic-inflammatory factors released into
the circulation of HIV-infected children may contribute to the pathogenesis of HIVARD and development
of glomerular permeability changes. The measurement of TEER and FITC- dextran permeability in
HGEC is a rapid and non-invasive biological assay for the identification of potential circulating
endothelial permeability factors in the urine of HIV-infected children. This assay might be also useful to
identify children with endothelial dysfunction secondary to the metabolic syndrome and/or HAART
toxicity.
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P188 IMPROVES SKELETAL MUSCLE STRENGTH AND PREVENTS
TACHYCARDIA INDUCED CARDIOMYOPATHY IN DYSTROPHIN
DEFICIENT MICE
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CF Spurney DA Guerron J van der Meulen K Nagaraju
Duchenne muscular dystrophy (DMD) is caused by the absence of dystrophin from muscle cell
membranes. This leads to loss of membrane integrity, cell death and fibrosis over time. As treatment of
the skeletal and respiratory complications of DMD improves, morbidity and mortality from cardiac
related diseases are increasing. In order to study the effects of membrane integrity on skeletal and cardiac
muscle function, we treated 12-15 week old dystrophin deficient female mdx mice with the membrane
sealant Poloxamer 188 (P188), a non-ionic polymer that inserts and seals damaged cell membranes. Also,
in order to increase cardiac stress, we exposed the mice to 0.5 mg/kg/day of isoproterenol via osmotic
pump delivery for two weeks (n=20). Treated mdx mice received 460 mg/kg/dose of P188 given i.p daily
(n=10). Non-invasive high frequency echocardiography was performed at baseline and two weeks.
Skeletal muscle (extensor digitorum longus) from treated mdx mice showed significantly increased
muscle force in an isolated whole muscle preparation (p=0.04). Significantly decreased serum creatine
kinase levels were found in treated mice (p=0.006). Echocardiography found significantly decreased
cardiac function in untreated mdx mice. The shortening and ejection fractions were both decreased by
approximately 11% (p=0.02; p=0.04). Heart rate was significantly decreased in untreated mdx mice
(p=0.002). Histological analysis showed a trend towards significantly increased collagen content in left
ventricular tissue stained with Sirius red. Intraperitoneal P188 treatment improved skeletal muscle
strength and prevented tachycardia induced cardiomyopathy in mdx mice.
DETERMINANTS OF HUMERAL BONE VOLUME IN MALES: A
GENOME-WIDE SCAN
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LL Tosi F Suer B Harmon C Brandoli H Gordish E Hoffman J Devaney
We sought to identify genetic markers for bone size in men, a polygenic trait with critical implications for
bone strength and bone quality. We believe that the humerus may be a particularly sensitive bone for
detecting genetic predispositions in bone health. It is a single bone and thus not subject to load sharing. It
is not weight-bearing and thus less affected by confounding factors such as fluctuations in weight or
activity. Total bone volume in the distal 9.6 cm of the humeral diaphysis of the non-dominant arm was
calculated from MRI’s using semi-automated software from Rapidia in 303 young adult Caucasian males
(23.68+ 5.47 years) participants from the FMS study. We performed genome-wide screening for the 60
males with the highest and lowest bone volumes in the cohort, using the AffymetrixВ® Genome-Wide SNP
Array 6.0. Results were analyzed using the bioinformatics software package Partek GS. We identified 138
SNPs associated with bone size in males. Among the most significant SNP’s, 121 of 138 were in five
linkage disequilibrium (LD) blocks and 129 fell on the X chromosome. All together we identified 8
regions of interest with 4 SNP’s falling in the UTR regions of genes, and 1 SNP falling in the coding
region of a gene. To date, many whole genome scans which have attempted to study bone density (a
combination of bone size and mineral properties) have shown inconsistent results. We believe that by
focusing on bone size, we may be able to identify genetic markers that are consistent in multiple
populations. Our future plans include 1) validate the results of the whole-genome analysis by genotyping
the most statistically significant SNPs in the entire FMS population, 2) fine-map and deep sequence the
identified regions and 3) develop hypotheses as to how these genes influence bone quality.
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HEAT SHOCK PROTEINS AND G PROTEIN-COUPLED RECEPTOR
SIGNALING AS KEY DETERMINANTS OF VIRAL MYOCARDITIS
RL DeBiasi S Maikarfi A Patel RL DeBiasi
Viral myocarditis is a severe disease, resulting in heart transplantation or death in 20-50% of patients.
Exact mechanisms by which viral infection results in cardiac injury are poorly understood and treatments
are ineffective. Reovirus infection is a well-characterized mouse model of viral myocarditis for both in
vitro and in vivo studies of pathogenesis. We performed microarray analysis of cardiac myocytes infected
with a panel of myocarditic and nonmyocarditic viruses at an early time point post-infection (preceding
apoptosis). Genes with significant differential expression (comparing myocarditic to nonmyocarditic
infection) were selected as potential key determinants of the myocarditic phenotype. Pathway analysis
revealed that transcripts encoding Heat Shock proteins (HSP) and members of G-protein coupled receptor
(GPCR) signaling pathways were over-represented. Specifically, we noted upregulation of several HSP
proteins in myocarditic virus-infected myocytes (including Hsp110 and Hsp1-like). Additionally, we
noted global downregulation of a variety of GPCR’s, (including P2yr4) and upregulation of at least one
inhibitory regulator of GPCR signaling (RGS16). We confirmed differential expression of Hsp110, Hsp1like, P2yr4 and RGS16 in cardiac myocytes infected with myocarditic viruses (compared to
nonmyocarditic virus-infected and uninfected cells) by semi-quantitative RT-PCR. We subsequently
characterized the differential expression of proteins encoded by these transcripts within cardiac tissues (in
vivo) from myocarditic virus-infected mice (compared to nonmyocarditic virus- and mock-infected mice)
by immunohistochemistry. These studies suggest that HSP and GPCR-related proteins may play
biologically relevant roles in the pathogenesis of viral myocarditis. We are further characterizing the
temporal kinetics and pattern of expression of these proteins in relation to viral binding, myocyte
apoptosis and histological injury, to guide the ultimate goal of targeted therapeutic interventions in these
pathways.
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VENTRICULAR TWIST IN INFANT MICE WITH SITUS INVERSUS: AN
ANALYSIS BY HIGH FREQUENCY ULTRASOUND IMAGING
LF Frank RF Francis QY Yu RS Samtani XL Li DS Sahn LL Leatherbury CL Lo
The Torrent-Guasp hypothesis of a continuous myocardial band has implications for ventricular
mechanics in abnormally looped hearts. Few studies exist describing the components of myocardial
motion in models of congenital heart disease. To evaluate ventricular mechanics in mice with reversal of
cardiac situs associated with situs inversus totalis (SI), we conducted high frequency ultrasound
interrogations of a mouse model of Kartagener syndrome with a recessive mutation in dynein Dnahc5.
The goals of the study were to demonstrate the feasability of high frequency echocardiography in
neonatal mice, to describe the direction of left ventricular twist in normal and mutant mice, and to
perform a quantitative analysis of this twist using speckle tracking echocardiography.
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DYSFERLIN-DEFICIENCY SHOWS COMPENSATORY INDUCTION OF
RAB27A/SLP2A THAT MAY CONTRIBUTE TO INFLAMMATORY
ONSET
A Kesari M Fukuda S Knoblach R Bashir G Nader K Nagaraju E Hoffman
Mutations in the dysferlin gene cause Limb girdle muscular dystrophy 2B (LGMD2B) and Miyoshi
Myopathy (MM). Dysferlin-deficient cells show abnormalities in vesicle traffic and membrane repair,
although onset of symptoms is not until late teens, often associated with sub-acute onset and marked
muscle inflammation. To identify molecular networks specific to dysferlin-deficient muscle that might
explain disease pathogenesis, muscle mRNA profiles from 10 mutation-positive LGMD2B/MM patients
were compared to a disease control (LGMD2I [FKRP]; n=9), and normal muscle samples (n=11). Query
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of inflammatory pathways suggested LGMD2B-specific increase in co-stimulatory signaling between
dendritic cells and T cells (CD86, CD28, CTLA4), associated with localized expression of versican and
tenascin. LGMD2B muscle also showed an increase in vesicle trafficking pathway proteins not normally
observed in muscle (Synaptotagmin-like protein Slp2a/SYTL2 and small GTPase Rab27A). We propose
that Rab27A/Slp2a expression in LGMD2B muscle provides a compensatory vesicle trafficking pathway,
able to repair membrane damage in the absence of dysferlin. However, this same compensatory pathway
may release endocytotic vesicle contents, resulting in an inflammatory microenvironment. Dysferlindeficiency enhances phagocytosis by macrophages,1 and this coupled with our findings of abnormal
myofiber endocytosis pathways and dendritic-T cell activation markers suggests a model of overstimulation of T cell – dendritic cell networks that may explain the sub-acute inflammatory presentation.
MECHANISMS OF ALTERED FOREBRAIN DEVELOPMENT IN THE
TS65DN MOUSE MODEL OF DOWN SYNDROME
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L Chakrabarti TF Haydar
Objective: Ts65Dn, the most widely accepted murine model of Down syndrome (DS), exhibits multiple
DS-specific postnatal symptoms including behavioral abnormalities. Abnormalities in the development of
hippocampus, cerebellum and neocortex, the structures involved in motor, sensory and cognitive
functions are found in Ts65Dn and DS. However, in contrast to hippocampus and cerebellum, the
maturation of cerebral cortex has not been studied in Ts65Dn. Methods: We studied the postnatal
development of Ts65Dn neocortex at the level of cell density, cortical layer specification, neuronal
differentiation and synaptogenesis. Results: We found that defective embryonic forebrain expansion
results in reduced cell density, impaired synaptic development, defect in deep layer specification and an
imbalance in the number of excitatory and inhibitory neurons in the postnatal Ts65Dn cortex.
Conclusions: These results demonstrate that embryonic defects in Ts65Dn neuronal precursor cell
properties lead to lasting defects in cortical maturation, specifically impacting layer specification and
differentiation.
THE ROLE OF RAC SIGNALING IN PDGF-MEDIATED CELL MOTILITY
IN MEDULLOBLASTOMA
LP Yuan TM MacDonald
The platelet-derived growth factor receptor (PDGFR) is overexpressed in metastatic medulloblastoma and
promotes medulloblastoma cell migration, but the mechanism is unknown. We thus investigated the role
of the PDGFR signaling effectors Rac/Rho in medulloblastoma. We show that Rac1 expression is
detectable by immunohistochemistry in over 90% of medulloblastomas analyzed (n = 144) and that high
expression levels of Rac1 correlate with metastasis and poor clinical outcome. Treatment of Daoy
medulloblastoma cells with PDGF-BB increases Ras activity, MEK/ERK phosphorylation and induces
the rapid formation of dorsal ruffles while decreasing actin stress fibers, as characterized by migratory
cells. PDGF treatment concomitantly increases Rac1-GTP and decreases Rho-GTP to shift the balance to
Rac1 activation, especially in the presence of the Rho/Rock specific inhibitor, Y27632, while the Rac1
specific inhibitor, NSC23766, completely abolished PDGF-mediated cell migration. Finally, we
demonstrate that MEK/ERK regulates PDGF-mediated medulloblastoma cell migration by maintaining
suppressed Rho activity. Together, these results confirm that the Rac/Rho balance is critical to PDGFmediated medulloblastoma cell migration and that Rac1 inhibition may be a novel strategy to prevent
medulloblastoma metastasis.
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IDENTIFYING GENES AND PATHWAYS THAT LEAD TO GOBLET
CELL METAPLASIA IN AN IL13 INDUCED MURINE ALLERGIC
ASTHMA
X Wu C Kovach AM Watson AM Colberg-Poley MC Rose
A major characteristic of asthma is an increased number of goblet cells that lead to mucus
overproduction. The pathways that lead to the increase of goblet cells in airway epithelium are not yet
understood. In this study, we are determining the master genes that are upregulated by IL13 activated
Stat6, which in turn regulate expression of switch genes. We investigated the Stat6 activated master genes
by temporal expression microarray analyses of murine trachea following in vivo exposure to IL13 or PBS.
We identified four candidate master genes (Tbx1, Foxq1, Etv1 and Ehf) which were upregulated at 1h,
decreased to baseline by 3.5 or 6 h following IL13 exposure, and contain Stat6 cis-elements in their
promoter. To further examine these genes, we established an in vitro system of primary differentiated
murine tracheal epithelial (MTE) cells in which robust goblet cell metaplasia (43% of MTE cells
differentiated into goblet cells) was observed at 48h following IL13 exposure. Immunofluorescence
analysis of MTE cells following exposed to IL13 for 0.5, 1, 2, 2.5, 3.5 and 6 h demonstrated that Stat6
translocated to the nucleus by 0.5 h and was still in the nucleus at 1h following IL13 exposure. The
previously identified master genes and one candidate switch gene (Foxa2) were further evaluated by qRTPCR in MTE cells. Results showed Etv1 and Ehf exhibited different temporal expression patterns in vitro
and in vivo. Tbx1, Foxq1, and Foxa2 expressed similar patterns in vivo and in vitro. Tbx1 and Foxq1
were increased at 1h and decreased to baseline at 3.5 and 6 h. Foxa2 was markedly decreased at 2 h, and
remained decreased at 3.5 and 6 h. These results suggest that Foxq1 and Tbx1 may serve as master genes
and Foxa2 as a switch gene during the differentiation of goblet cells in the murine airway epithelium.
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TYPE I AND TYPE II MUSCLE FIBERS ARE DIFFERENTIALLY
AFFECTED IN THE MAJOR HISTOCAMPATIBILTY CLASS I (MHC
CLASS I) MOUSE MODEL OF MYOSITIS
GS Pandey J VanderMeulen R Rawat E Pistilli N Raben R Sabina P Plotz K Nagaraju
Myositis patients shows unusually high expression of the MHC class I gene in skeletal muscles. The
MHC class I mouse model recapitulate some of the features of myositis including intracellular
accumulation of MHC class I in endoplasmic reticulum (ER) leading to ER Stress. The MHC class I
expression is more restricted to Type II (Gastrocnemius) than Type I (Soleus) muscle fibers. It is
proposed that increased level of MHC class I expression coupled with increased ER stress may lead to
increased level of damage in type II muscles fibers. This process is likely to facilitate fiber type
conversion, i.e. more type II fibers converting to type I fibers. We performed AMP deaminase
(AMPD)assay, immunohistochemical staining, muscle force measurements and gene expression profiling.
Present study demonstrates that there are significant differences in the muscle mass of type I muscle
fibers between control and class I mice. Control mice showed significantly higher levels of AMPD1
activity in type II fibers compared to type I fibers. In contrast, AMPD1 activity was significantly less in
Type II fibers of MHC class 1 mice compared to control mice. There was no statistically significant
difference in AMPD-1 activity between type II and type I fibers in class 1 mice. Slow myosin heavy chain
staining in type II fibers of MHC class I mice showed higher level of expression compared to the control
mice. Gene expression profiling of type I and type II fibers from MHC class I mice showed significant
differences in the expression patterns in general. We show that there is fiber type conversion in MHC
class 1 mice and the biochemical, molecular and immunohistochemical changes confirms these finding.
Experiments are currently in progress to understand the mechanism of fiber type conversion in these
mice.
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BETA 1 INTEGRIN SIGNALING IS NECESSARY FOR ANCHORAGE OF
NEURAL PROGENITORS AT THE MURINE VENTRICULAR SURFACE
18
K Loulier JD Lathia MS Rao MP Mattson C Ffrench-Constant TF Haydar
During neocortical development, how neural progenitors control the spatio-temporal patterning of
different subclasses of neurons is still not completely known. The recent implication of laminin/integrin
signaling in the regulation of neural stem cell proliferation in the murine ventricular zone (VZ) lead us to
further investigate the action of these extracellular matrix molecules (laminins) and their receptors
(integrins) on the division, cellular morphology, and fate of VZ progenitors during mouse cortical
development. To follow a population of VZ progenitors after perturbation of integrin signaling, we
combined in utero intraventricular injection of a beta 1 integrin (b1) blocking antibody with simultaneous
electroporation of DNA coding for fluorescent proteins. Compared to controls, we found that a small
population of these electroporated VZ progenitors exhibited aberrant morphology and was located far
from the VZ surface 18 hours after the co-electroporation/b1 blocking antibody injection. The presence of
these “detached” cells after b1 blockade was correlated with a decrease in the number of apical processes
anchored to the VZ surface. These data demonstrate a novel role for the laminin/integrin interaction in the
neocortical VZ: beta 1 integrin signaling is required for the anchorage of a subpopulation of dividing
neural stem cells during embryonic development.
DIFFERENTIAL REGULATION OF TELENCEPHALIC PALLIALSUBPALLIAL BOUNDARY PATTERNING BY PAX6 AND GSH2
19
RS Carney LA Cocas T Hirata K Mansfield J Corbin
In the embryonic telencephalon, the pallial-subpallial boundary (PSB) separates the subpallium from the
dorsal pallium. The PSB is comprised of progenitors derived from the Pax6+ ventral pallium (VP) and
Gsh2+ dorsal lateral ganglionic eminence (dLGE). Pax6 and Gsh2 are crucial regulators of VP and dLGE
identity. However, since Pax6 (Sey) mutants exhibit ectopic pallial Gsh2 expression and Gsh2 mutants
display ectopic subpallial Pax6 expression, a direct genetic requirement for each of these genes for PSB
formation could not be differentiated from the consequence of ectopic expression of Gsh2 and Pax6. To
directly test this, we compared the expression of a series of VP and dLGE markers in Sey;Gsh2 double
mutant mice to single Sey and Gsh2 mutants. We demonstrate that both Pax6 and Gsh2 are directly
required for major aspects of PSB progenitor specification, as well as revealing novel roles for these
genes in proper dLGE and VP patterning. Third, although EGFR+ PSB progenitors express pallial and
subpallial markers, the establishment of EGFR+ lateral cortical stream cells is Pax6-dependent. Thus, in
addition to their well-characterized cross-repressive roles in dorsal-ventral patterning our analyses reveal
novel important functions of Gsh2 and Pax6 in the regulation of PSB progenitor pool specification and
patterning.
EMBRYONIC ORIGINS OF EXCITATORY AND INHIBITORY CELLS IN
THE AMYGDALA
T Hirata JG Corbin
The mature amygdala, a major structure of the limbic system, plays an essential role in the processing of
input with emotional salience. Although the anatomy, neural connections and function of the amygdala
are relatively well understood, amygdala development is poorly understood. Recent studies have shown
that the lateral cortical stream (LCS), which arises from the cortical-striatal border (CSB) of the
telencephalon, is one of the sources of progenitor cells of the amygdala. To examine the contribution and
the cell fates of the LCS in the amygdala, we knocked in CreERT2 into the Dbx1 locus. Our analysis
reveals that CSB Dbx1+ progenitors give rise to excitatory neurons in the basolateral complex and
cortical amygdala nuclei. In addition, we find that diencephalic Dbx1+ progenitor cells also appear to
migrate to the telencephalon where they will generate subtypes of medial amygdala nuclei inhibitory
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neurons. Thus, both telencephalic and diencephalic Dbx1+ progenitors contribute to excitatory and
inhibitory cell diversity in the mature amgydala
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TUMOR CELL IYSATE SUPPRESSION OF MACROPHAGE FUNCTION
S Miles A Sandler A Boyajian M Kurzok
Tumor vaccines are of great interest but as yet are of unmet potential. A common method of providing
antigenic stimulation is the use of whole tumor cell lysate as a source of multiple antigen epitopes from
the tumor. This study was undertaken to determine the effect of tumor lysate on primary macrophages as
a source for antigen processing. Our results show that tumor lysate suppresses macrophage function and
that the cell membrane is responsible for this inhibitory effect. This effect was not only specific to tumor
cells, but was also found in lysate from normal cells. When the lysate was encapsulated in microparticles
or pre treated, the suppressive effect was reversed. These findings may be critical for the use of lysate as
the antigen source in tumor vaccine formulation.
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HYPEROXIA CAUSES ASTROGLIOSIS IN THE DEVELOPING BRAIN
TS Schmitz VG Gallo LC Chew
Periventricular white matter damage (WMD) is a major cause of impaired neurological development and
mental retardation in premature infants. Contributing factors currently include perinatal infection,
inflammation and hypoxia/ischemia. High oxygen (80%) concentrations have also been shown to cause
hypomyelination and oligodendrocyte progenitor cell death (Gerstner 2008). In many animal models of
perinatal white matter damage, the observation of astrocyte activation (astrogliosis) has often been
associated with CNS inflammation. It is possible that astroglial changes may also contribute to the
pathogenesis of white matter loss following oxygen exposure. We have chosen to investigate the effects
of oxygen on astrocytes in vivo and in vitro. In a transgenic mouse line bearing astrocyte-targeted reporter
expression (glial fibrillary acidic protein promoter, GFAP-EGFP), exposure of postnatal day 6 (P6) pups
to 48h hyperoxia initially decreased EGFP reporter expression in white matter regions immediately
following treatment, i.e. at P8. However, EGFP increased and exceeded control levels after 4 days
recovery (P12), an age at which developmental myelination begins. To determine whether hyperoxia
produces direct effects on astroglial cells, subconfluent cultured rat astrocytes were exposed to 80%
oxygen and analyzed for cell growth and survival at various time points after recovery. MTT reduction,
BrdU incorporation assays and Ki67 immunostaining revealed decreased proliferation immediately after
48h of oxygen exposure, which returned to control levels after 2 days recovery. After 4 days of recovery,
Ki67 immunoreactivity and percentage of GFAP+ cells in oxygen-treated astrocytes significantly
exceeded that of controls. Apoptotic cell death by TUNEL assay and cleaved caspase 3 staining was
undetectable in this culture paradigm. These results indicate that neonatal hyperoxia induces astrogliosis
in vivo and in vitro by a mechanism involving a temporary phase of growth inhibition, and suggest that an
astroglial reaction may play an important role in hyperoxia-induced white matter damage.
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ALTERNATIVE RESCUE REPLICATION PROGRAM IN KAPOSI’S
SARCOMA ASSOCIATED HERPES VIRUS IN RELATION TO HOST
CELL DEATH
A Prasad G Fernandez M Lu SL Zeichner
Kaposi’s Sarcoma-associated Herpes Virus (KSHV), also called Human Herpes Virus-8 (HHV-8),
belongs to Gammaherpesvirinae family. KSHV has a genome of approximately 140 kB with 90 Open
Reading Frames. KSHV is etiologically related to three types of diseases- Kaposi’s sarcoma, Primary
Effusion Lymphoma (PEL, also called body cavity based lymphoma) and Multicentric Castleman’s
Disease. One of the salient features of KSHV genome is that it has cellular accessory gene homologs, also
called “pirated genes,” which are known to interfere with a number of regulatory functions of cells (cell
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cycle control, apoptosis, anti viral immunity). Data from our previous studies on KSHV infected body
cavity based lymphoma-1 (BCBL-1) cells showed that the KSHV transcription program proceeds in an
ordered fashion, with immediate early genes, early genes, and late genes expressed in turn. The kinetics of
these genes was related to the function of these genes. For instance, ORF57 is a transcription activator
and is required early during the lytic replication of KSHV and therefore showed an early expression.
ORF17, a structural protein gene, was expressed late. Studies also showed that ORF50 is a transactivator
and lies at the apex of transcription program; the induction of ORF50 is sufficient to activate all KSHV
genes for completion of the viral replication. Our current efforts are aimed towards understanding how the
KSHV transcription program proceeds in the presence of a dominant negative mutant of ORF50,
ORF50/STAD. These studies lead to a serendipitous observation of an alternative rescue replication
pathway in KSHV in ORF50/STAD BCBL-1 cells undergoing apoptosis. Our data shows that host cell
apoptosis triggers KSHV replication via an alternative replication pathway that does not require ORF50.
Our data also shows that this alternative rescue pathway follows different kinetics and has a different gene
expression pattern compared to the normal KSHV replication program.
ROLE OF TUMOR NECROSIS FACTOR-ALPHA-RELATED
APOPTOSIS-INDUCING LIGAND (TRAIL) IN MEDIATING MUSCLE
FIBER DAMAGE IN MYOSITIS
24
R Rawat E Pistilli M Mistak D Francia N Raben P Plotz E Hoffman K Nagaraju
It is known that multinucleated muscle cells are relatively resistant to classical forms of apoptosis. We
propose that autophagic cell death may play a role in the pathogenesis of autoimmune myositis. Factors
that initiate autophagic cell death in muscle are not well defined. Recent literature indicates that TRAIL
may induce not only NF-kB activation but also autophagic cell death in epithelial cells. Therefore, we
have investigated its role in muscle biopsies of myositis patients. We have analyzed previously published
13 muscle disease gene expression profiling database for TRAIL expression. We have performed
immunohistochemistry for TRAIL expression. Western blotting and Immunofluorescence for Ik-B
degradation and NF-kB activation respectively in cell culture system. We have found that TRAIL is
expressed predominantly in muscle fibers of myositis but not in normal, DMD and LGMD2B muscle
biopsies. Likewise, autophagy markers such as Beclin and LC-3 are upregulated in myositis but not
control biopsies. The TRAIL expression in muscle fibers is restricted to atrophic fibers. It is also found on
blood vessels and some infiltrating lymphocytes. We have further shown in vitro in cell culture system
that TRAIL induces NF-kB activation and IkB degradation. Further we have shown small molecule Ik-B
inhibitor (BMS-345541) efficiently blocks TRAIL induced NF-kB nuclear translocation in cultured cells.
Our data demonstrate that TRAIL is expressed in myositis but not other muscle disease biopsies. TRAIL
expression is restricted to atrophic muscle fibers and may likely to mediate not only NF-kB activation in
myositis but also autophagic cell death in multinucleated muscle cells. TRAIL may be an attractive target
for therapeutic intervention in myositis.
CHARACTERIZATION OF THE MOLECULAR BASIS OF NEURAL TUBE
CLOSURE AND PLACENTAL DEFECTS IN THE OPENMIND MUTANT
MOUSE MODEL
AA Sarkar IE Zohn
Neural tube and placental defects are common complications of pregnancy. Neural tube defects lead to
death of the fetus or long-term disability, while placental defects cause preeclampsia, intrauterine growth
restriction and miscarriage. Genetic studies in mouse models indicate that morphogenesis of both the
neural tube and the placenta require the activity of a large number of genes. How these gene products are
organized into pathways and how their activities are fine-tuned by post-translational modifications to
regulate morphogenesis of the neural tube or the placenta remains unknown. The openmind (opm) mutant
mouse is a model of both neural tube defects and placental insufficiency. Exencephaly in homozygous
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opm mutants is associated with an abnormal expansion of the cranial mesenchyme during neurulation.
We are currently characterizing placental defects in opm mutants using cell-type specific markers to gain
a better understanding of the molecular pathways regulated by the opm mutant gene. Positonal-cloning of
the opm mutation revealed that the placental and neural tube defects are due to a null mutation in a novel
HECT domain ubiquitin ligase (Hectd1). Complementation assay using a genetrap allele for Hectd1
confirm that the absence of a functional Hectd1 gene is responsible for the opm phenotype. Since HECTdomain ubiquitin ligases directly interact with their substrates, we have conducted a yeast two-hybrid
screen to identify the molecular pathways regulated by Hectd1. From this screen we identified a number
of putative Hectd1 substrates, some of which play known roles in regulation of neural tube closure and
placentation. A few of the possible substrates are also known to be regulated by ubiquitination. We are
currently validating the putative Hectd1 substrates identified in our screen in binding and ubiquitination
assays. Finally, we will determine whether a failure of Hectd1-dependent ubiquitination of these
substrates contributes to placenta and neural tube defects in opm mutants.
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USE OF MAGNETIC RESONANCE IMAGING (MRI) TO DETECT
SKELETAL MUSCLE CHANGES IN MDX MOUSE MODELS OF
MUSCULAR DYSTROPHY
D Guerron S Fricke R Sze E Pistilli G Pandey K Nagaraju
MRI represents the best non-invasive method to visualize soft tissue changes and therefore is well suited
to study progressive pathological changes in skeletal muscle. We have performed repeated MRI
measurements of skeletal muscle in mdx mice and control mice to better understand the sensitivity of
MRI to differentiate normal and pathological mouse muscle. We used a 7-Tesla magnet equipped with a
customized stereotaxic positioning device that helps to locate the same tissue and region of interest
consistently across scanning sessions. Preliminary experiments using mdx and control mice demonstrated
that we were able to detect inflammation in the hind limb of mdx mice. Quantification of inflammation
using the image post-processing program “Image J” as well as a manual clinical assessment scale showed
significant differences between normal and disease mice. These evaluations concluded that: MRI can
reliably detect muscle inflammation and measure volumetric changes in skeletal muscle also, MRI is ideal
to monitor the progression of disease and response to therapeutic intervention(s). Efforts are also
currently underway to perform MR spectroscopy on mdx mice.
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CELLULAR MECHANISM UNDERLYING THE LEFT-RIGHT
ASYMMETRY DEFECTS OF CCDC40 MUTANT MICE
J Mao I Zohn
Left-right asymmetry, the characteristic asymmetric arrangement of the visceral organs, is initiated by
fluid flow generated by cilia in the embryonic node. Genetic experiments in the mouse have been
instrumental in identifying the genes involved in the determination of left-right asymmetry, most of which
are required for formation of nodal cilia. The links mutant mouse line exhibits defects in establishment of
the left-right axis and contains a non-sense mutation in an uncharacterized coiled-coil domain-containing
protein, Ccdc40. The nodes of links mutant embryos posses fewer, shorter cilia than in wildtype embryos,
implicating Ccdc40 in the formation and/or maintenance of nodal cilia. To further understand the cellular
mechanism of the cilia defects in links mutant embryos, we examined the effect of overexpression of
wildtype and mutant Ccdc40 in MDCK cells. As in the node of wildtype embryos, full length HA- or
EGFP-tagged Ccdc40 was localized to the cytoplasm of MDCK cells. Furthermore, formation of cilia was
disrupted in MDCK cells expressing pEGFP-Ccdc40, pEGFP-Ccdc40N but not pEGFP-Ccdc40C. Recent
data indicates that in order for cilia to form in MDCK cells, the cells must be polarized along their apicalbasal axis. To determine if Cdcd40 is required for polarization of MDCK cells we examined expression of
a number of proteins localized to the basolateral membrane. The basolateral markers such as E-cadherin,
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ZO-1 and beta-catenin were normal in cells expressing the Ccdc40 constructs. Interestingly, our
preliminary data indicates that the apical marker (gp135) was disrupted in MDCK cells expressing
pEGFP-Ccdc40, pEGFP-Ccdc40N but not pEGFP-Ccdc40C. These results indicate that Ccdc40 may be
required for the maintenance of apical-basal polarity and subsequent formation of cilia at the apical
membrane. Furthermore, disruption of this polarity in links mutant mice disrupts cilia formation and
development of left-right asymmetry.
ROLE OF INFLAMMATION AND REGENERATION IN MUSCLE
WEAKNESS IN DIFFERENT MOUSE MODELS OF MYOPATHY
28
J VanderMeulen G Pandey R Gernapudi A Sali V Farajian E Pistilli K Nagaraju
Inflammation and regeneration is a characteristic feature of autoimmune and genetic muscle diseases. We
propose that the extent of inflammation and regeneration may influence muscle weakness in myopathies.
Here, we have selected three muscle disease (Duchenne muscular dystrophy (mdx), Limb Girdle
Muscular Dystrophy 2B (A/J) and myositis (MHC class I)) models that show varying degrees of skeletal
muscle regeneration and inflammation and compared them to control (C57Bl/6) mice. We have conducted
in vitro force measurements (e.g., maximal force, specific force, fatigue, and stretch-induced injury) on
EDL muscles from 5 month old female mice. Muscle sections were stained with H&E and fibers were
analyzed for inflammation and central nuclei. EDL muscles of mdx and MHC class I mice at this age
showed significant inflammation, whereas mdx mice also showed significant regeneration in comparison
to A/J mice where neither inflammation nor regeneration were noted. Functional measurements indicate
there is a significant deficit in specific force and increase in fatigue and stretch-induced injury in the mdx
mice, whereas MHC class I mice showed a decrease in maximal and specific force and no force deficits
were noted in the A/J mice. These data suggest that inflammation and regeneration affect muscle function
in mdx and MHC class I but not in A/J mice indicating that inflammation and regeneration may
contribute to the muscle weakness in certain models of myopathy.
EFFECTS OF IL-15 ON MUSCLE PATHOLOGY AND FUNCTION IN A
MOUSE MODEL OF LIMB GIRDLE MUSCULAR DYSTROPHY TYPE 2B
(LGMD2B)
R Gernapudi E Pistilli D Guerron N Kanneboyin
Dysferlin deficiency causes Limb Girdle Muscular Dystrophy type 2B. The A/J mice show features of
human LGMD2B. It has been proposed that anabolic growth factors increase muscle mass and improve
muscle function in mdx mice. Here we have tested a cytokine, Interleukin-15 (IL-15) that is known to
have anabolic properties on skeletal muscle. Two groups of A/J mice (n=16) were treated either with IL15 (10ug/day/mice for 21days) or saline using mini-osmotic pumps. After 2 weeks of IL-15
administration, we have noticed rejection of osmotic pumps. Only 4 out of 16 IL-15 treated mice reached
21 day study period. We have found that heart, spleen, tibialis anterior, and plantaris weights are
significantly increased in comparison to saline treated group. Further, the Spleenocytes in IL-15 treated
group showed increased proliferation in response to LPS. Fiber diameter of soleus was smaller than saline
treated group between diameter 30-100 micrometers. We also noted significant increase in small cells
between diameter 10-30 micrometers in IL-15 treated group indicating presence of inflammatory cells or
small degenerating or regenerating muscle fibers. These data indicate that IL-15 activates immune system
and help to facilitate pump rejection. Further, we did not see the reported anabolic effects on skeletal
muscle in the AJ mouse model and future experiments will clarify these discrepancies between the
models.
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CELLULAR CHARACTERIZATION OF WHITE MATTER INJURY AFTER
PERINATAL HYPOXIA
B Jablonska J Scafidi V Gallo
The growing population of preterm infants with very low birth weight (VLBW) suffers from
neurodevelopmental and cognitive delays. The leading cause of brain injury in this context is
periventricular white matter injury (PWMI) that can be induced by hypoxia. To study white matter injury
after hypoxia, we used a mouse model that mimics most of the problems of preterm infants, including: i)
alterations in brain size and weight; ii) ventricular enlargement, and iii) significant reduction in white and
gray matter volume. Mice were exposed to hypoxia from postnatal day (P3) to P11, and their brains were
analyzed after one week of recovery in normoxic conditions. We found that hypoxia causes enlargement
of the lateral ventricle and expansion of the subventricular zone (SVZ). In the SVZ, we observed a
significant increase in cell proliferation, as determined by labeling cycling cells with anti-Ki67 antibody.
To trace oligodendrocytes lineage development, we used CNP-EGFP mice, in which various stages of the
oligodendrocyte lineage are visualized by EGFP expression driven by the CNP promoter. We found that,
in the SVZ, hypoxia upregulates the number of progenitors expressing Lex, Dcx, Dlx and NG2, whereas
the number of progenitors expressing Nkx2.2 is reduced. To study the effects of hypoxic injury on white
matter development, we labeled different stages of the oligodendrocyte lineage in corpus callosum (CC)
with specific markers including CC1, Olig2 and S100. The number of oligodendrocyte progenitors
expressing Lex, NG2 and Nkx2.2 is elevated in the CC after hypoxia. Conversely, a decrease in mature
oligodendrocytes was observed, together with a reduction in MBP expression. Our results suggest that
hypoxia exert differential effects on distinct progenitor populations in the SVZ and white matter.
Supported by R01NS045702 and IDDRC P30HD40677(VG), IDDRC T32 Program Training (BJ),
NSADA Award (JS)
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ALTERATIONS IN THE BASOLATERAL COMPLEX OF THE
AMYGDALA IN THE FMR1 MOUSE MODEL OF FRAGILE X
L Olmos S Paluszkiewcz W Kaufmann M Huntsman J Corbin
Fragile X Syndrome (FXS) is a neurodevelopmental disorder characterized by a variety of
neurobehavioral abnormalities, including cognitive impairment and enhanced anxiety. In line with the
latter, converging lines of evidence indicate that the amygdala, a key component of the brainВ№s limbic
system that is involved in circuitry that regulates fear and anxiety behaviour, is a primary brain structure
affected in FXS. Despite this understanding, little is currently known regarding the development of
amygdala circuitry in the Fmr1 mutant mouse model and the consequences of these putative abnormalities
on amygdala physiology. Using a combination of neuroanatomical and electrophysiological approaches,
we examined the development of the amygdala in the Fmr1 mutant mouse model, with specific focus on
the basolateral complex (BLC) of the amygdala. Our results reveal that the Fragile X Mental Retardation
Protein (FMRP) is highly expressed in BLC neurons during postnatal stages. Furthermore, our data also
reveal no specific alterations in the number of neuronal subtype markers in the BLC. At the physiological
level, we have found an enhanced excitability in excitatory cells and abnormalities in feedforward
inhibition in the BLC. Together, these findings suggest the presence of specific alterations in the
amygdala in Fmr1 mutant mice.
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IMATINIB MESYLATE(GLEEVEC): ATTACKING THE CULPRIT(PDGFR)
AND THE ACCOMPLICE (EGFR) IN CHILDHOOD
MEDULLOBLASTOMAS
32
TJ Abouantoun TJ MacDonald
Expression of the platelet-derived growth factor receptor (PDGFR) correlates with metastatic
medulloblastoma and PDGF treatment of medulloblastoma cells induces ERK, AKT and PI3K activity
and decreases PTEN expression and activity. PDGFR can also heterodimerize with and transactivate
EGFR. In this study, we sought to determine whether targeting PDGFR activity effectively inhibits
signaling required for medulloblastoma cell migration and survival and whether it blocks PDGFRinduced transactivation of EGFR. Daoy and D556 human medulloblastoma cells were transfected with
siRNA to PDGFRbeta and treated with Imatinib mesylate (Gleevec), a specific inhibitor of PDGFR, to
block PDGFR expression and activity, respectively. Cell migration, survival and PDGFR signaling
following PDGFBB stimulation of serum-depleted cells, with and without PDGFR inhibition, was
measured. PDGFBB treatment of cells enhanced survival and proliferation after 24h; increased
PDGFRbeta, PI3K, AKT and ERK activity, decreased PTEN activation and transactivated EGFR.
Gleevec (1uM) treatment of PDGFRbeta active cells induced apoptosis at 72h and inhibited migration at
24h after a single dose and concomitantly inhibited PDGFBB activation of PDGFRbeta, PI3K, AKT and
ERK but promoted PTEN activity. siRNA silencing of PDGFRbeta similarly inhibited survival, migration
and signaling and both siRNA and Gleevec treatment inhibited PDGFBB-induced EGFR trans-activation.
Inhibition of medulloblastoma PDGFRbeta by siRNA or Gleevec treatment effectively blocks
PDGFRbeta signaling and EGFR transactivation. These results indicate that PDGFRbeta tyrosine kinase
activity is critical for survival and migration of medulloblastoma cells, in part by decreasing PTEN
activity and transactivating EGFR, and thus may represent an important therapeutic target for eliminating
metastasis in this disease.
MIGLUSTAT IMPROVES FUNCTION IN JUVENILE GM1
GANGLIOSIDOSIS: IS THERE A PHRAMACOLOGICAL CHAPERONE
EFFECT?
CP Morgan DR Adams CJ TIfft
GM1 gangliosidosis, caused by a deficiency of lysosomal beta-galactosidase, is a neurodegenerative
disorder with a broad clinical spectrum reflecting the degree of residual enzyme activity. Miglustat, an
imino sugar, competitively inhibits glucosylceramide synthase the first step in glycosphingolipid
synthesis and can reduce the synthesis of GM1 ganglioside. Imino sugars have also been shown to act as
molecular chaperones with a number of acid hydrolases, including Гџ-galactosidase (Tominaga et al, 2001;
Yam G. et al, 2006). The patient is an 18-year-old male with precocious development until age 5 when he
developed deterioration of expressive and receptive language and gait disturbance. After a 7 year
diagnostic odyssey juvenile GM1 gangliosidosis was confirmed. He continued to decline, and by age 16
he had lost all speech skills and was non-ambulatory. After 3 years of miglustat therapy he has regained
the ability to speak in sentences and short paragraphs although remains dysarthric, and has gained some
tentative ambulatory skills limited by hip dysplasia. The patients fibroblasts were incubated with
miglustat for up to 4 days at 5, 25, and 50uM, which is within the range of plasma concentrations in
treated patients. We found that beta-galactosidase activity was significantly increased 35-45% in treated
cell lysates (p<0.01). Here we report a patient with juvenile GM1 gangliosidosis who showed rapid
clinical improvement following treatment with miglustat. In vitro studies on patient fibroblasts indicate
that this improvement may be due to an increase in Гџ-galactosidase activity resulting from a
pharmacological chaperone effect. Studies are underway in additional juvenile GM1 patients to further
characterize the effect of miglustat on beta-galactosidase activity.
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GENETIC VARIANTS ARE ASSOCIATED WITH BASELINE MUSCLE
STRENGTH AND SUBCUTANEOUS FAT VOLUME
K Adham RR Patel JS Larkin C Brandoli H Gordish-Dressman EP Hoffman JM Devaney LL Tosi
Understanding how genetic variants play a role in muscle phenotypes will give us insight into the
extremely complex biology of resistance training and its role in defining the physical health of a
population. Chemokines and chemokine receptors are part of the inflammatory system that is activated by
muscle damage and is involved in the regulation of communication between muscle and macrophages
after injury. Given effects of the chemokine (CCL2), we hypothesized that single nucleotide
polymorphisms (SNPs) in CCL2 and its receptor CCR2 would be associated with skeletal muscle
phenotypes after a resistance training program. Using DNA samples (746 individuals) and MRI images
from the FMS study, we sought significant associations between single nucleotide polymorphisms (SNPs)
and skeletal muscle phenotypes. The G allele for rs1024611 was associated with baseline muscle quality
and a reduced amount of baseline subcutaneous fat in females. In males, the G allele was associated with
a higher baseline 1RM (one repetition max), baseline isometric strength, baseline 1RM. The G allele for
rs1024610 in males was associated with a gain in baseline isometric strength and baseline isometric
torque. Males with two copies of the T allele for rs13900 showed greater baseline 1RM muscle strength
and 1RM torque. Females with two copies of the T allele for rs13900 showed a greater baseline 1RM
muscle quality. This study suggests that baseline values of 1RM and isometric strength are influenced by
SNPs in CCL2. In addition, female levels of subcutaneous fat are influenced by SNPs in CCL2. We did
not find any associations with SNPs in CCR2 with values of muscle strength and size in males or females.
The SNPs in CCL2 may play a role in the expression level of the gene in muscle and influence the
development of the strength component.
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GENETIC VARIANTS IN CCL2 AND CCR2 ARE ASSOCIATED WITH
MUSCLE DAMAGE
JS Larkin K Adham C Brandoli H Gordish-Dressman M Hubal E Hoffman JM Devaney L Tosi
Understanding how genetic variants play a role in muscle phenotypes will give us insight into the
extremely complex biology of resistance training and its role in defining the physical health of a
population. Chemokines and chemokine receptors are part of the inflammatory system that is activated by
muscle damage and is involved in the regulation of communication between muscle and macrophages
after injury. Given effects of the chemokine (CCL2), we hypothesized that single nucleotide
polymorphisms (SNPs) in CCL2 and its receptor CCR2 would be associated with skeletal muscle
phenotypes after a resistance training program. Using DNA samples (746 individuals) and MRI images
from the FMS study, we sought significant associations between single nucleotide polymorphisms (SNPs)
and skeletal muscle phenotypes. The G allele for rs1024611 was associated with baseline muscle quality
and a reduced amount of baseline subcutaneous fat in females. In males, the G allele was associated with
a higher baseline 1RM (one repetition max), baseline isometric strength, baseline 1RM. The G allele for
rs1024610 in males was associated with a gain in baseline isometric strength and baseline isometric
torque. Males with two copies of the T allele for rs13900 showed greater baseline 1RM muscle strength
and 1RM torque. Females with two copies of the T allele for rs13900 showed a greater baseline 1RM
muscle quality. This study suggests that baseline values of 1RM and isometric strength are influenced by
SNPs in CCL2. In addition, female levels of subcutaneous fat are influenced by SNPs in CCL2. We did
not find any associations with SNPs in CCR2 with values of muscle strength and size in males or females.
The SNPs in CCL2 may play a role in the expression level of the gene in muscle and influence the
development of the strength component.
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PROGENITOR POPULATIONS IN THE DEVELOPING VENTRICULAR
ZONE DISPLAY DISTINCT CELL CYCLE KINETICS
36
EK Stancik I Navarro-Quiroga TF Haydar
We have shown that the developing mouse telencephalic ventricular zone (VZ) contains progenitor
populations that can be separated based on promoter expression. In order to see if these populations also
differed in their cell cycle kinetics, we developed a new method to calculate cell cycle length that takes
advantage of these differences in promoter expression. Using in utero electroporation to label cohorts of
cells, we calculated cell cycle length based on the percentage of labeled cells in either S-phase or Mphase, as determined by BrdU incorporation or condensed chromatin, respectively. As a control, we used
a plasmid with the ubiquitously expressed CAG promoter (pCAG-RFP) and determined the length of the
cell cycle for RFP+ cells. We then electroporated with plasmids containing the preferentially expressed
GLAST and TО±1 promoters and compared cell cycle kinetics between the two populations. Our
calculations with CAG-RFP were highly comparable with published findings for cell cycle length in
embryonic mouse VZ. We found that cells expressing the TО±1 promoter took longer to reenter both Sand M-phase after electroporation than did cells expressing the GLAST promoter, suggestive of a longer
cell cycle. These results show that using in utero electroporation in conjunction with BrdU labeling
provides a reliable means of calculating cell cycle length of the different progenitor populations.
A GENETIC POLYMORPHISM OF THE MUC7 MUCIN GENE IN
ASSOCIATION WITH ASTHMA AND ETHNICITY
37
AM Watson W Ngor H Gordish-Dressman RJ Freishtat MC Rose
The onset and development of asthma is influenced by the environment and genotype. Different ethnic
populations are more susceptible to asthma. Mucins contribute to airway obstruction in asthma and
coding regions of mucin genes exhibit a high degree of variation in their number of tandem repeats (TR).
An increased frequency of the MUC7*5 polymorphic allele is associated with a decreased risk of being
asthmatic in a Northern European cohort (Kirkbride HJ et al., 2001), but has not been evaluated in other
ethnic populations. We evaluated the expression of MUC7 allelic polymorphisms and the associated risk
of being asthmatic in an African American cohort. We studied a population of 48 inner-city asthmatics in
the CNMC Asthma Severity Modifying Polymorphisms (AsthMaP) DNA database and 36 non-asthmatic
controls. In the asthmatic population we found the following frequencies: MUC7*6 allele, 0.99 and
MUC7*5 allele, 0.01. In the control population the frequency results were: MUC7*6 allele, 0.91,
MUC7*5 allele, 0.08 and MUC7*4 allele, 0.01. Logistic regression analysis of the association of MUC7
allelic polymorphisms and asthma gave an odds ratio of 0.08 (CI: 0.009 – 0.716) and a p-value of 0.023.
Therefore, these frequencies demonstrate that MUC7*5 allelic polymorphism is significantly associated
with a decreased risk of being asthmatic in African Americans, as in the Northern European cohort.
Additionally, the previously unidentified MUC7*4 polymorphism, confirmed by gel electrophoresis and
sequencing, resulted not only in a reduction in the number of TR but also a rearrangement of the TR. The
reduction in TR domains results in a reduction of O-glycosylation sites, which could have a positive
impact on the biological function of MUC7 mucin and disease outcome. Future studies will determine
mechanisms by which polymorphisms in the MUC7 gene alter the host innate immune response of MUC7
mucin.
QUANTITATIVE ANALYSIS OF THE MALIGNANT GLIOMA
SECRETOME
CA Formolo TJ MacDonald Y Hathout
The secretome constitutes an important class of proteins that control and regulate a multitude of
physiological and pathophysiological processes. Some proteins could be differentially secreted depending
on disease stage such as tumor grade or malignancy, thus making the secretome an attractive source for
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biomarkers and therapeutic target discovery. In the present study we used a proteomic approach to
identify potential brain tumor biomarkers secreted by astrocytoma cells of differing tumorigenicity in
vitro. We used stable isotope labeling by amino acids in cell culture (SILAC) to quantify differentially
secreted proteins from the U87 (tumorigenic astrocytoma) and T98 (non-tumorigenic astrocytoma) cell
lines. Our preliminary results show 27 differentially secreted proteins between U87 tumorigenic
astrocytoma cells and T98 non-tumorgenic astrocytoma cells. Most of these differentially expressed
proteins were found to be implicated in matrix remodeling, tumor cell invasion and metastasis and were
secreted by tumorigenic cells in abundance up to four-fold greater than by non-tumorigenic cells.
Interestingly, some proteins were only secreted by the U87 cells. Notable were the singular secretions of
cathepsin L which is known to be involved in metastasis, and complement C3 with no known relationship
to tumorgenesis. These differentially secreted proteins may provide insight into the distinct responses to
treatment between the two grades of malignant glioma or may act as distinguishing diagnostic markers. In
the future we hope to examine the biological significance of these proteins as well as verify their presence
in the cerebrospinal fluid of brain tumor patients.
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SORTING OF HUMAN CYTOMEGALOVIRUS UL37 PROTEINS
THROUGH THE MAM TO MITOCHONDRIA OR TO GOLGI
CD Williamson P Bozidis AM Colberg-Poley
Objective: To examine the utilization of mitochondria-associated membranes (MAM), specialized
endoplasmic reticulum (ER) subdomains, in facilitating ER to mitochondria trafficking of human
cytomegalovirus (HCMV) UL37 proteins. Methods: A centrifugation method, employing differential
sucrose and self-forming Percoll gradients, was adapted to separately purify MAM, ER, and
mitochondria. HeLa cells lipofected with expression vectors encoding a MAM constituent protein (EGFPPSS1) and wild-type or mutant HCMV UL37 proteins were observed using confocal microscopy, or
fractionated and examined by Western blot analysis. HCMV-infected primary human diploid fibroblasts
(HFFs) were similarly analyzed. Results: The predominant HCMV UL37 protein product, pUL37x1, as
well as the glycoprotein, gpUL37, were detected in MAM of transfect HeLa cells. Mutant proteins
defective in mitochondrial importation also appeared defective in MAM localization. Strikingly, the Cterminal cleavage product of gpUL37 (gpUL37COOH), known to traffic from the ER to Golgi, was found
concentrated in MAM subdomains, but was not detected in mitochondria. MAM localization of UL37
proteins was verified in HCMV-infected HFFs. Conclusions: Our findings indicate that MAM can act as a
sorting compartment for two different trafficking pathways, allowing ER synthesized proteins to be
targeted into the Golgi apparatus or into mitochondria.
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AN IN VITRO SCREENING ASSAY TO IDENTIFY POTENTIAL NF-ОєB
INHIBITORS TO TREAT AUTOIMMUNE AND GENETIC MUSCLE
DISEASES
AR Baudy EP Hoffman K Nagaraju
The NF-ОєB pathway plays a central role in the initiation and perpetuation of inflammation in several
human disorders. Molecular profiling studies from our group demonstrated that the NF-ОєB pathway is
strongly activated in skeletal muscle in both autoimmune and genetic muscle diseases. However, the exact
contribution of these pathways to in these disorders is unknown. Existing NF-ОєB inhibitors were
primarily developed to suppress NF-ОєB activation in immune cells but preliminary experiments from our
lab suggest that inhibitors that work on immune cells fail to work on skeletal muscle cells. Therefore, we
have developed an in vitro screening assay to screen compounds that inhibit NF-ОєB activation in C2C12
muscle cells. Commercially available (Panomics) NF-ОєB reporter C2C12 stable myoblast cells were
cultured with DMEM with 10% FBS (ATCC) or differentiated into myotubes and then both cell types
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were plated and allowed to grow for 24hr, then treated with various agents before inducing NF-?B
activation with TNF-a for the next 24 h. We have screened the following compounds for NF-?B inhibitor
potential: Pirfenidone, Fibroblast growth factor (FGF), EGCG, Prednisone, Thymosine beta4, Imatinib,
Withaferin A, IKK inhibitor VII, Celastrol, and Bay 11-7085. Some compounds significantly inhibited
NF-ОєB activity (e.g., Celastrol) while others (e.g., Pirfenidone) showed modest effects in skeletal muscle.
Some compounds that efficiently inhibited NF-ОєB in immune cells failed to show significant inhibition in
muscle cells (e.g. IKK VII). We have developed an in vitro assay to screen NF-ОєB inhibitors for
autoimmune muscle diseases. We have identified that compounds that efficiently block NF-kB with
minimal cytotoxicity both in myoblasts and myotubes. This assay will help to screen and identify
potential NF-kB inhibitors to treat autoimmune and dystrophic muscle disease.
DYNAMIC CELL MOVEMENTS AND THEIR RELATIONSHIP TO FATE
AT THE TELENCEPHALIC CORTICOSTRIATAL BORDER
41
LA Cocas RS Carney N Kessaris TF Haydar JG Corbin
During embryogenesis, the corticostriatal border (CSB) divides two compartments in the telencephalon:
the pallium, which will become the cerebral cortex and hippocampus, and the subpallium, which will
become the basal ganglia, piriform cortex, and amygdala. The CSB serves as physical and molecular
barrier, preventing mixing between the pallial and subpallial compartments from which telencephalic
excitatory and inhibitory neurons will arise, respectively. The initial patterning of the CSB occurs around
embryonic day 10 (E10) in the mouse via the genetic cross repression of the homeobox gene Gsh2
ventrally (subpallial) and the paired homeobox gene Pax6 dorsally (pallial). Previous work from our lab
and others has shown that the CSB is composed of a mixed population of cells, with certain cells
expressing markers for the ventral homeobox gene Dlx2, other cells expressing the dorsal Pax6 gene, and
a third population of cells expressing markers for both Dlx2 and Pax6 (Puelles, et al., 2000, J. Comp
Neurol; Medina, et. al, 2004, J. Comp Neurol; Carney et al., 2006, J. Neurosci). In addition, these cells
contribute to portions of the basal telencephalic limbic system, specifically, the developing amygdala and
piriform cortex. Our data indicate that formation of the CSB is a complex event in which pallial and
subpallial neural progenitor populations appear to undergo early patterns of cell mixing with subsequent
refinement into separate compartments. However, the dynamic neural progenitor cell movements that
occur during CSB formation and their relationship to the fate of pallial and subpallial neural progenitor
cells that are generated on either side of the border are unknown. We are currently examining this
question using a combination of multiphoton time lapse imaging and cell fate analysis using mice in
which these progenitor cell populations are genetically marked with fluorescent reporters.
PARENT REPORTED SLEEP COMPLAINTS IN YOUTH DIAGNOSED
WITH AUTISM SPECTRUM DISORDERS
H Bhatt E Huntley M Monaghan CA Alfano DS Lewin
Children with Autism Spectrum disorders (ASD) have myriad sleep problems that place a significant
burden on families. The current study describes: (a) specific sleep complaints reported by caregivers of
children diagnosed with ASD and; (b) the relationship between sleep complaints and psychiatric
symptoms. Intake data from 13 children with ASD presenting to a pediatric behavioral sleep medicine
clinic were examined. Fifty three percent had a language and motor delay, 30% had no speech, and 69%
received special services at school. Data were collected via a preliminary phone interview, a 1-2 hour inperson interview and observation of the caregiver and child, behavioral rating scales including a child
sleep questionnaire (CSQ) and the Child Behavior Checklist (CBCL). Associations between the CSQ and
CBCL were explored. Sleep complaints, especially problems initiating and maintaining sleep, are highly
prevalent among youth diagnosed with ASD. Overall, findings suggest that self-regulatory skills needed
for sleep onset and maintenance present a significant challenge for ASD youth.
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THE EFFECT OF PREDNISONE IN OVERALL PHENOTYPE IN CLASS I
MAJOR HISTOCOMPATIBILITY COMPLEX (MHC) – TRANSGENIC
MURINE MODEL OF MYOSITIS
V Farajian GS Pandey J VanderMeulen A Sali R Gernapudi HJ Cha D Francia K Nagaraju
Myositis is an autoimmune disease of unknown etiology. It is known that both immune and non-immune
mechanisms play a role in the pathogenesis of this disease. Transcription factor, NF-kB, links both
immune and non-immune components of muscle damage. The immunosuppressive properties of
glucocorticoids (Prednisone) are known to be through the inhibition of NF-kB activation. Therefore, we
propose that the administration of prednisone, in the class I MHC transgenic mouse model of myositis,
will help to alleviate disease phenotype. We performed southern and northern blotting for genotyping and
RNA estimation, blood serum Creatinine Kinase assay, invitro muscle force measurement, behavioral and
functional activity measurement, histology and fibrosis to estimate the effects of prednisone. Chronic
continuous administration of prednisone (1mg/kg) did not show significant differences in body weight
between treated and untreated mice. However, prednisone treated mice exhibited a trend towards
reduction in the maximal and specific force of Soleus (type I) and EDL (type II) muscle fibers when
compared to the untreated group. Even though behavioral activity measurements (vertical and horizontal
activities) showed slight improvements as opposed to decrease in the forelimb and hind limb grip
strength, none of these parameters were statistically significant in comparison to control mice.
Additionally, we noticed an increase in serum creatinine kinase levels and no change in muscle fibrosis.
Our present study indicates that prednisone may not significantly improve the muscle function and
phenotype in class I MHC mice. Currently, histological and biochemical analysis are underway in order
to understand the effects of prednisone at a molecular level.
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HUMAN NEURAL PRECURSOR CELL SUSCEPTIBILITY TO HUMAN
CYTOMEGALOVIRUS
JH Haile DP Panchision CP Colberg-Poley
Human cytomegalovrius (HCMV) is the leading viral cause of congenital birth defects. These birth
defects include mental retardation, blindness, microcephaly, and hydrocephalus, all of which can arise
from disorders of the developing central nervous system. Thus, HCMV has a significant neuropathogenic
effect. The aim of our study is to investigate the viral pathogenesis of HCMV on human neural precursor
cell (NPC) growth, differentiation, and survival.
45
S. PNEUMONIAE DETECTION: EFFECTS OF LOW DENSITY AND
DELAY TO PROCESSING
AS Sampath VB Balaji VV Valsan MS Steinhoff
While blood cultures remain a mainstay assessing bacteremia in children, the challenges of low volumes
of blood available, low levels of bacteremia, and time delay to process samples could prevent accurate
detection of bacteremia. There has been little research investigating the above factors using pediatric
blood culture bottles in the newer BacT/ALERT colorimetric devices. Through a two part preliminary
experiment in a community base hospital in India, we assess 1) the effect of time delay to processing due
to transport through an existing hospital system on known positive S. pneumoniae samples, 2) the effect
of a time delay of up to 24 hrs in detecting known positive S. pneumoniae samples of varying low
concentrations and dilutions.
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2D GEL MAPPING OF HUMAN BRAIN SAMPLES FROM VANISHING
WHITE MATTER (VWM) PATIENTS AND CONTROL PEDIATRIC
SUBJECTS
B Kulkarni A Vanderver Y Hathout K Brown A Takanohashi
Vanishing White Matter disease (VWM) is an autosomal recessive disorder affecting myelin growth and
maintenance. In most of the cases the neurological symptoms start in early childhood. The course of
disease is characterized by paroxysmal deterioration of neurological function after stressors such as
febrile infection and head trauma. Mutation in one of the five subunits of eukaryotic translation initiation
factor (EIF2B) is causative of the disease. Endoplasmic Reticulum (ER) stress and abnormal ER protein
trafficking play a role in pathogenesis, and are proposed to be responsible for paroxysmal attacks.
Proteomics is therefore a logical approach to study the molecular pathogenesis of the disease. This is the
first proteomics study where the 2D gel map of pediatric brain is studied in normal versus VWM patient
samples. Frozen autopsy brain samples from two mutation proven VWM patients and two age matched
control patients were separated into white matter and cortex sections and processed to extract proteins.
300 micrograms of proteins from each sample were then separated using two dimensional gel
electrophoresis. The proteins spots were then analyzed using in-gel trypsin digestion and 4700 proteomics
discovery systemв„ў by Applied BiosystemsВ®. In each gel, over 200 spots were visualized, and around
190 spots were identified. The interesting proteins include nervous system specific proteins such as
myelin basic proteins (MBP) and 2', 3'-cyclic-nucleotide 3'-phosphodiesterase (CNPase), ER related
proteins such as 78 kDa glucose-regulated protein precursor (GRP78) as well as proteins which are
known to play role in neurological diseases such as Alpha B Crystallin (multiple sclerosis), and
Glutamine synthetase (reactive gliosis) etc. We are planning to analyze more brain samples to
characterize remaining spots on the 2D gel map and analyze the differential expression of proteins
between VWM brains and healthy controls.
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A CROSS-SPECIES ANALYSIS OF PLATELET GENE EXPRESSION IN
SEPSIS SHOWS DYSREGULATION OF THE INNATE IMMUNE
CYTOKINES IL-1Гџ AND TNFa
AS Benton WM Ngor B Mojgani RJ Freishtat
Little is known about the role of platelets in sepsis such that insights can be gained by investigating
conserved platelet gene expression patterns across species. We aimed to identify evolutionarily conserved
novel gene products and networks using a genome-wide cross-species comparison analysis. We studied
two datasets of platelet mRNA in human and murine models of sepsis. In the murine dataset (n=3
microarrays) platelet mRNA was isolated from male BALB/c mice at 0, 24, and 48h status post cecal
ligation and puncture and profiled using Affymetrix MOE430_2 GeneChips. A second dataset of platelet
mRNA from septic children(n=6 microarrays) at 0 and 72h post diagnosis was profiled using Affymetrix
U133A GeneChips. Both datasets were processed through the dChip difference model probe set algorithm
to generate signals and imported into Genespring GX for analysis. We identified 2,381 probe sets
(p<0.05) showing differential regulation in the murine sepsis model. These probe sets were translated into
the human genome and used to generate a list of 247 significant probe sets (p<0.05) showing up- or
down-regulation in human sepsis. We clustered the 247 probe sets and by visual inspection found downregulation at 0h for two IL-1Гџ probe sets and TNFa in patients with severe sepsis versus those with milder
disease. Normalized expression values at 0h in severe sepsis were markedly lower than at any other time
point or severity state (all p<0.02). IL-1Гџ and TNFa are cytokines crucial to the innate immune response.
Failure of these genes to up-regulate early in severe sepsis may be due to a selective immune deficiency.
If this dysregulation is supported by ongoing studies, it will give new insight into the relatively poor
clinical course seen in some patients.
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CHROMATIN REMODELING OF THE MUC5AC PROMOTER DURING
DEXAMETHASONE-INDUCED GENE REPRESSION
YJ Chen AM Watson M Rahimi AM Colberg-Poley MC Rose
Dexamethasone (Dex), a glucocorticoid, is used clinically to treat exacerbations in asthmatic patients.
Mucin overproduction is a common outcome in airway diseases and the MUC5AC mucin gene is
upregulated in response to specific inflammatory mediators and is repressed by Dex. EMSA experiments
in our laboratory demonstrated that the Dex-activated glucocorticoid receptor (GR) binds to two
glucocorticoid response element (GRE) cis-elements in the MUC5AC promoter (Chen YA et al., Am. J.
Respir.Cell Mol. Biol. 34: 1-10, 2006). Functional analyses of MUC5AC promoter-luciferase plasmids
with mutations in the MUC5AC GRE3 and GRE5 cis-sites demonstrated that each GRE site
independently repressed MUC5AC gene expression following transfection into differentiated human
bronchial epithelial (HBE) cells. Chromatin immunoprecipitation (ChIP) experiments demonstrated that
GR and HDAC2 binds rapidly and transiently to the GRE3 and GRE5 cis-elements in the MUC5AC
promoter within 1h following Dex exposure. Western blot analyses showed that the nuclear abundance of
histone deacetylases (HDAC) HDAC1, HDAC2, HDAC3, and HDAC5 and the co-repressors NCoR,
Sin3A, SMRT and Mi2 are increased rapidly (0.5-6h) following Dex exposure of differentiated HBE
cells. These results suggest that following binding of GR to the GRE3 and GRE5 cis-sites in the
MUC5AC promoter, GR predictably recruits HDACs and/or co-repressors to the MUC5AC promoter for
chromatin remodeling. This is currently being evaluated by CHIP and re-ChIP experiments. Additionally,
Western analyses have shown significantly decreased levels of MUC5AC mucin in the apical secretions
of differentiated HBE cells following Dex exposure. These data support the relevance of mechanistic
investigations into chromatin remodeling at the MUC5AC promoter in airway epithelial cells following
Dex exposure to better understand the effect of Dex during asthmatic exacerbations.
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LARGE VARIATIONS IN MRNA AND PROTEIN EXPRESSION OF UREA
CYCLE GENES IN THE LIVER AND EXTRAHEPATIC TISSUES
49
H Datta Majumdar L Caldovic J Cabrera-Luque H Morizono M Tuchman
In mammals, the primary function of urea cycle is to protect the brain from toxic effects of ammonia by
conversion into urea in liver. Urea cycle enzymes are also expressed in intestines and kidneys. Northern
blots of mouse tissues showed N-acetylglutamate synthase expression in spleen, testis, and in the liver
and intestine. Western blotting confirmed presence of NAGS protein in all four tissues. Two prominent
NAGS bands were detected in the liver. NAGS-C was only species seen in the intestine, spleen and testis.
The expression of NAGS, carbamylphosphate synthase 1 and ornithine transcarbamylase mRNA was then
examined in mouse tissues using qPCR. NAGS expression was seen in kidney, ovary, spleen, testis and
stomach. CPS1 and OTC mRNA were highly expressed in the liver and intestine, but expression was also
observed in the testis and stomach. Western blotting was used to examine expression of the NAGS, CPSI
and OTC proteins in eight extra-hepatic tissues from four inbred strains of mice and the abundance of
these three proteins was found to be dependent on the genetic background of the mouse. Quantitative
Western blotting was used to measure abundance of NAGS-M and NAGS-C in the livers and intestines
from different mouse strains. The abundance of NAGS-M in liver was the highest in the FVB strain and
the lowest in the 129SSeM. The abundance of NAGS-C in liver and the ratio of NAGS-M to NAGS-C
varied with mouse strain. Variability of NAGS-C expression was also observed in intestines of different
strains. This suggests a natural variation in expression levels of urea cycle in liver as well as other tissues
that may partially be due to genetic background. Understanding this variability is important for a better
understanding of natural progression of urea cycle disorders, side effects of medications, and possible role
of urea cycle enzymes.
ANTIMICROBIALS THAT TARGET NOVEL MODES OF ARGININE
BIOSYNTHESIS
50
J Cabrera-Luque H Morizono
Stenotrophomonas maltophilia is an opportunistic pathogen found in soil and water that causes
respiratory, urinary and bloodstream infections in cystic fibrosis patients, immunocompromised cancer
patients and transplant recipients with an associated mortality of approximately 30%. Of particular
concern is that it shows a broad spectrum of antibiotic resistance. Unlike the majority of organisms
ranging from bacteria to plants to people, for which arginine biosynthesis has been studied, this organism
is unable to convert ornithine to citrulline directly. This enzymatic step is an essential part of microbial
arginine biosynthesis, and is normally catalyzed by ornithine transcarbamylase. S. maltophilia instead use
acetylornithine transcarbamylase to produce acetylcitrulline. The importance of arginine biosynthesis, in
combination with the specificity of this enzyme for its substrate makes this step an attractive target for
antimicrobial development. We have begun development of inhibitors of this novel transcarbamylase, and
have synthesized a candidate that is effective in the micromolar range against the purified enzyme. We
are currently developing methods to effectively deliver inhibitor into S. maltophilia with the hope that this
may lead to a strategy for treating S. maltophilia infection.
CHARACTERIZATION OF A TET-REPRESSIBLE MUSCLE-SPECIFIC
PITX1 TRANSGENIC MOUSE AS A MODEL OF FSHD
JF Cabotage M Dixit R Shi M Sutherland S Muger YW Chen
Facioscapulohumeral dystrophy is an autosomal dominant neuromuscular disorder, and is the third most
common form of inherited muscular disease. FSHD patients typically experience early atrophy of facial
muscles, atrophy of upper limb girdle muscles prior to the lower limb girdle muscles, and asymmetry in
effected muscles. A comparative profiling study on 12 neuromuscular disorders found that the gene Pitx1
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is specifically and significantly upregulated in FSHD (Dixit et al, 2007). To examine the effects of Pitx1
over expression, we generated a Pitx1 tet-repressible, muscle specific transgenic mouse line. Induced
Pitx1 expression in adult TRE-Pitx1/mCK-tTA mice resulted in a decrease of activity, loss of body
weight over time, reduced grip strength, and reduced weight of the gastrocnemius, tibialis anterior, vastus
lateralis, deltoid, biceps, and triceps muscles compared to control mice. There was no significant weight
difference between the soleus and diaphragm muscles of TRE-Pitx1/mCK-tTA and control mice. The
masseter muscle showed weight differences only among males, and not females. TRE-Pitx1/mCK-tTA
mice also exhibited pathohistological changes including large numbers of atrophic myofibers,
regenerating fibers, few necrotic myofibers and some cellular infiltration. This condition of muscle
atrophy and muscle weakness proved reversible as the re-repression of Pitx1 expression in TREPitx1/mCK-tTA mice resulted in increased activity and weight gain. Unlike human FSHD patients, TREPitx1/mCK-tTA mice appear to have no asymmetry in effected muscles, and facial muscles are only
effected in male mice. Further work needs to be done to determine the time at which atrophy begins to
occur in different muscle groups.
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EFFECT OF EPIGALLOCATECHIN-3 GALLATE(EGCG) AND
PIRFENIDONE ON MDX MOUSE PHENOTYPE
AA Sali
Effect of Epigallocatechin-3 gallate (EGCG) and Pirfenidone on mdx mouse phenotype Arpana Sali,
Daniel Guerron, Ying Cheng, Gouri Pandey, Nandita Saxena, Andreas Baudy, Ed Pistilli, Hee Jae Cha,
Christopher Spurney, Ramkishore Gernapudi, Viken Farajian, Eric Hoffman and Nagaraju Kanneboyina.
Absence of dystrophin results in muscle fiber degeneration/regeneration, inflammation and fibrosis
resulting in progressive muscle weakness in human Duchenne Muscular Dystrophy (DMD) patients. The
mdx mouse model of DMD reproduces some of the histological features of DMD. EGCG and Pirfenidone
are known to inhibit muscle inflammation and fibrosis respectively in several mouse models of human
disease. We have also shown in vitro that EGCG efficiently blocks TNF-alpha induced NF-kB activation
in C2C12 skeletal muscle cells. Therefore, 8-10 week old normal (BL10; n=8) and diseased (mdx; n=15)
mice were orally administered EGCG and Pirfenidone for 6 months. We have assessed for behavioral
(grip strength, rotarod and locomotor activity), imaging (echo), biochemical (CK assay) and histological
parameters (inflammation, fibrosis, degeneration and regeneration). There was a slight improvement in
rotarod function with both EGCG and Pirfenidone. In addition, Pirfenidone also showed an improvement
in grip strength as well as locomotor activity measurements. There was a trend towards decrease in heart
fibrosis both in EGCG and Pirfenidone treated mice. However, no significant differences were found in
serum creatine kinase levels. EGCG and Pirfenidone treated mice showed no undue side effects but
showed modest improvement in functional, imaging, behavioral and histological measurements.
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NEUROCOGNITIVE PROFILE IN A CASE OF MAPLE SYRUP URINE
DISEASE
53
KS Walsh MN Scott MM Berl
Maple Syrup Urine Disease (MSUD) is a metabolic disease with associated enzyme deficiency and an
inability to break down amino acids. Neurotoxic levels can occur resulting in neurological sequelae.
Information regarding cognitive functions has suggested greater verbal than nonverbal intellect. Specific
neuropsychological functions have not been studied. The objective of this study is to examine the
neurocognitive profile in a case of MSUD. This is a case study of a seven-year-old, African American
female who was diagnosed with MSUD and treatment was initiated within the first eight days of life. She
has imaging findings (edema, white matter abnormalities) common to the disease, and metabolic control
has been poor, with numerous crises. Basic language skills and verbal memory were below average, while
nonverbal skills and visual memory were consistently impaired. Fine motor coordination was impaired
although graphomotor control was adequate. Significant impairments in attention and executive
functioning were identified. Executive dysfunction was found to exacerbate visual-motor impairments,
but did not fully explain the difficulties with such tasks. The findings in this case identified particular
susceptibility to visual-motor, fine motor, attention, and executive function systems. Visual memory was
significantly impaired, and although verbal memory was below age expectations, it was a better modality
for learning. While language has generally been described as unimpaired in the literature, specific
weakness with verbal expression were identified in this case. These findings are generally consistent with
the literature, although the role of diagnosis age, neonatal course, leucine levels, and metabolic control in
neuropsychological outcomes remains unclear, which can be the focus of future research.
DIFFUSION TENSOR IMAGING DETECTS AREAS OF ABNORMAL
WHITE MATTER MICROSTRUCTURE IN PATIENTS WITH PARTIAL
ORNITHINE TRANSCARBAMYLASE DEFICIENCY (OTCD)
AL Gropman IL Kahn RR Seltzer J vanMeter
OTCD is the most common urea cycle disorder that hyperammonemia and substantial cognitive and
motor deficits. OTCD is regularly seen in hemizygous males and ranges in the degree of neurocognitive
impairment and age of onset. Neonatal OTCD survivors sustain brain injury with subsequent mental
retardation and cerebral palsy. Males with late onset OTCD, are not as severely affected, but show
deficits in executive function, motor planning and executive funtion. A broad phenotype is seen in
heterozygous females, with symptoms ranging from behavioral and learning disabilities, to protein
intolerance, stroke like episodes and hyperammonemic coma. A recent study of heterozygous females
found that although the women had average IQ scores, they displayed a specific neurobehavioral
phenotype consistent with a nonverbal learning disability, which is typically associated with white matter
or subcortical dysfunction. Previous autopsy and neuroimaging studies are also consistent with a pattern
of neuronal injury leading to white matter damage. However, the extent to which the deficits involve
specific pathways in the brain is unknown. We further examined the brain pathways involved in these
neurocognitive deficits, specifically, assessing and comparing microstructural white matter changes in
symptomatic heterozygous OTCD females, asymptomatic females and late onset hemizygous OTCD
males with age-matched controls. We hypothesized that with diffusion tensor imaging we will be able to
detect evidence of early white matter dysfunction in individuals with more subtle symptoms or metabolic
abnormalities. We also expected our OTCD subjects will exhibit differences in brain areas involved in
executive function and fine motor control, according to the number of hyperammonemic episodes and the
disease severity. The significance of this information is that it can open the doors to identifying
biomarkers for OTCD and help us better understand patients’ responses to therapy and the course of the
disease.
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VITAMIN D INSUFFICIENCY IN AFRICAN AMERICAN CHILDREN WITH
FOREARM FRACTURES
LM Ryan C Brandoli RJ Freishtat JL Wright EP Hoffman L Tosi JM Chamberlain
Half of all children fracture a bone; of those, nearly 40% sustain additional fractures. Fracture rates are
increasing. The goal of this study is to evaluate the hypothesis that pediatric fracture cases have an
increased prevalence of poor bone health compared to fracture-free controls. This case-control study is
enrolling cases (African American children, ages 5-9 years, with forearm fracture) and fracture-free
controls. Bone health evaluation includes measurement of serum 25-hydroxy vitamin D level and bone
mineral density (BMD) by dual energy xray absorptiometry (DXA) scan. Univariable and multivariable
analyses are used to test the associations between fracture status and the independent variables serum
vitamin D level and BMD with control for confounding variables. To date, we have enrolled 31 cases and
7 controls. Vitamin D levels are available for 23 cases and 4 controls. The mean 25-hydroxy vitamin D
level for cases is 21.5 + 7 ng/mL with a range of 10-38 ng/mL. This mean is at the cutpoint for vitamin D
insufficiency [serum 25-hydroxy vitamin D level < 20 ng/mL]. Ten cases (43%) were vitamin D
insufficient. The mean 25-hydroxy vitamin D level for controls is 29 + 3.3 ng/mL with a range of 24-31
ng/mL which is significantly higher (p=0.049). No controls were vitamin D insufficient. DXA scan
results are available for 27 cases and 5 controls and are consistent with normal bony mineralization for
age (z-score > -2) for all patients. These preliminary results show a significant proportion of African
American children with forearm fractures in our study are vitamin D insufficient. Mean serum vitamin D
levels are lower in fracture patients compared to controls. Analysis of vitamin D levels in additional cases
and controls will determine the significance of this finding. Vitamin D insufficiency may play a
previously unrecognized role in childhood fractures.
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SLEEP PROBLEMS AND THEIR RELATION TO COGNITIVE FACTORS,
ANXIETY, AND DEPRESSIVE SYMPTOMS IN CHILDREN AND
ADOLESCENTS
CA Alfano AH Zakem NM Costa LK Taylor CF Weems
Childhood sleep problems are common in the general population yet few data are available examining
unique relationships between sleep, anxiety, depression and cognitive factors in non-clinical samples of
youth. In addition, although it has been suggested that sleep disturbance may be more closely associated
with anxiety during childhood and with depression during adolescence (Dahl & Carskadon, 1995),
available data are somewhat conflicting. The current study examined these associations and relationships
among a large community sample (N=175) of children and adolescents ages 6-17 (Mean=11.4 years,
SD=3.4). Participants’ sociodemographic backgrounds were characteristic of the urban area from which
they were recruited (New Orleans, LA). Overall significant associations between sleep problems,
depression and anxiety were found, though depressive symptoms showed a stronger relationship with
sleep problems among adolescents (r=.58, p<.001) and anxiety symptoms were associated with sleep
problems in youth of all ages. Sleep problems were associated with several types of childhood anxiety.
Significant correlations with generalized anxiety (r=.34, p<.01), panic (r=.27, p<.05) and social anxiety
(r=.49, p<.01) only were found among adolescents. Cognitive factors, including cognitive errors and
control beliefs linked with anxiety and depression also were associated with sleep problems among
adolescents (r=.29, p<.01), though correlations were no longer significant after controlling for
internalizing symptoms. Findings are consistent with the suggestion that childhood sleep disturbance is
more closely associated with anxiety, while adolescent sleep problems are linked to depression. Results
also suggest links between sleep and anxiety to be non-specific in non-clinical samples of children,
though this association may become more explicit by the teenage years.
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DEVELOPMENT OF A QUANTITATIVE FUNCTIONAL STATUS SCALE
JT Berger MM Pollack JM Reardon P Glass
57
A problem for PICU outcome studies is the lack of a morbidity outcome with the following attributes:
functional status at a single time point, quantitative, unambiguous, rapid, objective and applicable to all
pediatric ages. Current methods are time consuming, and overly dependent on rater projections.
OBJECTIVE: Develop and validate a functional status measure analogous to activities of daily living
(ADL) used in adults. METHODS: We used the results of a single PICU pilot study (n=99) and a
consensus process (intensivists, neurologists, RNs, RTs, psychologists) to develop the FSS. The FSS has
6 clearly defined functional categories from normal to very severe dysfunction in the following domains:
mental status, sensory, communication, motor, feeding, respiratory. The study sites were PICUs from the
Collaborative Pediatric Critical Care Research Network. Patients were randomly selected from 3 groups
at high risk for disabilities: PICU discharges (40%), high risk hospital admissions (40%), and technology
dependent patients (20%). A research coordinator measured the FSS based on the patient’s status at that
moment in time. We correlated the FSS with adaptive behavior (Adaptive Behavior Assessment System
(ABAS) II) measured by the bedside nurse within 4 hours of the FSS assessment. Inter-rater reliability
using a weighted kappa (research coordinator and a MD) was assessed on ~10% of the sample.
RESULTS: The FSS on 839 patients from 7 sites (age mean 6.5 В± 5.4, median 5.0 yrs) ranged from 6 to
28 (10.7 В± 4.4). Correlations with ABAS II were > -0.575. Correlations of a re-weighted FSS with ABAS
II showed mild improvement. Inter-rater reliability was > 0.755 for all domains except mental status
(0.528). CONCLUSION: The FSS is well correlated with adaptive behavior and reliable. It meets the
criteria outlined above for a functional status measure appropriate for large PICU outcome studies.
COURAGEOUS CONVERSATIONS: FEASIBILITY & ACCEPTABILITY
OF FAMILY CENTERED ADVANCE CARE PLANNING
58
ME Lyon R McCarter L Briggs J Marsh J He L D'Angelo
The goals of the present study are to enhance quality of life and to integrate advance care planning
throughout the course of treatment for adolescents living with a life threatening illness, as well as to test
an intervention to support adolescents with a life threatening illness and their family members in order to
prevent depression and anxiety. More than 30,000 adolescents in the U. S. die annually from the effects of
all chronic illnesses (Mineno, et al., 2002). End-of-life (EOL) care for these patients is a public health
issue (Freyer, 2004; Rao, et al., 2002), because of its high burden and potential for the prevention of
suffering. Age appropriate communication that includes the child and family can prevent unwanted
interventions and facilitate a peaceful death. Specific Aims: (1) to test the feasibility and acceptance of a
culturally sensitive communication model, appropriate to the cognitive and emotional maturity of the
child, that involves him/her in decision making throughout a life threatening illness and death; (2) to test
in a randomized clinical trial the Family Centered Advance Care (ACP) Planning intervention with
adolescents living with HIV on hypothesized outcomes: optimal time to initiate end of life/advance care
planning discussions, decisional conflict, congruence in treatment preferences between adolescents and
families, and stage of illness as a marker of timing of satisfaction with timing of discussions. The Family
Centered ACP intervention curricula was developed with input from developmental specialists in the
field, community advisory group members, as well as patients and family. This pilot study was funded
through an R34 mechanism by the NIMH Office on AIDS.
PEDIATRIC NORMATIVE DATABASE IN BASAL GANGLIA MAGNETIC
RESONANCE SPECTROSCOPY
PB Peloquin S Fricke Z Khademian I Knerr P Khanna PL Pearl
In-vivo metabolite concentrations measured by proton magnetic resonance spectroscopy (MRS) provide
valuable diagnostic information. Diagnosis of neurometabolic disorders, such as creatine
synthesis/transport deficiency, requires detection of abnormal ratios of measured metabolites. The present
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study sought to develop a normative pediatric database from a retrospective analysis of MRS utilizing
basal ganglia voxel placement. A retrospective analysis was conducted of clinical and imaging data of
children who received MRS at our institution. Each patient was rated by a neurologist blinded to imaging
data, and neuroradiologist blinded to clinical data, for normal neurological status and structural imaging,
respectively. Peak heights of N-acetyl aspartate (NAA), creatine (Cre), choline (Cho), and NAA:Cre and
NAA:Cho ratios were determined using short and long echo time (TE=35ms) and long-echo (TE=288ms)
single voxel MRS. Short TE was 35 ms and long TE was adjusted to 288 ms for all studies. 137 children
with single voxel basal ganglia MRS were identified; 59 had both short and long TE studies available. Of
these, 23 (ages 4 mos to 21.2 y; mean 59 months) were classified as normal on clinical and radiographic
scales. NAA/Cre and Cho/Cre ratios were derived. This data may serve as a reference point for
interpretation of MRS.
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CLINICAL AND ECONOMIC IMPACT OF METHICILLIN-RESISTANT
STAPHYLOCOCCUS AUREUS ON NEONATES IN INTENSIVE CARE
UNITS
X Song T Taylor R Jamal B Short N Singh
Background: Rising incidence and mortality of MRSA in children has become a great concern. Objective:
To evaluate the association between MRSA infection or colonization and mortality, length of stay (LOS),
and charges in NICU patients. Method: A retrospective cohort study that included infants admitted to
NICU between 9/1/2004 and 12/31/2007. Patients colonized with MRSA were identified through active
surveillance culture or rapid PCR technique performed on nasal specimens collected at admission and
weekly thereafter, while MRSA infections were detected by clinical specimens. Results: This 3-year
study included 2053 infants (average age: 30 days). Of them, 170 (8.3%) had MRSA
infection/colonization. 58 (34%) developed =1 MRSA infections and 25 (43%) infants developed
invasive MRSA infections like bacteremia. Of 126 MRSA isolates for which antibiotic susceptibilities
were available, 101 (80.1%) were resistance to clindamycin. The mortality rate for MRSA patients was
18.8%, compared to 11.6% for non-MRSA patients. The multivariate Cox regression analysis revealed
that infants with MRSA infection or colonization had increased mortality risk (Hazard Ratio (HR) =1.1,
p>0.05), but the increase was not statistically significant. Overall LOS for MRSA patients was 53 days
(including 22 F/U days after the onset of MRSA), compared to 11 days for non-MRSA patients. The
multivariate median regression analysis showed that LOS after the acquisition of MRSA for MRSA
patients remained 5 days longer than the total LOS for non-MRSA patients (p<0.05). The median charges
for MRSA patients were $195,300 compared to $34,605 for non-MRSA patients. Conclusions: MRSA
infection/colonization in infants is associated with significant morbidity and financial burden, but not
with increased mortality. It is prudent to implement measures to prevent and control transmission of
MRSA in infant population.
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UNUSUAL MONITORING STRATEGIES DURING CRANIOSYNOSTOSIS
REPAIR IN A 15-MONTH OLD WITH COMPLEX HEART DISEASE
A Lee S Verghese C Sable R Keating
Monitoring for air embolism is of vital importance in patients undergoing neurosurgical procedures in the
sitting position. This is especially true during craniosynostosis repair which involves exposure of
noncompressible galeal veins and potential for significant blood looss. Escalation of intraoperative
monitoring may be necessary in small children with complex heart disease and inherent hemodynamic
instability undergoing such major surgery. We report the use of two infrequently used modalities of
monitoring: TEE and external debrillator / transcutaneous pacer in a 15 month old (9.5 kg) child with a
history with double outlet right ventricle s/p repair with an intracardiac baffle, recurrent bradycardia of
unknown etiology, hypothyroidism, and adrenal insufficiency undergoing total calvarial reconfiguration
for craniosynostosis.
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PHARMACOKINETIC-PHARMACODYNAMIC MODELING OF
MORPHINE IN PATIENTS WITH SICKLE CELL DISEASE: POTENTIAL
IMPLICATION FOR PAIN MANAGEMENT
62
DS Darbari M Neely J Finkel J Van den Anker S Rana
Morphine is frequently used in sickle cell disease (SCD) to treat pain, the most common reason for
hospitalization for patients with SCD. Adequate analgesia with standard doses of morphine remains a
major clinical challenge in many SCD patients. Despite a long history of its use, data on morphine
disposition in individuals with SCD is limited. It is not clear which general or SCD-associated factors
may contribute to variability in the pharmacokinetics (PK) and pharmacodynamics (PD) of morphine.
Morphine, an opioid analgesic agent is metabolized primarily in liver through conjugation with
glucuronic acid by the enzyme uridine diphosphate glycosyltransferase 2B7 (UGT2B7). Conjugation of
morphine leads to formation of two major metabolites, morphine-6-glucuronide (M6G) and morphine-3glucuronide (M3G) (Coffman 1997), which are then eliminated by glomerular filtration. In a study of
SCD children during vaso-occlusive crises, an increased clearance of morphine was reported but authors
were unable to conclude if this finding was due to acute changes associated with vaso-occlusive crises,
chronic pathological changes or genetic factors (Dampier 1995). We hypothesized that disposition of
morphine in SCD in steady state of health will be different from non-SCD individuals due to frequent
involvement of liver and kidneys in SCD. The current study was designed to determine pharmacokinetic
and pharmacodynamic parameters of morphine in young adults with SCD in a steady state of health to
avoid potential confounding factors that may contribute to the variability of morphine disposition.
IMMUNOHISTOCHEMICAL CONFIRMATION OF THE CUASITIVE ROLE
OF H. PYLORI IN SEVERE GASTRITIS IN CHILDREN
63
JD Snyder J Guarner YY Chen BD Gold S Czinn
Considerable concern has been raised about the ability of histology to identify all Helicobacter pylori (H.
pylori) infections, even when using modified silver stains. The possibility that staining techniques might
be able to identify especially small numbers of organisms has stimulated the search for more sensitive
diagnostic methods. We evaluated H. pylori-specific immunohistochemical (IHC) staining compared to
standard histologic techniques with silver staining and the rapid diagnostic CLO test to diagnose H. pylori
infection in selected pediatric patients.
NOVEL ROLE OF IRON AND IRON RELATED PROTEINS AS URINE
BIOMARKERS IN CHILDREN WITH HIV-ASSOCIATED RENAL
DISEASES
AA Soler-Garcia D Johnson Y Hathout PE Ray
Because of the risk of performing renal biopsies in HIV-infected children, we investigated the value of
urine protein biomarkers to identify children undergoing the early stages of HIV-nephropathy (HIVAN)
using proteomic methods. Nine urine samples from HIV-infected children with biopsy proven HIV-HUS
(n = 2), HIVAN (n = 4) or no renal disease (n = 3) were screened for protein biomarkers by twodimensional electrophoresis (2-DE). Samples were adjusted for protein/creatinine values and depleted of
albumin. Individual gel spots were cut and processed using MALDI-TOF/TOF MS. Proteins were
identified by GPS Explorer Software. Relevant findings were confirmed in HIV-infected children urines
with (n = 20) and without (n = 10) trace and proteinuria (>30 mg/dl protein) using commercially available
assays. By 2-DE, urines from children with HIV-HUS and HIVAN showed high levels of 11 proteins
previously identified and used by Varghese et al. (JASN 18: 913-922, 2007) to predict the diagnosis of
lupus nephritis, FSGS, membranous, and diabetic nephropathy in HIV-negative patients. These proteins
are: orosomucoid, transferrin, alfa-1-microglobulin, zinc alfa-2-glycoprotein, alfa-1-antitrypsin,
complement factor B, haptoglobin, transthyretin, plasma retinol binding protein, albumin, and hemopexin.
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However, a single marker was not sufficient to distinguish children with HIV-HUS from those with
HIVAN. Because of the presence of many iron related proteins, we assessed urinary levels of iron and
iron related proteins in these children. We found high levels of transferrin, hemopexin, haptoglobin,
lactoferrin, and NGAL in children with HIV-HUS or HIVAN when compared to HIV-infected controls.
Iron and iron related proteins might be promising candidate urine biomarkers to identify HIV-infected
children at risk of developing HIVAN and HIV-HUS. These findings suggest for the first time that the
prevention of renal iron accumulation and/or toxicity should be considered as an important goal in the
treatment of children with HIV-associated renal diseases.
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PSYCHIATRIC PROFILES OF PRESURGICAL PEDIATRIC EPILEPSY
PATIENTS
JA Salpekar L Foster S Cushner-Weinstein A Yaun T Tsuchida C Heath M Berl WD Gaillard
Patients with intractable seizures are believed to have a high degree of psychiatric comorbidity; however,
few studies have been done with high subject numbers or sophisticated psychiatric assessment. The study
aimed to characterize psychiatric illness in this population, and to ascertain whether specific seizure
localization was associated with higher psychiatric comorbidity. A retrospective chart review was done in
a tertiary care pediatric hospital where surgical procedures for epilepsy are commonly performed. Each
patient received a full evaluation by a child psychiatrist specializing in neuropsychiatric issues, and
parents completed a Child Behavior Checklist (CBCL) prior to their surgical procedure. Group
comparisons were made based upon seizure localization and associated psychiatric diagnoses or CBCL
subcategory t-scores of 65 or higher. 43 pediatric patients were identified who had undergone presurgical
evaluation. 22 patients had seizure foci restricted to the temporal lobe (T), and 21 had seizure foci outside
of the temporal lobe (EX-T). Both groups had a high amount of comorbid psychiatric illness. The most
common comorbidities were ADHD (8 T vs. 8 EX-T), Anxiety (7 T vs. 6 EX-T), and depression (7 T vs.
1 EX-T). Depression was more common in patients with temporal lobe seizure foci (p=0.046). Elevated
CBCL subcategory scores were high in both groups, although consistently higher in patients with
temporal lobe seizure foci. Depression-specific CBCL categories (anxious, withdrawn) were higher in
patients with temporal lobe seizure foci (p=0.045). Psychiatric comorbidity is very common in patients
with intractable seizures. CBCL scores indicated significantly higher and more severe behavior problems
in patients with temporal lobe seizure foci. Psychiatric diagnoses of Anxiety and ADHD were present in
both groups equally; however, depression was significantly more common in the temporal lobe group.
Overall, psychiatric illness, especially depression, may be more common in pediatric patients with
temporal lobe seizure foci.
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MASSAGE AND KINESTHETIC STIMULATION (EXERCISE) IMPROVES
WEIGHT GAIN IN VERY LOW BIRTH WEIGHT (VLBW) PRETERM
INFANTS
AN Massaro TA Hammad A Olaode B Jazzo H Aly
Preliminary studies have suggested that massage therapy and kinesthetic stimulation (exercise) have
positive effects on outcomes of premature neonates. It remains unclear if benefits are attributable to
massage alone or the combination of massage and exercise. We report results of a randomized clinical
trail evaluating the effects of massage with or without kinesthetic stimulation on very low birth weight
infants.
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INCIDENT REPORTS AND MEDICAL ERRORS IN PEDIATRIC
EMERGENCY DEPARTMENTS
67
JM Chamberlain
Critical incident reporting has been used successfully to improve safety in high-risk industries such as
aviation and nuclear energy. Incident reporting is widely used in United States hospitals but has not been
an effective mechanism for reporting safety events. Most importantly, lessons learned from one hospital
are not shared with others. Failure to disseminate this safety information to the medical community is a
wasted opportunity for shared learning and improvement of systems to prevent future errors. Uncommon
events may not appear in the data sets of even very large institutions. The objectives of the current study
was to establish a confidential network incident reporting system to describe and quantify medical errors
reported within hospitals in the Pediatric Emergency Care Applied Research Network (PECARN).
THE ROLE OF MIDDLE FRONTAL GYRUS IN VERBAL WORKING
MEMORY
68
MM Berl J Mayo B Yerys L Rosenberger L Yarger W Gaillard
Language processing involves core language skills (vocabulary, grammar) as well as non-linguistic
aspects (attention, working memory). Functional imaging may be one method of parceling out which
factors contribute to impairments in processing. This study aims to pilot a novel verbal working memory
task within the functional MRI environment to determine the functional signature of working memory
processes in language tasks. It is hypothesized that middle functional gyrus activation will vary with
working memory demands and will be correlated with neuropsychological measures.
NEUROPSYCHOLOGICAL FUNCTIONING IN INDIVIDUALS WITH
UREA CYCLE DISORDERS
69
LS Krivitzky M Berl J Mayo
Urea Cycle Disorders (UCD) refers to a group of genetic disorders in which there is a deficiency of one of
the enzymes in the urea cycle, which is responsible for removing ammonia (a highly toxic substance)
from the blood stream. Previous research samples have been small and have noted a variety of cognitive
outcomes. The goal of this study is to present some preliminary data from a larger, longitudinal, multi-site
study of individuals with UCD. Within this small sample, approximately one quarter presented with
mental retardation. In the remaining group, the neuropsychological profile suggested evidence of intact
skills in the area of verbal skills/crystallized knowledge. In contrast, relative weaknesses were noted in
nonverbal, complex and integrative skills. These results suggest possible dysfunction in the righthemisphere and/or white matter pathways. Further research is warranted to study more individuals with
UCD and to examine the relationship between markers of disease severity and cognitive functioning.
INCIDENCE OF PARENTERAL NUTRITION-ASSOCIATED
CHOLESTASIS IN THE NEONATAL INTENSIVE CARE UNIT AND
CHARACTERISTICS OF INFANTS
CJ Klein L Scavo M Revenis C Torres R Jamal C Kusenda
Sustained use of total parenteral nutrition (TPN) often results in parenteral nutrition-associated cholestasis
(PNAC). PNAC results in significant morbidity and mortality but its incidence at Children’s National
Medical Center is not known. A retrospective study was conducted to estimate the incidence and identify
characteristics of PNAC in the Neonatal Intensive Care Unit (NICU). This study was approved by the
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Institutional Review Board. The Neonatal Information System 5 database was searched using terms of atrisk diagnoses including gestational age < 28 weeks at birth to identify patients at risk for PNAC
between July 1, 2006 and June 30th, 2007. Patients with blood direct bilirubin > 3 mg/dL and duration of
TPN > 21 days were considered high-risk for PNAC. Of 678 NICU admissions, 46 cases (3 to 4 per
month) met criteria for high-risk of PNAC. The male to female ratio was almost 2:1.The cases were
ethnically diverse (60% Black, 26% Hispanic, 9% White, 5% Other). The age of admission was
(meanВ±SD) 11В±20 days of life and one-half (52%) of infants were admitted on the day of birth. The
majority (69%) weighed < 1000 g at birth. The median (range) use of TPN was 64 (22 - 168) days. The
median (range) for maximum direct bilirubin was 11.9 (3.2 - 24.8) mg/dL. Nine infants did not survive
(20.9% mortality; n=43). One additional infant expired while waiting for a donor organ after being
discharged. The length of stay was similar for survivors and those who expired, and averaged 85В±43 days.
This study confirms that NICU infants at risk for PNAC have relatively long lengths of stay, are
dependent on TPN for prolonged periods, and have substantial mortality (21%). Attempts to identify the
components of TPN that are causative are well justified.
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PSYCHOMETRIC PROPERTIES OF THE PARENT AND TEACHER
POST-CONCUSSION SYMPTOMS INVENTORY (PCSI) FOR CHILDREN
AND ADOLESCENTS
GA Gioia JA Janusz PK Isquith D Vincent
Full assessment of the functional issues following pediatric mild TBI requires the input of multiple
individuals. We investigate the normative development of the multi-informant Post-Concussion Symptom
Inventory (PCSI), hypothesizing low base rates and a significant relationship between parent and teacher
reporters. Parents (n=490), teachers (n=67), and children (age 5-12 n=213, age 13-18, n=208)
participating in a normative study completed the appropriate version of the PCSI (0-6 Likert scale). Base
rate frequencies of any level of symptom ratings (“1” or higher) were generated for each symptom and
ranked in order for each scale. The base rate levels of symptom reports by parents and teachers in the
normative population are generally low (Parent PCSI total score mean= 2.2, SD=4.8; Teacher mean =5.7,
SD=8.4). Reports of the cognitive symptoms were relatively higher for teachers. No significant
differences were found in parent or teacher ratings of child symptoms across the 5-7, 8-12, or 13-18 year
age groups. Spearman rho rank order correlations between the parent, teacher, child, and adolescent forms
revealed significant relationships (Parent-Teacher r=0.527,p=.006; Parent-Child5-12 r=0.519, p=.016;
Parent-Teen13-18 r=0.453,p=.02; Teacher-Child5-12 r=0.474,p=.03; Teacher-Teen13-18 r=0.404,p=04).
Base rate levels of symptom reports by parents and teachers are generally low. The parent, teacher, and
child/adolescent scales are moderately correlated, indicating a reasonable relationship among the raters
yet unique input as well. The inclusion of reports of symptoms in the home and school settings by parents
and teachers, respectively, will assist in the ecological treatment of concussion in the child and
adolescents’ real-world settings.
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DOCUMENTING RECOVERY TRAJECTORIES IN CHILD/ADOLESCENT
CONCUSSION
MS Shapiro GG Gioia
Assessment of the impact of concussion in children and adolescents includes both symptom survey and
neurocognitive assessment. Computerized measures of neuropsychological functioning can be useful in
detecting subtle improvements in neurocognitive functioning over time. The objective of this paper is to
examine the utility of a battery of computerized measures of memory, reaction time and processing speed
in capturing the nature and trajectory of the recovery process of childrne and adolescents who have
sustained a concussion. After accounting for variance in outcome due to the effects of time (linear and
quadratic change), we developed growth curve models accounting for significant proportions of
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remaining variance in ImPACT scores. Improvements over time were fairly linear. Gross categories of
cause of injury accounted for far more variance in outcome over time than such traditional factors as
presence of LOC, RTA, and/or ATA. Baseline symptom reports explained only minimal variance in
outcome scores. Results indicated that a multi-dimensional model that includes both symptom report and
computerized neuropsychological evaluation provides a comprehensive analysis of recovery trajetories
after child and adolescent concussion.
A TALE OF TWO BRIDGES: EFFECT OF THE BLOODLESS BRIDGE
ON BLOOD PRESSURE IN NEONATES MANAGED WITH VENOARTERIAL EXTRACORPOREAL MEMBRANE OXYGENATION
73
K Rais-Bahrami AN Massaro A Williams K Rais-Bahrami BL Short
We previously demonstrated significant effects on renal blood flow and blood pressure associated with
clamping and unclamping the traditional “blood-filled” ECMO bridge in newborn lambs. Following this
discovery, most ECMO centers transitioned to a circuit with a “bloodless” bridge that did not require
intermittent circulation. The aim of this study was to assess whether or not this change was associated
with improved renal function and lower incidence of hypertension in neonates requiring VA ECMO.
PRACTICE PATTERN VARIATION IN HEAD CT USE IN CHILDREN
WITH MINOR BLUNT HEAD TRAUMA IN THE ED: IS THERE AN
ASSOCIATION WITH PHYSICIAN TRAINING?
74
SM Atabaki R Stanley J Hoyle P Dayan S Atabaki L Lee et al. and the PECARN TBI Group
Background: The decision to order a head CT scan in the evaluation of children with minor blunt head
trauma (BHT) in the ED may be associated with physician, patient and environmental factors. Objective:
To determine if CT scan use in children with minor BHT is associated with type of attending physician
training. Design/Methods: We prospectively enrolled children < 18 years with BHT presenting to 25 EDs
in the PECARN network. The training of the treating attending physician was recorded as Pediatric
Emergency Medicine (PEM) subspecialty, Emergency Medicine (EM) only, Pediatrics and Emergency
Medicine (Peds and EM), Pediatrics only, or Other. Examining only patients with Glasgow Coma Scale
(GCS) of 14 or 15 with no significant underlying illnesses, we used multivariable logistic regression to
assess association between physician training and decision to obtain CT, while controlling for patient
factors (severity of head trauma as assessed by GCS and other signs of altered mental status, severity of
mechanism of injury, and age), and environmental factors (hospital location, ED pediatric volume,
teaching hospital). Our analysis excluded physicians with non EM or PEM training, as their role in the
ED may be different. Results: Of 57,158 eligible patients, 43,995 (77%) were enrolled, of whom 42, 517
(97%) had GCS 14-15 and no significant underlying illnesses. Of the 42,517, 14,881 (35%) underwent
CT scans in the ED; 774 scans (5%) were positive for traumatic brain injury. Physicians with PEM
subspecialty training were less likely to order head CTs than those with EM training, or Peds and EM
training. Conclusions: Important variation exists in CT use for evaluation of children with BHT by
physicians with different training. This variation will need to be accounted for in efforts to optimize CT
use.
OUTCOMES OF PATIENTS WITH NEONATAL ENCEPHALOPATHY
TREATED WITH WHOLE BODY HYPOTHERMIA
T Chang AN Massaro S Baumgart P Glass T Chang T Tsuchida BL Short
Whole body hypothermia has been shown to be protective against death and disability in patients with
moderate to severe neonatal encephalopathy (NE) and has recently been adopted as standard of care in
our institution. We report early neurological outcomes of patients with NE treated with a new
hypothermia program and explore if early clinical predictors can differentiate patients with adverse
neurological outcome.
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76
RECOMMENDED DOSE OF LOPINAVIR / RITONAVIR IS SUB-OPTIMAL
IN PROTEASE INHIBITOR-EXPERIENCED CHILDREN
N Rakhmanina J Van den Anker M Van Guilder S Soldin K Williams A Baghdassarian M Neely
Lopinavir/ritonavir (LPV/rtv, Kaletra®) is approved for use in children ≥6 months of age at a dose of 230
mg/m2 twice daily. In adult PI-experienced patients, trough LPV concentration of <5.7 mg/L has been
associated with failure of viral suppression. The aim of this study was to determine whether this target is
relevant in children, and achievable at the recommended pediatric dose. Over 52 weeks, prospective data
were collected from 50 HIV-infected children (4-17y) receiving LPV/rtv based therapy. Drug
susceptibility, HIV RNA viral load (VL) and adherence (self/family report) were measured at each study
visit. 12 hours PK blood samples were obtained after observed LPV intake, and LPV concentrations were
measured by a published, validated tandem-mass spectrometric method. By multiple logistic regression,
trough LPV concentration, adherence and resistance were modeled as predictors of virologic outcome
(SAS). PK data were fitted to candidate PK models (USC*PACK software), and the model with the
highest log-likelihood was used to simulate 5000 children (ADAPT 5 software) to determine the
percentage with trough LPV concentration <5.7 mg/L. LPV resistance (p=0.003) and trough
concentrations <5.7 (p=0.03) were significant predictors of never achieving VL <400 copies/ml during
the study period. Adherence was not a significant predictor of virologic outcome. The data from 35
patients was used to develop the PK model, while 15 patients validated the model. The regression line of
observed vs. predicted LPV had an R2 of 0.98, a slope of 1.00, and an intercept of 0.08. LPV trough was
<5.7 in 40% of the 5000 children simulated from this model. LPV trough of <5.7 mg/L was significantly
associated with sub-optimal virologic response, independent of viral resistance and patient adherence. In
this validated pediatric population PK model, the currently recommended dose of LPV will fail to
consistently achieve this target in a large percentage of children.
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HUMAN PARECHOVIRUS-3 INFECTION EMERGING PATHOGEN IN
NEONATAL SEPSIS
77
RE Levorson BA Jantausch BL Wiedermann HM Spiegel JM Campos
We report the first cases of neonatal infection with Human parechovirus-3 (HPeV-3) in the United States
(US). Human parechoviruses (HPeV 1-6) are members of the Picornaviridae family, with HPeV-1 and -2
previously classified as Echoviruses 22 and 23. Since identification of HPeV-3 in 2004, it has been
associated with neonatal sepsis, respiratory, GI, and neurologic disease outside of the US. HPeV are not
detected by standard enteroviral (EV) PCR assays. We present clinical details of 2 neonates with HPeV-3
infection, admitted to Children’s National Medical Center for evaluation of persistent fever and potential
sepsis: (1) A 13 day old male with fever and diarrhea less than 24 hours prior to presentation who
developed meningoencephalitis (rigidity, irritability, and somnolence), thrombocytopenia and
transaminitis. Brain MRI revealed bilateral frontal T1-hyperintense white matter lesions. (2) A 25 day old
male presented with fever and subsequently developed hepatitis, coagulopathy and thrombocytopenia. In
both infants, blood, urine, and CSF bacterial cultures, CSF HSV and EV PCRs, and mucosal HSV and
EV cultures were negative. All clinical and laboratory abnormalities resolved in both infants by hospital
discharge. Subsequent analysis of CSF from both patients at the Centers for Disease Control and
Prevention Enteroviral Laboratory yielded positive PCR for HPeV-3. This is the first case series of
neonates with confirmed HPeV-3 infection in the United States, and the first detailed reports of HPeV-3
associated hepatitis-coagulopathy and meningoencephalitis including description of neuroimaging
abnormalities. Clinicians evaluating neonates presenting with fever/sepsis should be aware of this
emerging pathogen. Although clinical manifestations may be similar to those seen in the setting of EV
disease, standard EV PCR testing is routinely negative, and specific diagnostic testing for HPeV-3 is
required.
PARENTAL SUPPORT AND EXERCISE-RELATED CONFLICT AND
REPORTED EXERCISE IN PREADOLESCENTS WITH TYPE 1
DIABETES
E Mackey J Shepard R Streisand
Physical activity is important in the management of type 1 diabetes, as is associated with better metabolic
control and decreased risk of obesity. However, pre-adolescents are at risk for decreased self-care
behaviors, including a decline in physical activity, during school-age years. Research from the general
population indicates that parental support can influence their children’s physical activity. As part of an
ongoing prospective RCT to promote adherence among preadolescents with type 1 diabetes, the current
study assessed the relationship of parental support, conflict, and child physical activity at baseline using
data from parent and child. Participants were 86 preadolescent-parent dyads (45 females, age 9-12 years,
M age = 10.8 years, 93% mothers; 73% Caucasian). Physical items were culled from parent and child
completion of the Diabetes Family Behavior Scale, Diabetes Related Conflict Scale, and Self-Care
Inventory. Children also completed physical activity items from the CDC’s Youth Risk Behavior
Surveillance System. Physical activity was further measured via 24 hour disease care recall interviews
(parents and children) assessing total minutes of activity the previous day. A structural equation model
was evaluated predicting physical activity from parent- and child- reported conflict and support around
physical activity.The model demonstrated excellent fit with the data (X2 = 56.68 (51), p = .21, CFI = .97,
RMSEA = .04, SRMR = .07). Findings suggest that parental support for physical activity is related to
more physical activity, whereas parental conflict around physical activity is related to less physical
activity in preadolescents with type 1 diabetes. The current study is important in that it examines the
relationship between parental support and conflict and one aspect of adherence in youth with type 1
diabetes. Possible applications of the findings include informing intervention efforts to help parents
encourage their children to engage in physical activity and to minimize related conflict.
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79
TELEVISION AND VIDEO EXPOSURE IN YOUNG CHILDREN AT RISK
FOR DEVELOPMENTAL DELAY
G Martin P Glass
In spite of the American Academy of Pediatrics recommendation that no children under age 2 be exposed
to television or electronic screens, 64-100% of all infants and toddlers watch 1-2 hours of television
before the age of 2 (Zimmerman, Christakis, & Meltzoff, 2007). No research has shown tangible benefits
of television exposure on children. In fact, research has found that exposure to television/videos is
associated with attention problems in young children, with language delay, and with fewer parent-child
interactions (Anderson & Pempek, 2005). Children with developmental disorders are a particualrly
vulnerable population and the amount of television/videos that these children are exposed to has not been
systematically documented. The aim of the present study was to investigate the type and frequency of
media exposure in children with developmental disorders and how that might relate to the child’s
developmental level. All parents/caregivers who attended a developmental evaluation with their child
(between the ages of 1 month-4 years) at the Developmental Clinic over a two-month period were invited
to participate in the study by completing an anonymous, brief questionnaire. Regardless of their
participation in the study, all parents/caregivers were provided an age-appropriate informational hand-out
about television/video watching in young children.
80
DEVELOPMENT OF SLEEP WAKE CYCLING AND PRESENCE OF
SEIZURES ON aEEG DURING WHOLE BODY HYPOTHERMIA
M El-Dib T Tsuchida A Massaro T John R Bernier S Baumgart B Short T Chang
Amplitude-integrated EEG (aEEG) has been used as an inclusion criterion in therapeutic hypothermia;
however, neither continuous EEG nor aEEG during hypothermia has been examined. The goal of this
study is to evaluate the progression of aEEG background, sleep wake cycling (SWC) and seizures in
newborns with neonatal encephalopathy receiving whole-body therapeutic hypothermia. Therapeutic
cooling for 72 hours was performed in 56 infants with neonatal encephalopathy. Continuous video EEG
was monitored. To date, 232 segments of two-channel aEEG from 34/56 treated infants have been blindly
evaluated. Fifty Six neonates (mean weight 3.35В±0.69 kg, gestation 38.6В±1.8 wks) were admitted at
3.48В±0.05 hours of life for hypothermia. Cooling was initiated at mean 4.31В±0.05 hours of life and EEG
monitoring at a mean 12.3 hours. Eight deaths occurred (14.3%). Background in 1st hour of recording
(n=34) showed flat tracing (FT) in 15%, burst suppression (BS) in 21%, low voltage (LV) in 15%,
discontinuity (D) in 9% and borderline normal (BLN) to normal (N) tracing in 27%. Background could
not be evaluated due to poor quality of recordings in 2 patients and status epilepticus in 3 patients. After
rewarming (80-92 hrs of cooling) (n=25), background was FT in none of the segments, BS in 16%, LV in
48%, D in 20 % and BLN to N in 16%. SWC showed progressive improvement during cooling. SWC
were clearly identified in 3.4% in day of life 1 (DOL1), 20.7% in DOL2, 35.5% in DOL3 and 55.6%
while rewarming. Changes in background pattern and development of SWC on aEEG may be useful
monitor during therapeutic hypothermia. EEG is necessary to confirm a clinical seizure, while aEEG may
over detect seizures. Earlier EEG monitoring is necessary to confirm whether reported clinical activities
are seizures or brainstem activity. Continuous EEG and aEEG are helpful tools for monitoring during
hypothermia.
81
SLEEP DISRUPTION IN PARENTS OF YOUNG CHILDREN WITH TYPE
1 DIABETES
MC Monaghan SS Berger D Lewin R Streisand
Parents of young children with type 1 diabetes assume the majority of responsibility for diabetes care and
frequently report increased caregiver stress. Although research indicates parents of children with chronic
illnesses experience significant sleep disruption, few studies have examined parent sleep in relation to
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diabetes management. The goal of this pilot study is to investigate parent sleep disruption related to
diabetes care regimens of young children. Twenty-four parents (88% female) completed parent-report
measures about diabetes regimen and child and parent sleep quality. Children (N=24; M age= 4.1 years;
50% female; 75% Caucasian) were in adequate metabolic control (M HbA1c=7.87%). The sample was
split between children on conventional insulin regimens (2-3 injections/day; n=11) and more intensive
insulin regimens (multiple daily injections (MDI); n=13). Child age and HbA1c did not significantly
differ by regimen. Parents reported an average of 6.3 blood glucose (BG) checks per 24-hour period;
children on MDI received significantly more BG checks (conventional M=5.27; MDI M=7.15; p=.05). Of
parents in the MDI group, 85% reported checking BG levels after their child went to sleep a few nights
per week, compared to 27% of parents of children on conventional regimens (p=.05). All parents of
children on MDI reported that diabetes disrupted their sleep sometimes (54%) or often (46%), compared
to 50% of parents of children on conventional regimens (sometimes=36%; often=14%; p=.01). MDI
regimen was related to increased child sleep disruption and increased parent-reported bedtime stress.
Parents slept for an average of 6.57 hours per night. Results suggest that a significant number of parents
of young children with type 1 diabetes experience sleep disruption and, further, having a child on a MDI
regimen appears to cause increased sleep disruption. Evaluating and making specific recommendations to
improve parent and child sleep is an important component of psychosocial interventions for this
population.
EXAMINING DEVELOPMENTAL NEUROPSYCHOLOGICAL
TRAJECTORIES IN CHILDREN WITH NEUROFIBROMATOSIS TYPE 1
(NF1)
82
PJ Duquette KS Walsh T Bouton MB Shapiro C Tifft K Rosenbaum GA Gioia M Acosta
Previous research has shown that children with NF1 are at risk for various neurocognitive problems,
including learning disabilities, visual-spatial deficits, and attention/executive dysfunction. However, few
studies have utilized a time-series design to identify early markers of impairment. The goal of the present
study was to determine the developmental trajectory in the neuropsychological phenotype of pediatric
NF1. Results indicated no significant difference in global cognitive functioning between early
developmental (M = 90.56, SD = 23.65) and more recent (M = 90.78, SD = 17.02) cognitive assessments.
Results from current neuropsychological evaluations indicated weaknesses in auditory/divided attention
(35–55%), executive functions (35%), planning/organization (48%) and working memory (25%),
visuospatial (50%) and visuomotor skills (39%), as well as verbal/nonverbal learning and memory (2539%). Findings highlight the stability and predictability of global cognitive functioning in children with
NF1. Future research should attempt to determine the stability of specific neuropsychological functions.
Understanding the trajectories of neuropsychological development in patients with NF1 will help to
clarify the implications of the lack of neurofibromin on brain development.
IMPLEMENTATION OF A REVISED HYPERGLYCEMIA MANAGEMENT
PROTOCOL FOR BETTER CONTROL OF BLOOD GLUCOSE IN NONDIABETIC, CRITICALLY ILL NEONATAL PATIENTS
L Pinchevsky S Eades N Menda
It is unknown if tight glucose control will lead to a reduction in morbidity and mortality in neonatal
critically ill patients. Our guidelines to manage hyperglycemia were revised in our neonatal intensive care
unit (NICU) in January 2008 to incorporate dosing of continuous insulin infusions by units/kg/hr versus
ml/hr and include titration parameters designed to maintain target blood glucose values of 60 to 150
mg/dL. The purpose of the study is to determine if the revised hyperglycemia management protocol
decreases the time to reach target blood glucose levels and decreases the number of hypo- and
hyperglycemic events in our NICU population.
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84
UTILITY OF AUTOMATED TRIGGERS ON ADVERSE EVENT
DETECTION IN A PEDIATRIC HOSPITAL
M Chehab D Stockwell Y Mulugeta S Eades B Jacobs
Existing methods of detecting adverse events in our institution include voluntary incident reporting,
morbidity and mortality conferences, and root cause analyses following sentinel events. It is estimated
that these methods capture approximately 5% of all events that occur within a hospital. Using electronic
“triggers” in existing databases may improve adverse event detection. Electronic triggers include the
administration of antidote-type medications and the detection of certain aberrant laboratory values. A list
of electronic triggers that are commonly linked to potential adverse events are monitored as part of a
quality assurance initiative within our institution. The objective of this study is to determine the utility of
these electronic triggers in adverse event detection in comparison to traditional reporting systems.
85
PARENT AND SELF-REPORTED CHANGES OF EMOTIONAL
FUNCTIONING POST CONCUSSION
JC Schneider GA Gioia B Elliott O Milgrom
Emotional dysfunction is common following concussion, although the exact mechanisms (e.g., temporary
brain compromise, secondary reaction to the injury) are currently unknown. This investigation examines
changes in emotional functioning early post-injury in adolescents who sustained concussions.
Adolescents (ages 12-18, mean=13 years), diagnosed with concussions, and their parents, presenting to a
concussion clinic, completed retrospective pre-injury and post-injury (median days post-injury=10) forms
of the Child Behavior Checklist (CBCL) (n=34), Youth Self Report (YSR) (n=28), and Profile of Mood
States-Adolescent (POMS-A) (n=11). Adolescents and parents report increased emotional dysfunction
early post-injury compared to retrospective pre-injury ratings with parents reporting greater change in
post-injury emotional functioning than adolescent’s ratings. Parent report a significant increase in
emotional and behavioral functioning on the CBLC Withdrawn/Depressed Scale (F=6.471, p=0.016),
Attention Problems Scale (F=7.241, p=0.011), and Aggressive Behavior Scale (F=5.200, p=0.029).
Similarly, on the POMS-A, parents report significant increases in their child’s emotional states on the
Confusion (F=7.932, p=0.017), Fatigue (F=9.707, p=0.010), Depression (F=5.500, p=0.039), and Total
Mood Disturbance scales (F=9.171, p=0.011), with declines on the Vigor scale (F=11.477, p=0.006).
Compared to pre-injury ratings, early post-injury self-ratings revealed significant increases on the YSR
Attention Problems scale (F=11.800, p=0.002) and significant declines on the POMS-A Vigor scale
(F=9.289, p=0.012). The present study revealed increased adolescent emotional and behavioral
dysfunction and declines in energy early post-injury, compared to retrospective pre-injury emotional
states. These findings have important implications for the adolescent’s recovery. Assessment of emotional
functioning post-injury should be part of the post-concussion evaluation to focus relevant interventions.
86
GENDER DIFFERENCES IN RECOVERY FROM POST-CONCUSSION
FATIGUE
BM Elliott R Fried JC Schneider GA Gioia PK Isquith
Males and females differ in their recovery from concussion. Fatigue is an important and common postconcussive symptom that can manifest in different ways. The present study investigated gender
differences in recovery of fatigue symptoms. 23 male and 11 females ages 13-18 (Mean age=15.28,SD
=1.5) completed the 18-item PEDS-QL Multidimensional Fatigue Questionnaire (Acute version) during 3
serial visits to a concussion clinic. At the first visit, participants also complete a retrospective baseline
version of the PEDS-QL. Sleep/Rest, Cognitive, and General fatigue scales were examined. Repeated
measures MANOVAs were conducted to examine changes in symptoms over time. At visit 1, males and
females report higher levels of fatigue than baseline. Females report significant cognitive (F (1, 10)=6.71,
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p<.05), sleep (F (1, 10)=11.70, p <.05), and general (F (1, 10)=3.93, p=.079) fatigue symptoms at visit 2,
and sleep symptoms at visit three (F(1, 10)=10.84, p<.05). Males report general fatigue and cognitive
symptoms to have returned to baseline by visit 3; sleep symptoms returned to baseline at visit 2, but then
reemerged at visit three (F (1, 33)=4.96, p<.05). Male and female adolescents experience significant postconcussive fatigue relative to baseline. While males report a more quick recovery from cognitive and
general fatigue, recovery from sleep symptoms appears to fluctuate. Females, in contrast, persisted in
their experience of fatigue symptoms longer than males, with sleep appearing to last the longest. Further
examination is necessary to determine factors that might explain these gender differences.
EXAMINATION OF THE EVERYDAY SITUATIONS SURVEY (ESS) IN
CHILDREN AND ADOLESCENTS
87
C Wells JA Janusz GA Gioia D Vincent D Goldberg
Following a concussion, it is important to assess not only the presence of a child’s symptoms but also the
extent of their manifestation in the home and school settings. The Everyday Situations Survey (ESS) was
developed to assess problems with memory, processing speed, and attention in everyday activities. This
study provides initial evidence of the measure’s reliability and validity. Parents (n= 620) and teachers (n=
69) of children age 5-18 completed the ESS Home or School Version, the Post-Concussion Symptom
Inventory (PCSI), and the Behavior Rating Inventory of Executive Function (BRIEF). Internal
consistency reliabilities were acceptable for the ESS-Home Version and ESS-School Version. The ESS
Home and School Versions were not significantly correlated. Most Parent and Teacher ESS scales
demonstrated moderate correlations with PCSI Cognitive Symptoms and BRIEF composites, with
stronger correlations between certain ESS-School scales and the BRIEF. Initial examination of the ESS
reveals evidence of adequate internal consistency, reliability, and validity.
IMPROVING THE DIAGNOSIS OF PEDIATRIC HYPERTENSION IN THE
PRIMARY CARE SETTING
88
SP Potiny RZ Zarr
Hypertension is more common among children than previously recognized, particularly now with the rise
of obesity among our youth. Ethnicity also plays a major role, with highest prevalence among hispanic
children. In Sorof et al (n=5102), 11% of obese children, and 25% of hispanic children were hypertensive.
However, our sensitivity in diagnosis of hypertension in the primary care setting is poor. In Hansen et al,
only 26% of hypertensive children had a diagnosis of hypertension or elevated BP in the medical record.
The purpose of this project is to help improve the diagnosis of hypertension in the primary care setting via
two interventions: (1) a training session to pediatric providers at the clinic that reviews indications for
screening for hypertension and how to check blood pressure in children (2) distributing a palm pilot
program, STAT BP, to help facilitate diagnosis of BP in children. A pre-and post intervention chart
review and surveys of providers were performed to assess the strength of the intervention. The site is a
community health clinic that primarily serves a Latino immigrant population, the population of children at
greatest risk for hypertension.
AN INTERNATIONAL APPLICATION OF EQUIPMENT GUIDELINES
FOR PEDIATRIC EMERGENCIES
DM Walker VR Tolentino VC Ribaya JM Chamberlain
Background: Lack of essential equipment is one potential reason for inadequate pediatric emergency care
in developing countries. Little has been published on this aspect of quality. We performed a survey in a
developing country to determine availability of equipment for pediatric emergency care. Objective: To
characterize the availability of pediatric emergency equipment in emergency departments (EDs) in
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Manila, Philippines. Design and Methods: Medical directors at a convenience sample of EDs with
pediatric emergency capability in Manila, Philippines answered a survey regarding the presence or
absence of 139 pediatric emergency-related equipment items. Previously published joint American
Academy of Pediatrics (AAP) and American College of Emergency Physicians (ACEP) pediatric
guidelines were used to construct the equipment survey. An interview elicited previous knowledge of the
AAP/ACEP guidelines and factors that influence equipment availability. Results: The 9 EDs (2 pediatric
and 7 mixed adult-pediatric) had a mean census of 15,392 pediatric patients per year. EDs possessed an
average of 77% (range 58%-90%) of items on the equipment list. 40% of items on the survey were
possessed by all EDs. 27 items were possessed by less than 50% of EDs. No EDs possessed CO2
detection methods to monitor endotracheal tube placement and 5 of 7 sizes of laryngeal mask airways.
One third of respondents were previously aware of the AAP/ACEP equipment guidelines. Perceived
barriers to equipment unavailability included limited resources, length of procurement process, lack of
manpower to use equipment and rarity of usage of equipment. Conclusions: The EDs studied possessed
the majority of items, although a wide variation was noted among hospitals surveyed. The average
number of items possessed was similar to that possessed by EDs in the United States. Most of the items
missing were rarely used, expensive and/or not crucial for resuscitation. Resource constraints were the
most prevalent barrier to equipment availability.
90
INITIAL EXAMINATION OF SELF-REPORTED POST-CONCUSSION
SYMPTOMS IN NORMAL AND MTBI CHILDREN AGES 5 TO 12
CV Vaughan GG Gioia DV Vincent
The self-report of symptoms of mild traumatic brain injury (mTBI) in young children is not often studied.
Our study’s purpose was two-fold: 1) evaluate the psychometrics of age-specific symptom reports of nonconcussed children, 2) identify key symptom clusters that differentiate children with and without
concussions. the Post-Concussion Symptom Inventory (PCSI) is a self-report inventory used to assess
cognitive, somatic, emotional and sleep symptoms. Eighty six normal children and 25 children with mTBI
ages 5 to 7 completed the 13-item PCSI, and 250 normal and 82 children with mTBI ages 8 to 12
completed the 25-item PCSI. Internal consistency reliability of the PCSI was good for both age groups
(alpha=0.793, ages 5-7; alpha =0.911, ages 8-12). Appropriate scale membership was indicated by itemtotal correlations for all (13) items in the younger group and 23 of 25 items in the older group. Both
normative age groups endorsed fatigue (48.1%), numbness (43.7%), and headaches (34.8%) most
frequently, and vomiting (4%), diplopia (7.5%), and difficulty tying shoes (7.9%) least frequently.
Discriminant function analyses significantly predicted (p < .05) group membership (normal vs.
concussion). The symptom of headaches predicted membership in the younger group, and the symptom
cluster of thinking slowly, numbness, moving slowly, sleeping more, difficulty remembering, and
difficulty concentrating predicted membership in the older group. We demonstrate adequate internal scale
structure of a pediatric self-report measure of concussion symptoms with initial results identifying
symptoms useful for predicting concussion group membership compared with controls.
91
CHARACTERISTICS OF PEDIATRIC HOSPITALISTS PROVIDING
MODERATE TO DEEP SEDATION: A STUDY FROM THE PRIS
NETWORK
DW Marseille J Maniscalco M Turmelle M Ottolini D Carlson
Characteristics of Pediatric Hospitalists Providing Moderate to Deep Sedation: A Study from the PRIS
Network Background There is large variation in the practices of Pediatric Hospitalists (PHs) who provide
moderate and deep sedation (MDS). Objective To better define the epidemiology, training and practices
of PHs who provide MDS. Design/Methods 265 surveys of PHs from the Pediatric Research in Inpatient
Settings (PRIS) Network were collected and evaluated. Statistical analyses of hospital and physician
characteristics were determined based upon sedation practice. Results 54.4% of surveyed PHs indicated
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that they provide MDS. 76% of those who felt that they needed additional procedural training when
deciding to become a hospitalist, desired sedation training. There was a strong association between
providing MDS and work as a PH in non-academic and community hospitals (p-values = 0.019 and
<0.0001, respectively). There was no significant difference between providing MDS and PH gender,
residency type, residency size, or involvement in teaching. PHs who completed residency more than 10
years ago were more likely to provide MDS (p=0.013). PHs providing sedation were significantly less
likely to conduct any type of research (p=0.011). They were also significantly more likely to provide
PICU care (p=0.0035) and care for children in other areas of high acuity (ER, p=0.055) (Step-down Unit,
p=0.12). Lastly, PHs spending greater than 75% of their time as hospitalists were more likely to provide
MDS (p=0.045), as were those working as hospitalists for over 6 yrs. Conclusion Approximately 55%
PHs provide MDS; 78% of PHs who felt that they needed additional procedural training, desired sedation
training. Significant factors associated with PH providing MDS include completion of residency more
than 10 years ago, work as a PH for over 6 years, service as primary attending in the PICU, and lack of
research performance.
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92
HYPOCALCEMIC RICKETS AND DILATED CARDIOMYOPATHY: CASE
REPORTS AND REVIEW OF LITERATURE
JB Brown CS Spurney SN Nunez MR Russell
Internationally, there are isolated case reports published of children presenting with dilated
cardiomyopathy (DCM) in the setting of undiagnosed rickets (Olgun, 2003). Although there has been an
increased prevalence of rickets in the US, there has only been one documented case of associated DCM in
the US. At our institution, a hospital database search was conducted from the years 2000 to 2006 to
identify patients with confirmed Vitamin D deficiency in addition to DCM. Through this search, four
exclusively breast fed African American infants were identified. These infants presented in congestive
heart failure secondary to DCM and during their admission were found to have laboratory evidence
consistent with hypocalcemic rickets. These patients responded dramatically to treatment with vitamin D
and calcium, as cardiac function returned to normal within months. Early recognition of Vitamin D
deficiency was an important factor in these patients’ clinical course. These case reviews document a rare,
serious complication of Vitamin D deficient rickets and support evidence for prevention of this nutritional
deficiency with Vitamin D in exclusively breast fed infants.
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COMPARISON OF THE EFFECT OF VENOARTERIAL VERSUS
VENOVENOUS ECMO ON RENAL FUNCTION
LA Bone AN Massaro A Williams K Rais-Bahrami BL Short
Debate exists as to whether the pulsatile flow of venovenous (VV) ECMO better supports renal function
compared to the non-pulsatile flow of venoarterial (VA) ECMO. Ingyinn (2004) previously demonstrated
that newborn lambs experienced a transient decrease in renal blood flow at the initiation of VA bypass
whereas those on VV ECMO maintained stable renal blood flow. There are limited studies comparing the
effect of VA versus VV ECMO on renal function in humans. The goal of the present study was to
evaluate the effect of VA versus VV bypass on renal function in neonates requiring ECMO life support.
94
SLC30A8 VARIANT IS ASSOCIATED WITH MUSCLE SIZE AND
RESPONSE TO RESISTANCE TRAINING
RR Patel K Adham JS Larkin JM Devaney HA Gordisch-Dressman C Brandoli EP Hoffman LL Tosi
SLC30A8 is a gene that produces a 369 amino acid protein known as zinc transporter-8 (ZnT8) and is
expressed mainly in the islets of Langerhans of the pancreas. Zinc deficiencies and transport problems
have been associated with both Type I and Type II diabetes. We hypothesized that the single nucleotide
polymorphism (SNP) rs13266634 (+C37447T; R325W) of the SLC30A8 gene would be associated with
skeletal muscle changes before and after a resistance training program. This SNP has also been shown to
be have a protective effect against the development of type II diabetes. Participants (n = 626) in the FMS
study were placed on a scheduled resistant training program (twice a week for 12 weeks). Baseline and
post training values of measures such as muscle volume, subcutaneous fat volume, baseline one repetition
(1RM) strength and triglycerides, were taken from the participants. DNA samples were genotyped using
commercially available Taqman assays. A semi-automated MRI analysis tool, RAPIDIAВ®, calculated
muscle volume. Analysis of covariance (ANOVA) statistical analysis determined significantly different
mean levels of muscle and fat volume among genotypes. Females (n = 323) with two copies of the T
allele gained muscle volume (p = 0.03) and lost fat (p = 0.003) with the resistance training program.
Males (n = 303) with two copies of the T allele were associated with great 1RM strength (p = 0.04),
greater baseline whole muscle volume (p = 0.002) and higher triglyceride values (p = 0.006). Thus,
variations in the SNP rs13266634 found in the gene SLC30A8 are associated with overall positive results
in muscle training. This finding may provide insight into its role as a protective allele in diabetes.
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SLIPPED CAPITAL FEMORAL EPIPHYSIS AT CHILDREN’S NATIONAL
MEDICAL CENTER
95
EJ Silverman C Brandoli LL Tosi
Gather information about the population of patients who are diagnosed with slipped capital femoral
epiphysis (SCFE) at Children's National Medical Center and identify areas in which clinical pathways
might improve care. This was an IRB approved, retrospective review of patients treated surgically for
SCFE at Children's National Medical Center from 6/2005 to 5/2007. Information potentially relevant to
the etiology of SCFE, such as: age, gender, height, weight, calculated BMI, race, vitamin D levels and the
presence of co-morbidities, was collected. 101 patients were diagnosed and treated surgically for SCFE
during the two-year period. The average age of patients was 11.1 for females and 13.3 for males. 88 of the
101 patients were of African American descent. 11 patients were treated for unilateral SCFE of both hips
on different occasions and 17 were bilateral at presentation. Only 16 patients had Vitamin D levels taken.
Of these 16, 87.5% had vitamin D levels that were insufficient (25-OH D btw. 20 and 32 ng/ml) or
deficient (25-OH D < 20 ng/ml). Of the 74 cases in which complete information was available, 21
patients were overweight (BMI between 25 and 29.9) and 33 patients were obese (BMI = 30). Children
who develop a SCFE can develop premature arthritis and significant disability. Thus optimizing their
care, and, in the case of a unilateral slip, developing strategies to prevent a contralateral slip, are essential.
Our future plans include designing a standardized care pathway that ensures better data gathering at
presentation and improved efforts at obesity management. In addition, we are planning a prospective
study to investigate the relationship between vitamin D levels and SCFE.
EPIDEMIOLOGY OF INVASIVE GROUP A STREPTOCOCCUS IN
CHILDREN
96
N Niforatos P Manicone N Niforatos S Nambiar J Campos X Song N Singh
Invasive Group A Streptococcus (IGAS) causes significant morbidity and mortality in the United States.
The CDC estimates 8950-1150 episodes of IGAS and 1050-1850 deaths per year. The pediatric
population has been identified in population-based studies to have one of the highest incidence, morbidity
and mortality rates. Nonetheless, limited data exists on children with IGAS, particularly who is at risk for
complications and sequelae of this disease. The goals of this study are to describe the epidemiology of
IGAS in children over a 3 year time period at a large, urban, tertiary care pediatric hospital and to
determine risk factors associated with complications and sequelae of IGAS.
EXERCISE TESTING IN CHILDREN WITH CHEST PAIN
S Anwar RE Kavey
Chest pain is a relatively frequent complaint in the pediatric population, and one that can cause significant
distress among patients and their parents. Selbst (1997) reports chest pain occurs in 6 in 1,000 children
who present to an urban pediatric emergency department or clinic. Chest pain can originate from several
organ systems besides the heart, and in fact, some studies suggest that the incidence of chest pain
attributable to a cardiac etiology is less than 5% (Cava, et. al 2004). However, every year many exercise
stress tests are conducted in Cardiology clinics to evaluate this complaint. The aim of this research project
is to conduct a retrospective chart review to evaluate the findings of treadmill exercise testing carried out
in the exercise physiology laboratory for children presenting to the Cardiology clinic with the complaint
of chest pain. The specific aim of this research study will be to evaluate the following hypothesis
statement: Among children presenting to the Cardiology clinic with chest pain, we hypothesize that
treadmill exercise testing will not reveal positive cardiac diagnostic findings in the majority of patients.
For the purposes of this study, positive findings on stress testing indicates a serious cardiac diagnosis, and
are defined as: a stress test finding that is significantly abnormal when compared to a normal study, or a
cardiac diagnosis which emerged because of the treadmill exercise test results. By reviewing the results of
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stress testing to evaluate pediatric chest pain, this study aims to call to question the utility of this test in
the work-up of pediatric chest pain. The ultimate goal of this study is to prompt prospective investigation
of the outcomes of exercise-testing in order to produce a standardized evaluation plan incorporating the
role of stress testing in the work-up of pediatric chest pain.
98
DC PEDIATRIC PALLIATIVE CARE COLLABORATION PARENT AND
CHILD RESOURCE MANUALS
SE Hall
An increasing amount of research indicates that the needs of children and families confronted by a lifethreatening condition are not being met by our healthcare system. The Institute of Medicine (2003) noted
in its report that children and their families too often fail to receive competent, compassionate and
consistent care, which meets their physical, emotional and spiritual needs. In particular, parents caring for
children both in the home and in the hospital report inadequate, or insensitive communication about
treatment and prognosis; preventable oversights in procedures or policies, failure to include or meet the
needs or siblings, inconsistent bereavement follow-up; difficulty accessing important services; feeling
abandoned and overwhelmed; and emotional and financial struggles (Contro et al, 2002; Meyer, Ritholz,
Burns & Troug, 2002). The DC Pediatric Palliative Care Collaboration (DCPPCC) is a cooperative
venture of four health care organizations in Washington, D.C. that seeks to provide comprehensive care to
children and families facing life-threatening and life-limiting illnesses. An important objective of the
DCPPCC initiative is to provide information regarding existing resources in the District of Columbia to
these families. The goal of the present project was threefold: 1) to compile a comprehensive list of
community resources available in the District of Columbia 2) to create booklets for parents containing ,
and 3) to create a booklet for children referred to the DCPPCC.
99
BREASTFEEDING INTENT, PACIFIER USE, AND THE DURATION OF
BREASTFEEDING IN AN URBAN POPULATION: A PILOT STUDY
OO Omojokun RY Moon
Pacifier use is common in the United States, with prevalence estimates ranging from 36% to 74%.(1-4) In
2005, the American Academy of Pediatrics updated their recommendations for the reduction of Sudden
Infant Death Syndrome (SIDS) risk; parents are now encouraged to consider offering a pacifier to infants
at bedtime, up to 1 year of age.(5) This recommendation was based on a detailed review of available
literature, including a meta-analysis published in the same issue, and has garnered some controversy
regarding the effect of pacifier use on breastfeeding.(6-10) Several publications have reported shortened
breastfeeding duration and lower rates of breastfeeding initiation in infants who use a pacifier, but the
literature is inconsistent and is far from proving causality.(11-18) Review of the literature reveals very
limited data from the United States’ urban populations, and almost no data that specifically analyzes
maternal intent to breastfeed as a variable in determining the effect of pacifier use on breastfeeding. The
aims of this study were as follows: To determine the rate of initiation of pacifier use in mother-infant
dyads who plan to breastfeed, compared to those who do not plan to breastfeed; and to determine the
effect of pacifier use at =7 days, 3 months, and 6 months of age, on the duration of breastfeeding in
mothers with intent to breastfeed.
100
NEONATAL URINARY TRACT INFECTION (UTI): VARIABILITY IN
LENGTH OF INTRAVENOUS ANTIBIOTIC THERAPY (IVAT)
NS Shah KP Perry WP Pastor JM Maniscalco
Urinary tract infection (UTI) has been well studied in infants and children secondary to the potential
adverse outcomes of inadequate evaluation and treatment, such as renal scarring and hypertension as an
adult. Infections of the urinary tract affect 2.4%-2.8% of children every year and account for more than
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1.1 million office visits annually and thousands of dollars in hospital costs. UTI is the most common
serious bacterial infection identified in febrile neonates presenting to the emergency department. The
standard treatment for neonatal UTI is 7-14 days of antibiotic therapy; moreover, it is recommended that
this course be given intravenously secondary to the theory that neonatal UTI is hematogenous in origin.
Thus, recommended therapy requires extended hospitalization and may include invasive procedures, such
as central line placement. In practice, pediatricians will occasionally treat newborns that have a febrile,
uncomplicated UTI with a combination of IV and oral antibiotics once an organism has been isolated in
culture, as this has been the trend in older infants. This may lead to a wide range of variability in
treatment protocols amongst practitioners, as there is no clear, evidence-based clinical guideline for
combination treatment in neonates.
NEGATIVE PRESSURE WOUND THERAPY IN CHILDREN: EXPANDED
INDICATIONS
101
C Brandoli D Contractor J Larkin J Amling C Brandoli L Tosi
To demonstrate the expanding range of indications for negative pressure wound therapy (NPWT) in the
pediatric population. Methods: We conducted an IRB approved, retrospective medical record review of
NPWT provided to children at CNMC between January 2001 to May 2007. Data evaluated included
wound etiology, wound location, significant co-morbidities, and days on NPWT. Results: 171 children,
newborn to age 21, were identified: 6 newborns, 20 infants, 39 children age 1-5, 51 from age 6-10, 43
from age 11-18, and 12 from age 19-21. Etiologies included 60 surgical wounds (avg 17.8 days of
treatment), 33 traumatic wounds (avg 18.5 days), 32 burns (avg 8.7 days), 18 pressure ulcers (avg 41.2
days), 10 osteomyelitis (avg 18.6 days), 10 other infection/abscesses (avg 20 days), 5 pilonidal cyst (avg
4.4 days), 3 i.v. extravasation (avg 29.7 days). Important co-morbidities included cerebral palsy, spina
bifida, cancer, sickle cell anemia, scoliosis, obesity, and diabetes. 55% of surgical wounds and 33.3% of
traumatic wounds were infected prior to NPWT treatment. Surgical wounds included 23 chest and 25
abdominal wounds. 21 of 32 burn wounds were full-thickness. All pressure ulcers were stage 3 or 4. In
addition to NPWT, 29 of 171 children also required split thickness skin grafts to achieve wound closure.
Conclusions: This six-year retrospective study documents the use of NPWT in 171 children who had a far
broader range of indications than those examined by previous authors. The results of this study will be
used to develop an improved wound care pathway at CNMC.
EZETIMIBE IN THE TREATMENT OF PEDIATRIC PATIENTS WITH
HYPERCHOLESTEROLEMIA
102
K Wai SB Clauss K Wai RE Kavey KS Kuehl
Atherosclerosis and its sequelae are major health problems and the leading causes of morbidity and
mortality in the United States as well as other industrialized countries. Studies have shown that
atherosclerotic changes found in adults begin to develop in childhood. In a scientific statement by the
American Heart Association, McCrindle et al (2007) offered 3 recommendations: 1. Increased screening
for hyperlipidemia, 2. Use of statins as the first line of drug therapy in patients with hyperlipidemia, and
3. Medical therapy in patients prior to age 10 who have high risk lipid values. Ezetimibe (Zetia) is a
newer class of cholesterol lowering agents which works by blocking cholesterol absorption from the GI
tract at the intestinal brush border, leading to a decrease in cholesterol availability in the liver. It has been
proven to be effective in lowering LDL and increase HDL in adults, and has a more favorable side effect
profile. To date, there are no published data on effectiveness, safety and tolerability of ezetimibe as a
monotherapy in children with hyperlipidemia. The goal of the present study was to retrospectively review
the efficacy of ezetimibe for treatment of hypercholesterolemia in pediatric patients. Our hypothesis is
that ezetimibe is efficacious and well tolerated in our pediatric hyperlipidemic population
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PSYCHOLOGICAL COUNSELING IN THE ADOLESCENT DIABETIC
POPULATION IS AN IMPORTANT COMPONENT IN MAINTENANCE OF
ADEQUATE GLYCEMIC CONTROL
M Russell F Cogen
Diabetics have double the risk of developing depression. Adult studies have also shown that nondiabetics
with depression have a higher incidence of developing peripheral insulin resistance and poorer glycemic
control. The link between depression and hyperglycemia is not clearly understood. The goal of the present
study was to demonstrate that adolescents who underwent psychological counseling had a greater degree
of improvement in glycemic control as compared with those who did not receive counseling.
104
YOGA TO IMPROVE FITNESS IN CHILDREN WITH DISABILITIES
CH Sullivan
Yoga programs for children have increased exponentially over the last five years and they are frequently
found in fitness centers and recreation programs. Typically developing children have shown improved
visual-spatial skills, grip strength and motor control following a yoga program(Dash and Telles, 1999;
Manjunath and Telles, 2001; Manjunath and Telles, 2004). Yoga is purported to be beneficial to improve
physical fitness in children with disabilities (Sumar, 1998, NCPAD, 2006). There is limited clinical
evidence to support yoga as a fitness activity for children with disabilities (Galantino, Galbavy and
Quinn, 2008). This study tested the hypothesis that yoga will improve physical fitness in a child with a
disability. It will provide preliminary data on the use of yoga to improve the health related fitness
components of strength and flexibility and the performance related component of balance.
105
LANGUAGE LATERALIZATION IN FOCAL EPILEPSY AS DETERMINED
BY FOUR METHODS
JD Mayo LR Rosenberger MM Berl WD Gaillard
Determining language laterality in epilepsy patients has clinical and experimental utility. Clinically, it is
important to identify language areas in planning for surgery. Experimentally, language laterality is a
useful variable in the study of language development. Determining language laterality through direct
methods such as the Wada procedure or ESM is invasive and therefore limited to patient populations.
While fMRI offers a non-invasive technique for determining language laterality and allows for control
studies, classification of laterality based on fMRI is vulnerable to different methods and thresholds that
are used. As a result, functional imaging studies carry with them a debate about which methods and which
thresholds yield the most accurate results. In the present study, we compared four methods of determining
language dominance using fMRI.
106
THYROID STATUS HAS ONLY A MINOR EFFECT ON WEIGHT GAIN
OR LOSS IN CHILDREN
MK Crocker P Kaplowitz
Clinicians often believe thyroid hormone levels can significantly impact weight; thus, thyroid functions
tests are usually part of the work up for obesity as well as weight loss. There is little pediatric data that
evaluates the relationship between thyroid status and weight in detail. Patients seen over a three year
period for an initial diagnosis of hyperthyroidism or hypothyroidism were studied. Weight and BMI were
recorded at the time of diagnosis and at two subsequent follow-up visits. Hypothyroid patients were
slightly heavier than hyperthyroid patients at diagnosis, but this difference was not statistically significant
and much smaller than the range of BMI SD scores among both groups. In the first months of treatment
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weight changes were quite variable but on average were modest and smaller in hypothyroid patients.
Furthermore, by the second follow-up visit, hypothyroid patients had regained the lost weight and BMI
SD had not fallen any further. Hyperthyroid patients, however, continued to gain weight at the second
follow-up visit. Males in the hyperthyroid group exhibited lower BMIs at diagnosis and gave a history of
weight loss more often than girls. No sex differences were seen in hypothyroid patients. Since thyroid
status has a minor effect on weight status in children, particularly among those with hypothyroidism,
primary care physicians should not feel obligated to check thyroid tests in patients with obesity or weight
gain unless other hypothyroid signs and symptoms including goiter are also present. Weight loss should
prompt consideration of hyperthyroidism, which usually presents with goiter and tachycardia, as well as
multiple typical symptoms.
SIMPLIFYING CONGENITAL CYSTIC PULMONARY LESIONS: A
SPECTRUM OF RAD-PATH CORRELATION
107
DA Rabinowitz D Bulas A Atif R Murray
Cystic congenital pulmonary lesions have classically been identified as separate and unique lesions, such
as CCAM, CLE, and sequestration. This convention underestimates the complexity and feature crossover
often seen with these lesions. Pathologists have derived a classification system identifying these lesions as
part of a spectrum of cystic lesions resulting from an in-utero insult, likely airway obstruction. This poster
reviews the current recommendations for descriptions of congenital cystic pulmonary lesions with multimodality fetal/pediatric imaging and pathologic correlation.
THE ROLE OF THE EMERGENCY DEPARTMENT IN YOUTH VIOLENCE
PREVENTION: A SYSTEMATIC REVIEW
108
TJ Johnson
Youth violence is a major cause of morbidity and mortality. More than 750,000 young people are seen in
the emergency department (ED) annually for intentional injuries, and homicide is the second leading
cause of death among youth ages 10-24. While the ED's role in identification, evaluation and treatment of
violent injuries is clear, questions remain regarding the appropriateness, feasibility and effectiveness of
ED-based youth violence prevention efforts. The goal of this study was to discuss the role of the ED in
youth violence prevention and provide a systematic analysis and review of ED-based interventions.
EPIDEMIOLOGY OF CLOSTRIDIUM DIFFICILE ASSOCIATED DISEASE
IN INFANTS YOUNGER THAN 2 YEARS OF AGE
TM Tran X Song JM Campos N Singh
Clostridium difficile is the leading cause of nosocomial diarrhea accounting for 20-45% of diarrhea that
occurs in healthcare settings (Alfa, 1998). Clostridium difficile-associated disease (CDAD) generally is
considered a disease of elderly patients. Children are not commonly considered at high-risk for
contracting CDAD, rather it is common for infants to be asymptomatically colonized with toxinproducing C. difficile strains (Benson, 2007). A recent study at a children's hospital found an
unexpectedly high rate (6.7%) of C. difficile toxin among diarrheal pathogens (Klein, 2006). Recent data
suggest that the incidence of CDAD in children is rising, but little information is available in infants
younger than 2 years of age (Benson, 2007). The findings from this study emphasize the importance of
recognizing C. difficile as a possible diarrheal pathogen in infants under 2-years old. CDAD may cause
serious morbidity and mortality in young infants. Further efforts to develop the most optimal clinical
management of CDAD in young infants are warranted.
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SUCCESS RATE OF FMRI SCANS IN CHILDREN WITH ASD,
EPILEPSY, ADHD, AND TYPICAL DEVELOPMENT
KF Jankowski D Shook L Rosenberger BE Yerys MM Berl CJ Vaidya JW Van Meter WD Gaillard
The field of pediatric functional neuroimaging is a growing method for analyzing brain development;
however the feasibility of this technique lacks thorough investigation. Although the scanning efficacy of
adult populations has been studied, pediatric literature is limited to typically-developing (TD)
populations. Prior research has found that TD children between the ages of four and six years old have a
success rate of 47%, seven to nine year olds have a success rate of 76%, and ten to twelve year olds have
a success rate of 96%. (Byars et al., 2002) The aim of the current study is to test the success rate of
pediatric clinical (Autism Spectrum Disorders, Epilepsy, Attention Deficit Hyperactive Disorder) and
control populations across development to provide a feasibility analysis of pediatric functional
neuroimaging. Prior research has revealed distinct age boundaries where scan success improves greatly.
The current study aims to build upon these findings to define three age groups: four to six year olds,
seven to nine year olds, and ten to twelve year olds. In addition, the current study adopts a more stringent
measure of scan success than what has been used previously. Our modified definition includes the former
requirement of task completion, as well as the additional necessity for producing usable data. We predict
that while this new definition will give better representations of scanning efficacy, it will also produce
lower estimates than what were previously reported. The results of this study will be imperative for
accurate budgetary planning and research proposals in pediatric research.
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SLEEP DURATION IN HEALTHY AFRICAN AMERICAN CHILDRENA
AND ADOLESCENTS
E Huntley D Lewin H Bhatt
Introduction: The purpose of this study is to establish estimates of sleep duration in a sample of healthy
African American children and adolescents using actigraphy (a standardized objective measures of sleep)
over one week. In addition, comparisons were made to norms based on a European sample of children
and adolescents. Trends in sleep behavior and accrued sleep debt are also described. Methods: (N= 92)
African-American children and adolescents from an urban environment, aged 6 to18, were recruited.
Mean age was 11.2 (В±3.3) years and 56% were female. Participants wore wrist actigraphs and completed
daily sleep logs for 7 days. Conclusions: This sample of healthy, urban African American children and
adolescents sleep significantly less compared to normative data derived from a sample of European
children and adolescents. Youth ages 9 -18 tended to sleep more on weekends than on weekdays
indicating that a sleep debt accrues during the course of the week.
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WORKING MEMORY PROFILES IN CHILDREN WITH AUTISM
SPECTRUM DISORDERS AND ELEVATED ADHD SYMPTOMS
JL Sokoloff D Shook KF Jankowski BE Yerys GL Wallace J James C Vaidya L Kenworthy
Both children with Autism Spectrum Disorders (ASD) and children with ADHD exhibit deficits in
Executive Function (EF). EF is related to cognitive abilities that require self regulation and the integration
of specific skills such as working memory, inhibition, planning, and cognitive flexibility. Currently,
DSM-IV excludes a co-morbid diagnosis of these disorders. To date, there is little available data that
addresses the EF profile of ASD children with elevated ADHD symptoms (Combined) as compared to
ASD children without ADHD symptoms, children with ADHD, and typically developing (TD) children in
real life context. This Study Investigates whether children in the Combined group present unique working
memory deficits relative to pure ASD and ADHD groups and to a group of TD children. For this study,
132 school-aged children (TD, N = 57, FSIQ = 117; ASD, N = 16, FSIQ = 115; ADHD, N = 23, FSIQ =
114; Combined, N = 17, FSIQ = 110) were recruited for research studies conducted at Children’s
National Medical Center. Parents reported on everyday EF, developmental history, inattention and
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hyperactivity symptoms. Administered measures included the Autism Diagnostic Interview, the Autism
Diagnostic Observation Schedule, the ADHD rating scale, and the BRIEF, a widely used parent report
measure of EF in real life settings and situations. This study found that in parent reports the Combined
group was rated significantly more impaired on BRIEF measures of working memory, behavioral
regulation index, metacognition index, and global executive composite compared to TD and ASD groups.
There were no significant EF differences between the Combined and ADHD groups. Our findings suggest
that children with co-morbid symptoms exhibit unique EF impairments to children with out co-morbid
symptoms. These findings indicate that a new subtype may exist on the Autism Spectrum and therefore
future studies should explore this distinction further.
VALIDATION STUDY FOR TWO TYPES OF RESTING ENERGY
EXPENDITURE EQUIPMENT
113
S Villavicencio N Mirza C Klein R Hirsch
Although equipment that measure resting energy expenditure (REE) utilize similar scientific principles,
differences in measurement among different models are expected. When Children’s began utilizing a
new equipment model, it was necessary to test its agreement in REE measurement with the earlier model.
Participants were measured by two different REE equipment in the same day and in random order. In one
test, REE was calculated from indirect calorimetry using a VMAX Spectra 29N model (SensorMedics
Corporation, Yorba Linda, CA). Then in the second test, REE was calculated from indirect calorimetry
using an Ultima CardiO2в„ў system (Medical Graphics Corporation, St. Paul, MN). Anticipated REE was
calculated using the Institute of Medicine’s energy equations. Measured values falling outside 0.71 to 1.4
times the predicted value were considered to be equipment failure and those subjects were excluded from
analysis. REE measures by the two types of equipment were compared using the Bland-Altman method,
Intraclass Correlation, and Paired T-Test. Measures that differed by 8% or less were considered to be in
agreement. Data were collected from a sample of 17 healthy participants, aged 7 – 52 years, that was
racially diverse. The Intraclass Correlations were: R2 = 0.80 (p=0.0055) between predicted and observed
2900, R2 = 0.82 (p=0.004) between predicted and observed Ultima, and R2 = 0.58 (p=0.0795) between
observed 2900 and observed Ultima. Paired T-Test analysis of REE between 2900 and Ultima equipment
yielded a mean difference of -191kcal (95%CI: -418, 35) with a t-value of -1.91 (p=0.0883). This mean
difference is 14% of the magnitude of the mean REE from the 2900 equipment. Although the difference
between REE from 2900 and REE from Ultima was not statistically significant, it was clinically relevant.
Thus, caution should be taken when comparing measurements from one type of equipment with
measurements from another type of equipment.
NEURORADIOGRAPHIC FEATURES OF PATIENTS WITH THE
CEREBELLAR MUTISM SYNDROME: A LONGITUDINAL MRI STUDY
EM Wells ZP Khademian KS Walsh R Sposto G Vezina RF Keating RJ Packer
The etiology and long-term outcome of the cerebellar mutism syndrome (CMS), a postoperative
syndrome of diminished speech, hypotonia, and ataxia, which affects 25% of patients with
medulloblastoma, is poorly elucidated. The current study was undertaken to determine: factors associated
with development of CMS; means to determine its severity or etiology; and outcomes of these patients.
The pre-, immediate post-, and one year post-operative MRI, clinical features, and neurologic and
neurocognitive outcomes of 28 consecutive children with medulloblastoma, 11 (39%) of which had CMS,
either operated on or referred soon after surgery to our institution, were reviewed. The preoperative scans
showed no differences in tumor size, hydrocephalus or peritumoral edema. There were trends toward a
correlation with tumor location at the brainstem (p=0.05) and the cerebellomedullary angle (p=0.08).
Immediate post-operative scans showed cerebellar edema in 92% of all patients; CMS patients had more
superior cerebellar peduncle edema (p<0.05) and a trend toward more middle cerebellar peduncle edema
(p=0.07). At one-year, patients with CMS had more moderate to severe atrophy/gliosis of total cerebellum
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(p<0.01), vermis (p<0.01), and brainstem (p<0.05). Mean IQ was eleven points lower in patients with
CMS (IQ=78.5, sd 19.8) compared to those without (IQ=89.5, sd 26.5), difference not significant. CMS is
associated with lasting post-operative damage to the cerebellum and brainstem; damage not identified on
immediate post-operative MRI. Our incidence of CMS has diminished following change in surgical
technique, but long-term outcome for those affected is poor.
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ALL FOR ONE: REDUCING PIV INFILTRATES THROUGH A
MULTIDISCIPLINARY APPROACH
115
LW Williams TT Taylor AN Nguyen-Massaro CK Kusenda DM McCoy KN Nelson GC Chai LT Talley
The NICU Care Delivery Team noted a significant and sustained increase in peripheral IV infiltrates. The
team subsequently implemented a collaborative initiative to reduce the incidence of infiltrates. Baseline
data collection was conducted through a nutrition-nursing partnership and identified many opportunities
to improve practice. A comprehensive literature review identified several interventions as critical to the
success of reducing infiltrates in the neonatal population. The following interventions were identified as
best practice: eliminate albumin in parenteral nutrition solutions; limit the maximum amount of calcium
added to TPN (250 mg elemental calcium per 500 mls); decrease the maximum osmolality of peripheral
parenteral nutrition solutions (from 1050 mOsm to 900 mOsm/kg); and begin filtration of TPN by bedside
nurses. Because the initiative was multifaceted, the collaborative efforts between the involved
departments were assumed critical to its success
VISCOSITY IN INFANT DYSPHAGIA MANAGEMENT:
STANDARDIZING PRACTICE
116
SL Stuart JM Tanner
Our study was performed in response to a need to standardize liquid consistencies in infant dysphagia
management. More specifically, we set out to determine comparability between viscosity measures of
varied thickened liquids used at bedside and in fluoroscopic assessment of swallow with the long-term
goal of establishing better standards of continuity between assessment and treatment. Measurements were
taken using a Brookfield Engineering LVDVII+Pro Cone/Plate Viscometer. Spindle and speed
combinations to maximize shear rates consistent with swallowing were utilized whenever possible.
Statiscially there is no comparability between barium and formula mixtures, regardless of thickener
utilized. Conclusions regarding viscosity used in bedside management in relation to videofluoroscopic
studies are discussed.
COMPREHENSIVE ASSESSMENT AND RELATIONSHIP BETWEEN
NUTRITION AND GROWTH IN PEDIATRIC HEMODIALYSIS PATIENTS
KL Sgambat K Kher M Fragale K Moylan D Silverstein
Nutrition and growth predict outcome in pediatric hemodialysis (HD) patients. We assessed these
parameters in 14 Ped HD patients over 1 year (y): 6 females, 8 males; 13 African-American, 1
Hispanic; Age at end of study=15.9В±0.6 y. For the entire cohort over 1 y, serum albumin (Alb) was
4.3В±0.0 g/dl and normalized protein catabolic rate (nPCR) was 1.0В±0.0 (correlation=0.33, p<0.0001). The
relationship between Alb and nPCR was less evident in individual patients, being significant in only 4/14
(29%), and negative in one. The mean standard deviation and variance was higher for Alb compared to
nPCR, suggesting that the lack of correlation between Alb and nPCR may be due to the greater degree of
variability in monthly Alb levels. Among all patients over one year, spKt/V was 1.59В±0.01. Using spKt/V
of 1.6 as a cutoff, nPCR trended with spKt/V (for spKt/V=1.6, nPCR=1.03; for spKt/V<1.6, nPCR=0.97).
In contrast, Alb had no relationship with spKt/V: for spKt/V=1.6, Alb=4.31 g/dl; for spKt/V<1.6,
Alb=4.25 g/dl). Body mass index percentile (BMI%) was 35.5В±2.9, Percent ideal body weight (%IBW)
was 96.2В±1.5, Height-SDS (Ht-SDS) was -1.30В±0.11, and percent weight gain (PWG) was +4.9В±1.9%.
The highest incidence of reaching our yearly mean targets for growth were seen for BMI% (64% of
patients) and PWG (79% of patients).The most commonly missed target was for Ht-SDS (21% of
patients).There was a negative, significant relationship between Alb and nPCR with BMI%, %IBW, and
Ht-SDS, and a significant, positive relationship between Alb and nPCR with PWG. Except for a positive
correlation between BMI% and %IBW (r=0.97, p<0.0001) there was no correlation among the other
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growth parameters. We conclude that the relationship between Alb and nPCR is not strong, especially on
an individual patient basis. In addition, nutritional status does not predict growth in Ped HD patients.
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PEDIATRIC RAPID RESPONSE TEAMS DECREASE
CARDIOPULMONARY ARRESTS ON ACUTE CARE UNITS
KW Woronick JC Cepero DS Stockwell HD Dalton
In 1990, Australia first reported that Rapid Response Teams have been effective in decreasing hospital
mortality, ICU admissions, arrests prior to transfer to the ICU and post infarction/post surgical deaths in
the adult population. Rapid response teams (RRT) have been associated with a decreased number of
cardiac arrests on adult acute care units by facilitating early intervention. We assessed the implementation
of an RRT at our urban academic children’s hospital. A pediatric rapid response team was developed as
result of increased patient acuity and cardio-pulmonary arrests on acute care units. The goal was to
determine if rapid response teams would actually decrease the total number of cardiopulmonary arrests
occurring on acute care units by 50%. The RRT consists of a pediatric ICU fellow, ICU nurse, and
respiratory therapist. The team began with activation by nursing, medical and ancillary personnel when
concerns of patient status occurred. Family-initiated RRT was introduced in February 2007. Data was
collected from July 2006-June 2007 and compared to the prior year.
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BRAIN MALFORMATIONS IN PATIENTS WITH NEUROFIBROMATOSIS 1
TC Bouton R Kutteruf G Vezina TC Bouton R Blatt RJ Packer MT Acosta
Objective: Evaluation of brain malformations in patients with Neurofibromatosis 1 (NF1), their frequency
and association with other clinical findings. Methods: The 604 NF1 patients in the Children’s National
Medical Center (CNMC) NF1 database were evaluated for presence of brain malformations. Records of
patients found to have malformations were assessed for cognitive/behavioral and motor deficits, and
seizures. Results: Of the 604 patients, 35 were found to have brain malformations. Most prevalent were
hypothalamic hamartomas (14 patients, estimated at 1 in 50,000-100,000 persons) and Chiari I
malformations (15 patients, estimated slightly less than 1 in 1000 for the general population). In 5 patients
other malformations were found, including diencephalic hamartoma, gray matter heterotopia,
hemihypertrophy, and double cortex. Cognitive/behavioral deficits were identified in 46% of the NF1
population at CNMC. Of 32 patients with brain malformations, 50 % suffered from cognitive/behavioral
deficits (50% in hamartoma, 40% in Chiari and 80% in other), while 60 % demonstrated motor deficits
(50% in hamartoma, 60% in Chiari and 60% in other) and 12.5% had seizures (14% in hamartoma, 13%
in Chiari and 40% in other). Conclusions: Brain malformations are more frequent in patients with NF1
when compared with general population. Migration disorders and hypothalamic harmatomas are may
represent deficits in organization process during the pre-natal development. Brain malformations are
strongly associated with other neurological findings in NF1 patients. . Understanding the mechanisms
affected in brain development by the absence of nerofibromin will help us to better understand the
molecular and cellular implications on learning in NF1 patients.
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NURSING RESEARCH PROGRAM AT CNMC FROM CLINICAL
QUESTIONS TO RESEARCH TO IMPROVE CARE
S Feetham P Broadnax C Guzzetta N Robinson C May J Pryor C Jacobsen-Williams R Bannerman
A strategic goal of CNMC is that nurses be actively involved in nursing research and evidence-based
practice as a means of achieving excellence in nursing practice. Our aim is to describe the purpose of our
nursing research program at CNMC and its resources, infrastructure, and outcomes. The purpose of
CNMC’s nursing research program is to provide resources and infrastructure for conducting nursing
research and translating research to practice and education with the aim of advancing scientific
knowledge of nurses and other health professionals and to improve the quality and safety of care for
children and families. Nursing research is interdisciplinary and involves the conduct of clinical and basic
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research on health and illness across the lifespan to promote and improve the health of individuals,
families, communities, and populations. Resources for conducting nursing research include the NursingResearch Advisory Committee, an arm of the Clinical Improvement and Nursing Research Council and
nursing research consultants to mentor nurses in identifying research questions, developing research
protocols, preparing IRB submissions, seeking funding for research, conducting nursing research, and
disseminating findings. Resources also include a nursing doctoral support group, nursing participation in
the Washington Regional Institute of Clinical and Translational Science, and collaboration with nurse
scientists in the Washington Regional Nursing Research Consortium to promote a regional environment
conducive to inquiry and scholarship that will foster clinical and translational research. Currently there
are 12 active nursing research studies at CNMC (e.g., effects of a PICC team on blood stream infections,
treatment decisions of parents of seriously ill patients, interventions to reduce emergency department
throughput times, effects of family presence during trauma stats, fasting times of
surgical/radiology/procedure patients, factors associated with monitor alarm generation, and
thermoregulation of the perioperative neonate). Two studies have been funded and two more have been
submitted for funding.
IDIOPATHIC TOE WALKING: CASE REPORT
121
AJ Joslyn KL Lefert OM Morozova
Idiopathic toe walking (ITW) is a condition in which children walk with a toe-toe gait pattern in the
absence of developmental, neurological, or neuromuscular conditions. It is considered a diagnosis of
exclusion. ITW is rare and occurs in 1:100 new patients. Consistent heel strike generally appears at 18
months of age and at a mean of 22.5 weeks after independent walking. The association of delays in
various domains including language, learning problems, fine motor, visuomotor and gross motor, with
ITW suggests that toe-walking may be another manifestation of a more global neurodevelopmental
condition. ITW results in fixed contractures of the achilles tendon. Long standing equinus contracture can
lead to a valgus deformity at the hindfoot resulting in possible surgical intervention. Fox et al. reported
that out of 44 children with ITW treated with serial casting, 66% improved in their gait, a majority of the
children ceased toe walking and there was an increase in DF range of motion. Hirsch et al. treated
children with ITW with PT, casting, orthoses or combination. Long term results reflect natural history
with spontaneous resolution in the majority of children (7-21 yrs after initial diagnosis). Our goal is to
review current research to determine and follow the best evidence based practice for the management and
care of idiopathic toe walking in order to prevent or prolong the need for surgical intervention.
PENTOXIFYLLINE FAILS TO RESCUE MUSCLE STRENGTH AND
FUNCTION DETERIORATION IN PREDNISONE TREATED DUCHENNE
MUSCULAR DYSTROPHY
A Zimmerman A Arrieta F Hu D Escolar A Kornberg K Gorni A Dubrovsky R Leshner
Duchenne muscular dystrophy (DMD) is the most common and devastating type of muscular dystrophy
due to a complete absence of dystrophin, resulting in progressive weakness and wasting of skeletal,
cardiac and respiratory muscle. The currently accepted treatment with corticosteroids is only effective for
slowing disease progression and not halting DMD. We reasoned that a synergistic medication could
extend the benefit received with corticosteroids. Recent advances in the understanding of molecular
disease pathogenesis havw provided an opportunity to identify additional approaches to DMD treatment.
Pentoxifylline (PTX) has the potential to act on several of the pathways present in DMD. An open-label
pilot study of an oral PTX solution was conducted and was not well tolerated in the DMD population.
Thus, this double-blind randomized study employed the capsule formulation of PTX and tested for
additional benefit in DMD over corticosteroid treatment alone. The addition of PTX to steroid treated
boys with DMD did not improve or halt deterioration of muscle strength and function or improve
contractures and quality of life over 12 months period. PTX treatment significantly increased the risk of
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mild to moderate gastrointestinal and hematological adverse effects. However, should the drug had been
effective, these would have been within the acceptable range for clinical use (mostly nausea and
bruising). Based on this study, the addition of PTX to an stable dose regimen of prednisone or other
corticosteroid in ambulant DMD boys age 7 years or older is not recommended.
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APPROPRIATE PROPHYLACTIC ANTIBIOTICS IN REDUCING
SURGICAL SITE INFECTION IN PEDIATRIC PATIENTS UNDERGOING
CARDIOVASCULAR SURGERY
J Schexnayder C Chen X Song J Campbell
Appropriate prophylactic antibiotics (Prophy-Abx) has demonstrated favorable results in reducing
surgical site infections (SSIs) among adult patients, but its impact on pediatric patients remains unclear. A
1:4 matched case-control study was conducted in children with congenital anomalies undergoing CVS
repair at Children’s National Medical Center between 1/1/2005 and 9/31/2006 to describe the
characteristics and rate of SSIs in this study population and to evaluate the association between ProphyAbx and SSIs. SSI was identified based on physician diagnosis (i.e. mediastinitis), clinical symptoms,
and/or wound culture(s). Cases of SSI were individually matched to four controls selected from non-SSI
patients based on the surgery date (as close to that of case as possible) and type of surgery (cardiac vs.
cardiac other vs. both) as defined by the National Healthcare Safety Network (NHSN). Prophy-Abx was
defined as administering 1st dose of cefazolin within 1 hour or 1st dose of vancomycin within 2 hours
prior to the initial surgical incision. During the study period, 21 SSIs were identified and were matched to
84 controls. Compared to controls, cases were younger at surgery, disproportionately male, and less likely
to undergo CVS surgery within 1 day following admission. Ninety percent and 7% of patients received
cefazolin and vancomycin, respectively, as the 1st dose of Prophy-Abx. Of the patients who received
Prophy-Abx, 80% were administered within the appropriate pre-operative period. Unadjusted analysis
revealed that appropriate Prophy-Abx was not associated with reduced risk of SSI (OR=0.66, p>0.05).
Delayed sternal closure independently increased patient risk for SSI by 15 times (OR=15.33, p=0.013).
Findings from this study suggest that enforcing appropriate prophylactic antibiotics alone in children
undergoing CVS may not be sufficient to prevent SSI. Further studies are warranted to determine if a
bundle approach including both prophylaxis and post-operative wound management would reduce the risk
of SSI in this population.
124
RIGHT HEMISPHERE CONTRIBUTION TO LANGUAGE AT THE COST
OF VISUALLY BASED SKILLS IN LEFT HEMISPHERE
LR Rosenberger MM Berl J Mayo D Weber GA Gioia N Ratner C Vaidya WD Gaillard
Epilepsy is known to have debilitating consequences on cognitive abilities. This study compares
neuropsychological performance on intellectual, reading, and memory measures. In addition, the
functional correlates of memory are explored in children with left hemisphere focus epilepsy during a
reading task
125
MOLECULAR TESTING EXPLAINS THE PRODUCTION OF ANTI-D IN
AN RH POSITIVE TODDLER
PP Pary S Vege CM Westhoff VR Criss EC Wong NL Luban
Molecular testing is widely used for HLA typing and infectious disease testing, but in Transfusion
Medicine, serological testing is still the standard. We present a case of a pediatric patient in which
molecular testing helped explain the serological findings. An 8 month old Haitian male was admitted for
symptomatic anemia. The CBC revealed a hemoglobin of 3.9 g/dL, and hematocrit of 14.6% with a
reticulocyte count of 52.4%. The blood type was O positive and the antibody screen was 2+ positive with
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all three screening cells by gel methodology. The patient had no history of transfusion. The antibody
work-up revealed a warm auto antibody in the plasma and a panagglutinin in the eluate supporting a
diagnosis of Warm Autoimmune Hemolytic Anemia (WAIHA). The patient received 4 aliquots of O
negative, leukoreduced, irradiated, CMV negative red blood cells from a single donor unit and was begun
on steroids. Nine months later the patient returned with fever and a hemoglobin of 3.2 g/dL and
hematocrit of 10.7%. No additional antibodies were identified by the reference lab, and 2 more aliquots of
O negative, leukoreduced, irradiated, CMV negative RBCs were transfused. One month later the patient
was re-admitted, and testing by the reference laboratory revealed a strongly reactive anti-D following
adsorption of cold and warm autoagglutinin from the plasma on allogeneic cells. Since the patient was
D+, molecular testing was requested which demonstrated the presence of two altered RHce and RHD
genes. The RHCE genes identified were: RHCE*ceAR and RHCE*ce16Cys; the RHD genes were:
RHD*DAR and RHD*DAU-0. Although serologic typing did not hint to the presence of an altered D,
molecular testing revealed the presence of two altered RHD alleles in this patient, and explained how a
serologically normal D+ positive patient could have made an anti-D.
THE PREVALENCE OF INSULIN RESISTANCE IN SEDENTARY,
OBESE AND OTHERWISE HEALTHY MINORITY ADOLESCENTS IN
THE DC METROPOLITAN AREA
126
GM Many ME Hurtado GM Many JJ Park GI Uwaifo LJ D'Angelo EP Hoffman
There is a dramatic increase in obesity prevalence in pediatric populations, with many urban minority
populations showing among the highest rates in the US. While the association of insulin resistance (IR)
and obesity is well established in adults, IR and other metabolic syndrome components have been less
well studied in younger minority populations. It is postulated that a surge in the incidence of insulin
resistance, plays a major role in the development of type 2 diabetes in pubertal and post-pubertal
adolescents (Shulman et al., 2000). We initiated a study in our mainly African American (AA)
ambulatory adolescents to investigate the relative rates of insulin resistance and impaired glucose
intolerance. Methods: Participants were recruited from urban adolescent and subspecialty departments of
ambulatory services and through local community advertisements. Interested participants were screened
and invited for further eligibility determination and the consent process. Eligible subjects were sedentary,
BMI-for-age > 95th percentile, nonsmokers, not pregnant, having no history of chronic illness nor
medications known to affect glucose metabolism or preclude exercise. A 2-hr OGTT was administered;
fasting glucose and insulin and 2-hr glucose levels were measured. Results: Thirty-one subjects were
enrolled (5 M, 26 F). Sixteen (52%) of those screened met diagnostic criteria for insulin resistance
(fasting insulin > 17 ВµU/ml) and 1 met criteria for impaired glucose tolerance (2-hour OGTT plasma
glucose =140 mg/dl < 200 mg/dl). The HOMA insulin resistance index was significantly higher in the IR
group vs. non-IR group (6.09 + 2.26 vs. 2.50 + 0.84). Conclusion: We studied 31 AA teenagers with
BMI-for-age > 95th percentile and found that over half fulfilled the diagnostic criteria for insulin
resistance. Our data suggests that undiagnosed insulin resistance occurs at a high rate in obese AA teens.
Screening may be warranted to implement interventions and possible treatment for this population.
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DIETARY BEHAVIOR CHANGES IN A FAMILY-CENTERED
INTERVENTION PROGRAM FOR OVERWEIGHT LATINO YOUTH
N Mirza C Caroline M Palmer E Elsbury A Jaramillo J Yanovski
The high prevalence of childhood obesity in the US has been suggested to be due in part to unhealthy
dietary practices such as consumption of calorie dense low nutrient foods and excessive intake of
sweetened beverages. These dietary practices are common among Latino youth, who are at high risk for
overweight. The goal of the present stduy was to determine the effect of a community-based familycentered intervention program on dietary knowledge and behavior among overweight Latino youth.
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AN ETHNOGRAPHIC STUDY OF PROFESSIONAL CARE
APPROACHES AND ROLES WHEN DEALING WITH SEVERE AND
PROFOUND ILLNESSES
GR Lotrecchiano S Stuart
The major goals of the Leadership Education in Neurodevelopmental Disabilities (LEND) program are to
provide didactic and experiential opportunities for trainees, professionals, and families to understand
neurodevelopmental disabilities from an interdisciplinary perspective. Presently, the LEND program at
Children’s National Medical Center (CNMC) in Washington DC, is in the fourth year of its first funding
cycle. It supports 13 disciplinary foci that come together in an interdisciplinary collaboration. National
LEND curricular objectives share basic criteria. Professional approaches however, evidence knowledge
variability (sometime contradictory and at other times complimentary) which affects attitudes about the
care and treatment for children with severe and profound disabilities. This variability spectrum causes one
to question the mechanisms and processes which shape professionals attitudes and approaches when
dealing with these special cases. “What is their personal history and process providing them with
information about how to serve children with chronic, severe illnesses?, “What experiences are shared
between and across specific disciplines that identify trends in career development?”, and “What part does
variation in these trends play in achieving a truly cross-disciplinary vocabulary of knowledge between
practitioners and professionals?” In essence, an inquiry into the variability of knowledge among
professionals provides a two-part challenge. Firstly, there is a need to identify individual training and
experience in different disciplines which make up the interdisciplinary team (AIM 1). This phase of study
will provide insight about how these disciplines differ in approach and vocabulary. Secondly, after
identifying these disciplinary “vocabularies” of knowledge, the investigation turns to trying to understand
how these professional trends support and/or contradict each other. Ultimately, the research will lead to a
better understanding of the trending of training and mentoring needed for more integrated
interdisciplinary care (AIM 2).
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HEALTH LOCUS OF CONTROL AND FACTORS THAT INFLUENCE
AFRICAN AMERICAN WOMEN'S BREAST CANCER EXPERIENCE
PA Broadnax
Breast cancer is the most common cancer among women in the United States. While the incidence of
breast cancer is greater for white women, the mortality rate is higher for African American women. In the
Washington area, 1 out of 6 women with breast cancer will die from the disease (ACS, 2007). Factors
contributing to a late diagnosis and poor outcomes are decreased access to health care, lack of utlization
of early detection methods, disproportionate rates of poverty and perceived inferior medical care (ACS,
2007; DHHS, 2003;NCI, 2007). Using a triangulation design, the aim of the study was to describe
African American women's experience with breast cancer and identify possible delays in obtaining care.
Data was collected using a demographic form, Multidimensional Health Locus of Control survey and
focus groups. The sample was recruited from various non-institutionally connected sites. The women
were middle-class and well-educated professionals who had medical insurance at the time of diagnosis.
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The majority of the women were diagnosed in Stage I. Descrptive statistics were used to describe sample
variables but correlations could not be established due to the small sample size. Recurrent themes in the
focus goups were identified. This sample of women did not experience delays in obtaining healthcare for
breast cancer. The study did underscore the importance of engaging in health promotion in the early
detection and treatment of illnes. The relationship between the patient and physician was paramount to
accessing a coordinated plan of cancer treatment. For additional study, I recommend that women who did
not have positive treatment experiences be surveyed to identify areas for improvement in the care of
African American women with breast cancer.
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MAPPING IMMUNIZATION RATES IN WASHINGTON, DC
M Nguyen R Engstrom N Cowan L Fu
Despite significant improvements in overall childhood immunization rates in the last decade, undervaccination is still a significant concern among poor, urban American populations. Healthy People 2010
specifies a goal of 90% vaccination rate for all children. Recent data from the CDC reports immunization
completion rates for children ages 19-35 months in the District of Columbia was 78% in 2006.
Differences in socioeconomic status, cultural beliefs and access to care affect population vaccination
rates. As a result, neighboring communities that differ in regard to these factors may have very different
immunization rates despite being geographically near each other. The aim of the present study is to map
the location of under-immunized neighborhoods in the District of Columbia.
131
AUTISM RESOURCES FOR CHILDREN UNDER 3 YEARS OF AGE IN
THE DC/METRO AREA
L Sanchez M Oconnell G Martin P Glass
Autism is a neurodevelopmental disorder that affects approximately 1 in 150 children in the U.S.
(ADDM, 2007). Autism is generally considered to be a spectrum of disorders that is characterized by
deficits in social interaction, language, and restricted pattern of interests/behaviors. In the past decade
there have been many advances in the sophistication of diagnostic tools, and increasing awareness in the
scientific and general community of the prevalence of this disorder. As a result, there has been a surge of
research in an effort to describe the genetic/neurological basis of the disorder and discover methods of
effective treatment. Although there is no known cure for autism, there are educational and behavioral
treatments that are proven to improve core symptoms of autism as well as adaptive functioning.
Importantly, earlier detection and intervention are associated with better prognosis (AACAP, 1999). It is
unclear to the extent to which the rapid progress of scientific findings has kept pace with educational and
treatment programs offered in the community for young children with autism. The purpose of the present
study was to compare the National Research Council’s (2002) recommendations for treatment of young
children with autism spectrum disorders and results of published scientific studies with the programs and
services offered in 8 early intervention programs (serving children birth to age 3) in the local communities
served by CNMC. A critical analysis including implications and barriers to effective treatment will be
discussed.
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DEVELOPING A RESOURCE GUIDE FOR SIBLINGS OF CHILDREN
WITH DISABILITIES
132
BR Bordonaro
This project was developed to increase awareness of services available to siblings of children with
disabilities in the metropolitan area. Research on the specific needs of the population was considered in
preparing this project. Siblings of children with disabilities are affected in many positive and negative
ways. Stalker and Connors (2003) reported children experience a variety of feelings about their sibling
with a disability from fondness and loyalty to irritation and embarrassment. These feelings can be a result
of the children’s own thought processes, interactions with their sibling with a disability and parents, or
interactions with peer groups and society. Often the children are forced to bridge the gap between their
“normal” lives, and their different families on their own. Stalker and Connors (2003) found that some
siblings have little difficulty while others struggle greatly. Those that struggle may experience negative
cognitions that could follow them into adulthood. There is a need to address the social and emotional
needs of siblings of children with disabilities in order to ensure their wellbeing.
CAN PRENATAL EDUCATION PROGRAMS BE ADAPTED FOR
WOMEN WITH MILD INTELLECTUAL DISABILITY?
133
R Margolis
Women with mild intellectual disability (ID) are at a severe disadvantage when it comes to having a
successful pregnancy as well as successful childbirth and parenting experiences; however, little attention
has been paid to the pregnancies of this particular population. Research has shown that the rates of sexual
activity and pregnancy among women with mild ID are similar to the rates of sexual activity and
pregnancy among women of typical intelligence. Unfortunately, research has also shown that individuals
with ID know significantly less regarding pregnancy and childbirth than the general population. Mothers
with ID are at a higher risk of facing additional challenges including poverty, lack of support and lack of
resources on pregnancy and parenthood. In addition to the challenges faced by mothers with ID, there are
known risks to children associated with maternal ID. These risks include maltreatment, inadequate
parenting due to lack of parental knowledge, poverty, and intellectual disability. Given the challenges that
mothers with ID face and the risks of maternal ID to the child, it is clear that pregnant women with ID
could benefit from some type of intervention, and while several interventions for parents with ID have
been developed in the past thirty years, none of them focus on the time period of pregnancy. At the same
time, numerous prenatal and early childhood parenting programs have been developed for parents of
typical intelligence. This project examines a number of these programs with an eye toward adapting them
for use with pregnant women with mild intellectual disability.
RESOURCE GUIDE FOR FAMILIES OF CHILDREN WITH AUTISM IN
THE WASHINGTON, DC METROPOLITAN AREA
LC King
In light of the recent rise in the diagnosis of Autism in childhood, increasing numbers of children and
families are in need of specialized assessment, diagnostic, and treatment services in a variety of
disciplines. The diagnosis of an Autism Spectrum Disorder (ASD) often entails difficulties in several
areas, including speech/language delays, repetitive and/or inflexible behavior(s), and a primary difficulty
with negotiating social interactions. As a result of the wide array of challenges faced by children with
ASD, parents may have difficulties locating adequate services to address all of areas of need. Also,
service provision to children with ASD is often disjointed because effective services must be provided by
professionals in different disciplines and, most often, in different locations. Families would greatly benefit
from being able to access a list of resources which includes service providers in their geographic area, as
well as other supplemental materials, including books and websites offering support for children with
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Autism and their families The Georgetown University Medical Center-Autism and Communications
Disorders Clinic provides a multidisciplinary approach to assessment and diagnosis of ASD. The primary
objective of this project was to compile a list of the resources and service providers recommended by the
Georgetown University Medical Center—Autism and Communications Disorders Clinic, supplemented
by additional community resources. This resource guide will give the Clinic team a cohesive guide of
individual/group providers, agencies, and written and electronic materials to offer to the families they
serve within the Clinic. Furthermore, the resources will be organized by county and specialized area of
service provision, thereby presenting a great deal of information in a user-friendly format in an effort to
facilitate families locating effective services for their children. The grouping of resources by locality also
highlights the relative differences in service options available for families based on geographic residence.
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ON THE SAME PAGE: A WEB-BASED TOOL TO FACILITATE
INTERDISCIPLINARY COLLABORATION FOR A CHILD WITH
SIGNIFICANT NEURODEVELOPMENTAL DELAYS
LE Emma JB Burns MJ Jarrett
Interdisciplinary collaboration is an evidence-based practice in the provision of services for young
children with disabilities (Drotar, 2004). However, multiple systemic barriers challenge and often impede
interdisciplinary collaboration, both within and across educational, therapeutic, and medical institutions
(Pringle, Levitt, Horsbough, Wilson, & Whittaker, 2000). For young children with significant
neurodevelopmental disabilities, parents face the daunting challenge of translating and transporting key
information regarding intervention and treatment protocols from provider to provider. The goal of the
present project was to design and create a web-based tool to facilitate interdisciplinary collaboration for
team members who support a young child with significant neurodevelopmental disabilities; the evidencebased principle of family-centered care and regulations protecting the exchange of private medical and
educational records informed and guided the development of the site.
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EFFECTS OF ABUSE ON MATERNAL MOOD DISORDERS AND
TREATMENT-SEEKING BEHAVIORS: PRELIMINARY ANALYSES OF A
COMMUNITY SAMPLE OF AFRICAN AMERICAN MOTHERS
A Barber C Ronzio
Mood disorders in the postpartum period have a negative impact on the mother, her social role
functioning, and infant well-being and development. Incidence of maternal mood disorders has been
reported to be up to three times as high in African American mothers compared with White mothers.
African American mothers are also less likely to seek mental health treatment compared with White
mothers. In order to gain a better understanding of maternal mood disorders in African Americans, this
project examined specific psychosocial issues common to African American women as well as factors
associated with maternal mood disorders in general. Experiencing abuse is associated with an array of
adverse social and emotional outcomes; it is also a risk factor for developing postpartum depression
(Elliot et al., 2005). Preliminary data from this project show 30% of all participants reported a history of
abuse. Literature suggests that social support can assist many African American women cope with
adverse events, such as abuse, and social support has been found to be a protective factor for maternal
mood disorders (Todd & Worell, 2000; Ugarriza,1996). The goal of this preliminary analysis was to
determine the association between abuse during childhood and adulthood and maternal mood disorder and
mental health treatment-seeking behavior. We also examine whether social support reduces the impact of
abuse on mothers’ mental health.
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QUALITY CARE: CREATING A COMMUNICATIVE ENVIRONMENT FOR
THE COMMUNITY PHYSICIANS WITH THE DIVISION OF SURGERY
137
KT Hoff B Mikesell E Lamb
The purpose of this pilot was to target a group of patients admitted to the general surgery service to
improve the quality of our communication at the time of admission and discharge with the referring
physician. The diagnoses of pyloric stenosis and trauma/burns were targeted for this pilot because these
infants and children require follow up either post-operatively to ensure weight gain and hydration, or post
admission for follow up for their injury. For these reasons, communication is critical between the services
caring for the child. In addition, this will improve quality and continuity of care and communication
among the medical community treating a particular patient. At admission, information regarding the
referring physician was obtained by the physician assistant or nurse practitioner. This information was
cross-referenced with the most current database at CNMC. An admission facsimile was sent with both
contact information from our department and admission information regarding the patient. Patient
information included name, date of admission, diagnosis, and any studies completed to confirm diagnosis.
Surgical contact numbers were provided as well. At the time of discharge, a discharge summary was
faxed to the same provider. Verbal communication was an option offered to the referring pediatrician on a
24-hour basis. Data were collected regarding the date, physician name, office number, address, and
facsimile number as well as any response from the pediatrician. A short survey utilizing a Likert rating
scale was utilized to gain further information regarding satisfaction with the process. Future impact of this
pilot includes implementation of a standardized communication tool for all patients admitted to the
Division of Surgery and Trauma service, and maintaining an up-to-date database of community
physicians to improve overall communication and, thus, enhance quality and continuity of care for
children.
WALK AND TALK YOUR WAY TO HEALTH: A PILOT PROGRAM TO
INCREASE ACTIVITY AND NUTRITIONAL KNOWLEDGE
L Wagner G Ukpabi S Keller J Morris
Overweight and obesity rates are rising in the United States (https://www.cdc.gov/PDF/Facts_About_
Obesity_in_the_United_States.pdf accessed July 21, 2006). Our proposed program was in accordance
with Healthy People 2010 goals which include, increasing the proportion of worksites that offer
comprehensive employee health promotion programs and increased employee participation in employersponsored programs. The purpose of the study was to increase activity and nutritional knowledge of
CNMC employees. The sample consisted of 50 female CNMC employees. Participants joined one of two
30 minute walking sessions scheduled per week. Topics for discussion (with attendant links), were sent to
participants using the internal e-mail system. Handouts were made available to participants with no access
to e-mail.The topics were discussed at monthly group meetings. Participants completed an activity and
nutritional questionnaire pre and post study. Their Height, Weight and Blood Pressure were measured pre
and post study and monthly. The Waist and Hip circumferences were measured pre-study and at months
1, 3, and 6. The BMI was calculated monthly and the waist to hip ratio calculated pre-study and at months
1, 3 and 6. The average weight loss for the group was between 0.23 kg. and 2.31 kg with participants who
exercised between 4-6x week losing the most. Thirteen participants who completed the nutritional
questionnaire at the end of the study either improved or had no change in their dietary habits. The project
helped a small number of motivated employees become more active,learn more about nutrition and
maintain or lose weight. The aim of the project is to be a springboard for future wellness events which
will include an on-going worksite wellness program to include the entire CNMC network.
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EXAMINING KNOWLEDGE GAINED IN A LATINO PARENTING
PROGRAM
M Palmer M Hoyos N Mirza
Parent Management Training (PMT), or the use of didactic instruction, modeling, role playing and home
practice, is a common practice employed to teach parenting skills for child encouragement, monitoring
discipline, and parental problem solving. PMT is considered one of the most efficacious outpatient
treatments for childhood behavior problems. Despite its common practice among many communities,
there is limited research examining the effect of PMT within the Hispanic community. In the US, Latino
families confront challenges with language and social lifestyle barriers. These result in difficulties with
their children such as school problems, disruptive behavior, and poor self-esteem, difficulties that have
been associated with poor parenting skills in an American context. The goal of this present study is to
determine the effect of the program in increasing the knowledge acquired by participating parents.
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PROTEIN-PROTEIN INTERACTIONS BETWEEN NACETYLGLUTAMATE SYNTHASE (NAGS) AND
CARBAMYLPHOSPHATE SYNTHETASE I (CPSI) IN THE REGULATION
OF UREA CYCLE
N Haskins H Morizono L Caldovic
N-acetylglutamate synthase (NAGS, EC 2.3.1.1) catalyzes formation of the N-acetylglutamate (NAG), an
essential allosteric activator of carbamylphosphate synthetase I (CPSI) in mammals. CPSI and ornithine
transcarbamylase (OTC) catalyze the first two steps of ureagenesis in mammals. CPSI is presumed to be
the rate-limiting enzyme of the urea cycle. Therefore, NAGS could regulate ureagenesis by supplying
variable amounts of NAG for activation of CPSI. The amino acid sequences of mouse NAGS consist of
three regions with different degrees of conservation: the mitochondrial targeting signal (MTS), the
variable segment, and the conserved segment. Removal of the MTS results in mature NAGS (NAGS-M)
while removal of the MTS and the variable segment results in conserved NAGS (NAGS-C). Our goal was
to examine if the three mitochondrial enzymes of the urea cycle, NAGS, CPSI and ornithine
transcarbamylase (OTC) form a multiprotein complex that could explain channeling of the urea cycle
substrates observed by other researchers. Previously OTC and CPSI were shown to interact. Because
NAGS provides an essential cofactor for CPSI activity, we hypothesized that NAGS interacts with CPSI,
and used co-immunoprecipitation to test our hypothesis. We found that only NAGS-M interacts with
CPSI. The variable segment was capable of completing with NAGS-M and displacing it from the
complex with CPSI. Mitochondrial fractionation and western blotting were used to determine localization
of the multiprotein complex in the liver mitochondria. Significant fractions of NAGS-M, CPSI and OTC
were found to be loosely associated with the inner mitochondrial membrane, while NAGS-C binds
strongly to the membrane. These results suggest that distribution of NAGS, CPSI and OTC in the
mitochondria is not random and may contribute to high efficiency of ureagenesis.
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THE RELATIONSHIP BETWEEN PERCEIVED SUSCEPTIBILITY AND
HIV SCREENING
DS McCoy MA Kadeshe CG Lawrence
Despite the Centers for Disease Control and Prevention’s recommendations and the fiscal and clinical
feasibility of routine human immunodeficiency virus (HIV) screening, HIV continues to go undiagnosed
resulting in increased transmission and mortality. This study used secondary data from the 2002 National
Health Interview Survey. Chi-square analysis was conducted to examine the relationship between
perceived HIV susceptibility, as defined by the Health Belief Model (HBM), and HIV testing of
respondents’ ages 18-25 living in the Southern region of the United States. There was no statistically
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significant relationship (p=0.10) between perceived susceptibility and HIV testing, however a significant
correlation (r = 0.28) existed. Of the respondents who had received HIV testing, 56% identified routine
medical or prenatal care as the main reason for testing. Conversely, 67% of respondents who had not been
tested, reported “unlikely to have been exposed to HIV” as the main reason for not being tested. These
findings suggest that universal routine screening may promote early diagnosis and treatment of HIV.
START EARLY, START RIGHT: CHANGING THE TRAJECTORY OF
CHILDHOOD OBESITY IN THE LATINO COMMUNITY
AM Jaramillo M Palmer C Collins N Mirza
Ana Maria Jaramillo, Matilde Palmer, Caroline Collins,BS, and Nazrat Mirza, MD, ScD, Start Early,
Start Right: Changing the Trajectory of Childhood Obesity in the Latino Community The prevalence of
obesity and its complications are highest among Latino children and adolescents. Obesity affects all ages,
with recent national surveys showing increased prevalence of overweight among preschoolers. There is
evidence to suggest that dietary and physical activity patterns are established in childhood, which may
explain the association between obesity in childhood and adult obesity. Parents appear to have a strong
influence on diet and activity behavior of children. The effect can be either direct, by facilitating the right
environment, or indirect, through modeling. From a public health perspective, prevention of onset of
overweight from an early age, and before the child has developed unhealthy eating and inactivity habits,
are more likely to be effective. The goal of this study is to determine the efficacy of a program designed
for parents of Latino preschoolers to treat and prevent childhood obesity through promotion of healthy
dietary practices, increasing physical activity and the reduction of sedentary behavior.
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SUPPORT STAFF EXPERIENCES ON SPECIALIZED HOSPITAL
TEAMS
RJ Roberts W Thompson
Unlicensed personnel on special teams of licensed health care staff do not always speak up, render
feedback, participate equally with the rest of the team, or interact with outside staff as equals. A
phenomenological interview method was used in this qualitative study to answer the central research
question: How do support staff on special hospital teams experience being on the team? Interviews with
two support staff with 5 years of experience were audio taped and transcribed. Trustworthiness was
ensured using epochГ©, peer review of coded data, peer debriefing, triangulation, member checks, and
thick description. Analysis of interviews revealed common textural descriptions of the following
experiences: busy-ness, working with different personalities, unexpected schedule changes, multiple
expectations, learning, pride in the service provided, and changing team dynamics. Common structural
descriptions included: responses to workload, responses to different personalities, communication as a
struggle, response to the unexpected, feeling empowered to speak up, dealing with personal emotions, and
personal growth. Composite structural and textural descriptions resulted in identification of a key theme:
communication. Active learning, enhanced team communication and participation, and a personal positive
outlook are the primary drivers maximizing the experience of support staff. This study had clear
implications for staff development, patient care and further research.
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USING A PEDIATRIC HOSPITALIST ON A SUBSPECIALTY SERVICE:
UTILIZATION OUTCOMES
BF Fine WP Pastor BK Kerzner
The GI department at CNMC employed a pediatric hospitalist for three year to care for inpatients. While
allowing for the GI specialists to see more outpatients and perform more procedures, the hospitalist
showed decreased length of stay, fewer insurance denials, and and fewer unplanned readmissions.
Trainees, staff and patients all found the intervention to be successful.
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IMPLEMENTATION OF A GLOBAL CHILD HEALTH CURRICULUM FOR
PEDIATRIC TRAINEES
NS Shah MR Rosenberg
Most of the world's children along with the greatest burden of disease exist in the developing world.
Pediatricians therefore need to be trained as global physicians, with capacity and knowledge to improve
healthcare for all the world's children. Few pediatric residency programs offer formal training in global
child health. The objectives of this intervention were to assess the need for a global child health
curriculum at one tertiary-care, academic pediatric hospital and to examine the impact of a one-week
elective on knowledge of participants. Ninety pediatric residents were surveyed to assess needs, barriers
and interest in global child health. Over 90% of those surveyed felt that formal training in global child
health was needed as part of their residency curriculum. Based on these results, a formal curriculum was
developed and implemented. The major component of this curriculum was a one-week intensive didactic
elective comprised of didactic lectures, faculty-led discussions and a laboratory practicum intended to
increase knowledge about basic global child health topics. Participants completed a pre- and post-test to
identify knowledge deficits and to determine impact. Ten trainees completed the course. There was a
statistically significant increase in scores after the one-week elective, both by participant and by subject
category. In conclusion, pediatric residents at this institution perceive a need for formal training in global
child health. The one-week elective demonstrated positive impact on knowledge of trainees. Further
research is needed to determine the long-term effects of this curriculum on knowledge, international
experiences and career choice.
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EXPERT MENTORING TO CHANGE PRACTICE WITH EVIDENCE:
FAMILY PRESENCE DURING CARDIOPULMONARY RESUSCITATION
146
CE Guzzetta AP Clark
Families are not allowed to be present during emergency situations because it is feared they will lose
emotional control and interrupt patient care. Yet, scientific evidence to support these fears is lacking. Our
aim is to summarize the impact of expert mentoring on family presence during resuscitation in changing
clinical practice and policy with evidence. Our work has included a variety of systematic processes. 1)
Mentoring nurses to conduct research: As mentors for research teams at Parkland Hospital and Children's
Medical Center Dallas, we guided some of the first studies to evaluate the effectiveness of existing family
presence guidelines. We studied over 100 family presence events and demonstrated that patient care was
not interrupted and benefits for family members were clear. 2) Mentoring nurses to publish research
findings: Research teams at Parkland and Children's have shared their work by publishing multiple
articles about processes and findings. 3) Disseminating research evidence to consumers: Our findings
have been widely publicized to introduce consumers to family presence. Results appeared in US News &
World Report, Newsweek, USA Today, Washington Post, and New York Times. Findings aired on all
major television stations, reaching an estimated total audience of 8,636,000 and appeared on Good
Morning America, NBC Dateline, ABC World News Tonight, and CNN. 4) Facilitating ongoing research
by others: Healthcare providers throughout the United States and the world have requested permission to
use our family presence policies, family presence attitude surveys, and family presence research tools. 5)
Developing national guidelines on family presence: We developed ENA's 2007 Family Presence
Guidelines and the American Association of Critical-Care Nurses' 2008 Family Presence Practice Alert.
Family presence is an option, not an expectation, but based on current evidence, it should be available to
all those who want to be offered the choice.
LEARNING TO CONNECT: STUDENTS' REFLECTIONS ON DOCTORPATIENT INTERACTIONS
147
T Kind VR Everett MC Ottolini
Background/Objective: Fostering humanism in medical education remains challenging. Reflective writing
is an established method for teaching medical students empathetic interactions, though little is known
about students' reflections on connecting with patients during the clinical clerkship. The purpose of this
study was to describe factors that medical students perceive contribute to or detract from making
connections with patients and families. Methods: Reflective essays submitted by third year medical
students about interacting with patients and families during their pediatric clerkship were qualitatively
analyzed for thematic content. Results: Major themes emerging in the 44 essays analyzed included time,
knowledge, language and culture, actions, personality, and feelings. Barriers to connecting for some
students were considered resources by others. Critical reflection was present in 31.8%. Conclusions: The
reflective process can be modeled on the pediatric clerkship. Medical students, amidst their clinical
responsibilities, can reflect on how, why, and whether or not connections with patients and their families
have occurred. Practice Implications: Recognizing what factors medical students perceive as enhancing
and detracting from connecting with patients and families will help preceptors foster those connections
and mitigate barriers. We recommend the inclusion of brief reflective writing exercises during clinical
clerkships in medical school.
RECOMMENDATIONS FROM BEREAVED PARENTS FOR FAMILYCENTERED PEDIATRIC PALLIATIVE CARE
R Selove JP Brooks KD Caminiti DL Dokken
As part of quality improvement efforts, pediatric hospitals with strong family-centered care policies and
programs solicit parents’ input about the quality of care. We offered bereaved parents and guardians of
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133 hematology-oncology patients the opportunity to complete a survey by mail, and a randomly-selected
sample was invited to participate in focus groups. Our qualitative study aimed to evaluate the degree to
which we have delivered family-centered care, and to obtain parents’ recommendations for improving the
quality of care we provide. Sixteen completed surveys were returned, and seven parents participated in
two tape-recorded focus groups. Despite the relatively few numbers of parents who participated, the
project was considered to be successful. Parents were glad that staff members asked for feedback and
suggestions for improving care provided to other families. Parents welcomed the opportunity to meet with
staff members who had cared for their child, and appreciated the opportunity to meet with other bereaved
parents. Parents who participated in our study advised us that the primary indicators of quality care are: 1.
Staff are experienced as trustworthy and competent by parents and their children. 2. Communication
along the course of the child’s diagnosis, treatment, and during bereavement among members of the
health care teams and with parents and their children is adequate for meeting family members’ needs. 3.
Parents and their children are offered a variety of supportive services from the hospital and community
agencies. 4. Hospital staff members assess the unique needs of each family, and are able to identify and
provide integrated services that match the specific needs of patients and their family members.
Conclusion: Focus groups are a powerful tool for obtaining information from bereaved parents, and for
communicating to parents that their experiences and point of view are valued by the institution that
provided care.
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URGENT RIGID BRONCHOSCOPY FOR REMOVAL OF PLASTIC
BRONCHIAL CASTS IN A TODDLER WITH FONTAN PHYSIOLOGY
SV Verghese MJ Jackson JV Vaughns DP Preciado
Plastic Bronchitis is a condition where solid bronchial casts of lymphatic origin form in the tracheobronchial tree producing airway obstruction. A 21 month old 12 kg child s/p Fontan operation at 18
months of age and a pacemaker for recurrent arrhythmias was admitted to the hospital with coughing and
dyspnea and deteriorating O2 saturation,. A chest x-ray showed total white-out of his right lung with
worsening tachypnea/ desaturation and he was emergently intubated. In the ICU, a bedside flexible
bronchoscopy showed rigid white foreign body in the right bronchus. In the OR, with the patient in a
slight head-up position, monitors were placed and small doses of fentanyl /Sevoflurane and vecuronium
given prior to rigid bronchoscopy. A large white solid arborizing cast was then removed rapidly with
improvement in ventilation and oxygenation. He was transferred to the CICU sedated and ventilated for
48 hours prior to being placed on ECMO due to reformation of rigid pulmonary casts bilaterally. Severe
hemodynamic instability can occur when high mean airway pressure is used in an attempt to oxygenate
and ventilate Fontan patients. The Fontan physiology requires sufficient gradient between the Fontan
circulation and the pulmonary atrium to provide forward flow, regular rhythm, good contractility as well
as a competent tricuspid valve for optimum function. However, the solid rubbery cast in the bronchi lead
to acute airway obstruction, increase in PVR and low cardiac output, deterioration in oxygenation/
ventilation. Endobronchial lymph drainage may contribute to the formation of bronchial casts. Since no
single therapy for this condition has been found to be consistently effective, it carries a poor prognosis.
The cardiologists, pediatric intensivists and anesthesiologists should be aware of this potential life
threatening complication of plastic bronchitis in children after Fontan operation in order to diagnose and
treat this condition early.
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IMPROVED SCREENING FOR CYSTIC FIBROSIS RELATED DIABETES
IN THE PEDIATRIC AND ADULT CYSTIC FIBROSIS PROGRAMS
C George E Boeckler K Witzmann B Harkness
We aim to improve the nutritional status and respiratory function of CNMC patients with Cystic Fibrosis
(CF) by early identification and aggressive treatment for complications of CF, particularly CF related
diabetes (CFRD). The process begins with standardized screening for CFRD. The process ends with all
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patients having been screened by established protocol. By improving the process we expect to identify all
patients with CFRD, thereby expediting appropriate treatment.
UNILUNG VENTILATION / LEFT THORACOTOMY / LEFT MODIFIED BT
SHUNT IN AN ADULT WITH CRITICAL LPA STENOSIS & RIGHT
EISENMENGER’S SYNDROME
151
SV Valairucha SV Verghese
Modified BT shunt placement in adults with unilateral Eisenmenger Syndrome is a very challenging
situation for the anesthesiologist especially during one lung ventilation for thoracotomy. CD-a 38-yearold female was born with D-TGA, large VSD with common ventricle and underwent bilateral PA branch
banding at the age of 3. At 10 years of age, she underwent a takedown of a right PA band with atrial
septostomy. She developed severe dyspnea with increasing cyanosis over the past few years. At
preoperative evaluation, oxygen saturation was 45-60% on room air. Cardiac catheterization showed
unilateral Eisenmenger syndrome with severe pulmonary vascular disease in her right lung from excess
pulmonary blood flow and severe LPA stenosis from PA banding with normal PVR resistance in her left
lung. The BT shunt placement via left thoracotomy was scheduled to improve pulmonary blood flow
selectively to her left lung. Patient was intubated with double lumen endotracheal tube for one lung
ventilation, which she tolerated very well. Her oxygen saturation on 100% oxygen before shunt
placement, during one lung ventilation and after shunt placement were 72%, 71% and 77%. She was
extubated on postoperative day one and discharged on postoperative day seven. At one-month follow-up,
she reported significantly improved functional capacity and 63%oxygen saturation on room air. The
decision to proceed with a left thoracotomy was made because we speculated that her oxygen saturation
would be maintained well during one lung ventilation since V/Q matching might improve during right
lateral decubitus position. Because of severe LPA stenosis and further decreased perfusion of the nondependent lung, almost no blood flow to left lung would match well with absent ventilation of the left
lung during right lung ventilation. This was confirmed by the unchanged oxygen saturation before, during
one lung ventilation with or without left pulmonary artery clamping for shunt anastomosis.
EXPERIENCES THAT CONTRIBUTE TO CLINICAL PATHOLOGY
LEARNING BY PATHOLOGY RESIDENTS
EC Wong
The purpose of this phenomenological study is to understand what experiences effectively contribute to
clinical pathology learning for pathology residents. It is important for pathology residents to establish
competency in clinical pathology in order to provide optimal patient care, avoid costly litigation and
medical malpractice (Copeland, 2007). The literature is lacking on how resident experiences contribute to
learning clinical pathology. In order to understand what experiences are important to pathology residents,
a qualitative pilot study using phenomenology was undertaken. Participants included two senior
pathology residents who felt they performed well on the ASCP resident in-service exam. Interviews were
conducted using semi-structured format using predetermined questions, audiotaped and transcribed. Data
analysis performed according to Moustakas (1994). Epoche, member check, peer debriefing, peer review,
and thick description utilized to ensure trustworthiness. Based on this pilot study, major elicited themes
included reading, case review/discussion, laboratory test approval and laboratory teaching. Preliminary
implications for teaching clinical pathology suggest that there is a need to foster self-directed learning via
case review and discussion/presentations, promote test approval that is integrative/consultative, encourage
case based learning and Socratic method of teaching by faculty and provide opportunities for residents to
interact with technologists in review and observation of test procedures and to participate in laboratory
operations and inspections. Further phenomenological studies with additional residents are needed to
ensure that all experiences have been captured and to determine barriers to learning.
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COMPASSIONATE USE OF INTRAVENOUS OMEGA-3 FATTY ACID AS
AN ALTERNATIVE TREATMENT FOR CHILDREN WITH INTESTINAL
FAILURE-ASSOCIATED LIVER DISEASE
AL Trautman CK Klein LS Scavo GC Chai MR Revenis CT Torres
At Children’s Hospital Boston, an omega-3 fatty acid solution (Omegaven®) in place of standard
intravenous lipid reversed the course of liver disease in more than 50 infants, and in some cases prevented
liver transplantation. The U.S. Food and Drug Administration (FDA) has not approved use of
OmegavenВ®. However, FDA has given approval on a case-by-case basis for compassionate use to treat
infants with severe intestinal failure-associated liver disease (IFALD). This educational poster describes
the process for obtaining FDA approval and for proposed use of Omegaven® at Children’s National
Medical Center (CNMC). Infants will be selected for OmegavenВ® treatment based on specific criteria,
including progressive cholestasis, both a blood direct bilirubin > 3 mg/dL and requirement for total
parenteral nutrition for more than three weeks, and the failure of other therapies to reverse IFALD. After
the patient is selected and consent is received from the family, FDA approval must be established by
submitting an Investigational New Drug application, and the proposed protocol submitted to the CNMC
Institutional Review Board for expedited review. Once approved, the product will be obtained by the
CNMC pharmacy from the manufacturer located in Hamburg, Germany. The patient’s growth and lab
values will be monitored, including blood omega-3 fatty acids and close follow up of liver function tests.
The physician will evaluate the patient’s response to treatment and discuss either the continuation or
termination of the product with the family. These outcomes will be reported to the FDA. Expected results
are based on the findings of Children’s Hospital Boston, that Omegaven® use will reverse the course of
liver failure and prevent the necessity of liver transplants. Coordination of the new process for
compassionate use by physicians, nurses, pharmacists, and dietitians is essential to ensure successful
outcomes.
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EVALUATION OF THE PEDIATRIC CERVICAL SPINE IN TRAUMA
AE Egloff NK Kadom DB Bulas GV Vezina
The radiographic evaluation of the pediatric cervical spine in trauma varies significantly, raising the
concern for unnecessary studies. The purpose of the study was to improve the imaging evaluation
algorithm by doing a retrospective evaluation and applying the NEXUS criteria and Canadian C-spine
rule in a cohort of pediatric patients that came to the emergency department and had a radiographic
examination. One of the main problems encountered is the lack of documentation in the ER department,
making it difficult to assess the usefulness of the Canadian C-spine rule in the pediatric population. If the
NEXUS criteria is systematically used, classification of our population into a low and high risk for
cervical injury is possible and a reduction of the radiographic studies and the amount of radiation can be
obtained.
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WHAT NEXT? A GUIDE FOR FAMILIES WITH CHILDREN RECENTLY
DIAGNOSED WITH HEARING LOSS
LM Spies SE Markel
Little focus is placed upon guiding parents, teachers, school administrators, audiologists, and speech
pathologists to aid in a more comprehensive treatment of patients recently diagnosed with hearing loss.
Families are often faced with many hard decisions as the result of coping with a diagnosis of hearing loss.
Choices about communication options, listening strategies, and treatment options as well as
advancements in the field can affect families choices for treatment. Often, families do not have access to
these advancements and options. In response to this problem, a resource guide was created which is aimed
at helping families of children of deaf and hard of hearing patients explore choices for communication
methods. This information is presented in an unbiased way, listing services and support available to
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families and professionals. This compilation of resources includes a list of local practitioners, information
regarding communication choices and amplification options, questions to ask professionals, language
stimulation techniques for the home environment, and a resource guide including books, articles, and
website addresses for further information.
COGNITIVE ADOLESCENT ANGER MANAGEMENT PROJECT
RE Bannerman L Rayala B Robertson S Anderson N Montiero
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Anger is a normal emotion experienced by everyone. Human beings react to feelings of anger in different
ways. Some people respond to it by turning their anger inwards which can be manifested through
isolating self, being quiet, and engaging in self-harming behaviors like cutting or getting involved in highrisk activities. Some people respond to it outwardly through outbursts, cursing, punching that may lead to
aggressive and violent reactions including hurting others. Societal and environmental factors and peer
pressure may affect adolescents’ responses to anger. It may be attributed to their developmental age and
maturity and learned behaviors from their environment. Some teen-agers may become self-destructive
and violent in an attempt to adapt to normalcy. As a result some of them may lack the appropriate coping
skills to adapt to stressful situations and will act out their frustration. Review of literature revealed that a
successful implementation of Cognitive Behavior Anger Management in adolescent care settings can
reduce acting out behaviors, aggression, and violence (Synder et al., 1999). Our purpose is to describe
CNMC’s new Cognitive Adolescent Anger Management Program (CAAMP) curriculum that was adapted
from the S.T.E.P. process developed by Snyder- Badau & Esquivel (2005). The goal of this program is to
help the participants from the Inpatient Adolescent Psychiatric Unit recognize the sources and triggers of
their anger. It also aims to create patient awareness about the outcomes of their responses and evaluate
alternative ways of handling difficult situations. The BECK Inventory Angry Scale will be used to assess
levels of anger before and after completing the CAAMP. The behavior outcome criterion is targeted to
developing a population who is more aware of the cues to anger and able to use coping skills to manage
their own anger before it results in self-harming behaviors, aggression and violence.
EXPLORING ACCESS BARRIERS TO EARLY INTERVENTION
SERVICES IN WASHINGTON, DC
157
E Thursby
This poster project explores the multiple barriers to receiving early intervention services in the District of
Columbia, using clinical case examples. The District of Columbia has one of the most restrictive
eligibility requirements for early intervention and serves fewer children than the two percent goal set by
the Department of Education. This project explicates the barriers to services and contradictions in policies
that limit the benefits of early intervention in the District of Columbia. Early intervention was designed to
coordinate the multiple services available to infants and toddlers with disabilities. In DC, this
coordination of services is missing leading to a scattered array of services that are not easily accessed by
families. The poster consolidates information to promote advocacy among providers and families.
COMPARING THE BAYLEY-II AND BAYLEY-III: ARE WE LOSING
CHILDREN IN NEED OF EARLY INTERVENTION?
MA O'Connell P Glass
The revised Bayley Scales of Infant Development, 3rd edition (Bayley-III) continues to be the gold
standard for assessing infant and toddler development. During development of the Bayley III, both the
Bayley III and the Bayley-II were administered in counterbalance order to children ages 1 to 42 months.
That preliminary study revealed that scores on the Bayley- III were approximately 7 points higher than
those on the Bayley-II. The developers suggest that these differences may be due to changes in the
demographic characteristics of the normative sample. However, these differences may also be the result
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of the normative sample now including children with Down Syndrome, Cerebral Palsy, Pervasive
Developmental Disorder, premature birth, and language impairment (for a total of almost 10% of the
sample). This inclusion of clinical cases to the normative sample seems to have “flattened” the normal
curve, thereby inflating scores. While Bayley-III standard score inflation is consistent with inflation in
other tests (WPPSI-III, PLS-4, and the PDMS-2), what does this mean for how professionals identify
children who are eligible for early intervention? The present study compares Bayley-II and Bayley-III
scores in a metropolitan sample of children ages 4 – 42 months. The Bayley-II and Bayley-III were
presented in counterbalanced order to all children (recruitment ongoing) and the Bayley-II MDI was then
compared to Bayley-III Cognitive Composite score. Preliminary analyses reveal significant differences
between scores, with Bayley-III scores averaging 12 points higher. These differences were larger for
children with scores below 75 on the Bayley-II MDI, or those with a 25% delay. This discrepancy could
mean that children eligible for early intervention services based on the Bayley-II would no longer be
eligible for services based on the Bayley-III. Implications for early intervention eligibility and suggestions
for practitioners will be addressed.
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CAPTIONS OF HOPE
159
LD Dukes
The aim of this project was to create an educational resource specifically for children coping with illness
in an inpatient hospital setting. The process of creating this book titled, "How Do You Feel: Captions of
Hope" took place at Children's National Medical Center in Washington, DC. A series of group art
therapy sessions were conducted over the time span of two months. Patients discussed their
personal feelings about coping with illness. Examples of their art work and captions of their personal
experiences shared were collected for the creation of this educational resource. There remains a great
deficiency of such resources geared specifically for the use of children. My goal is that copies of
this book would be made readily available both for the use of patients as well as non-hospitalized children
that could benefit from the shared experiences of others.
NEXT STEPS: A RESOURCE GUIDE FOR PARENTS OF CHILDREN
WITH AUTISTIC SPECTRUM DISORDER
160
JS Metz
The recent and dramatic rise in diagnosis of children with an autistic spectrum disorder is driving more
and more families and healthcare providers in search of established, new or alternative interventions,
therapies, and services. Pediatricians are utilizing screening tools to identify children at an earlier age.
School systems are examining their special education practices to ensure appropriate educational services
are in place at the preschool and elementary grades. Parents of children newly diagnosed with an autistic
spectrum disorder are confronted with multiple competing recommendations for therapies and
interventions, so much so that they can be easily overwhelmed with the amount of information required to
make an informed choice. At the same time, parents are being told that the earlier intervention begins, the
higher the outcome of in terms of development progress. The Next Steps Parent Resource Guide provides
parents of new diagnosed children with an autistic spectrum disorder with a summary of three
intervention modalities: DIR/Floor-time, Relationship Development Intervention, and Applied Behavior
Analysis. These three therapeutic modalities were chosen as a result of feedback from parents with
diagnosed children, a review of the literature on autism interventions, and the emphasis each places on
parental involvement and empowerment within the therapeutic setting. The resource guide provides a
general overview of each program, including: theoretical underpinnings; assessment practices; general
descriptions of treatment protocols; measurement tools and practices; parental involvement; ability of the
intervention integrate with other therapies and services; professional training and certification; research
with an emphasis on evidence-based practices; professionals in Northern Virginia, Maryland and
Washington D.C.; and resources (publications, associations, Internet-based groups and sites). The
resource guide is written for parents and places an emphasis on what is offered and expected of the family
in implementing one of the three interventions.
PEDIATRIC MOCK CODES: IMPROVING RESIDENT RESUSCITATION
D Friedman P Zaveri K O'Connell
BACKGROUND: Resuscitation of the acutely ill child is a necessary skill for pediatric residents. Data
show that lack of practice in resuscitation skills results in rapid deterioration of BLS and PALS
knowledge and increased anxiety and poor performance among nurses during actual resuscitations. The
effects of a residency-program-wide mock code program on involvement, anxiety, and leadership in
codes have not been studied. We hypothesized that after one year of a mock code program, pediatric
residents would report 1) increased participation in codes, 2) decreased anxiety and increased comfort
with knowledge needed in codes, and 3) increased likelihood of leading codes and feeling capable
running a code. METHODS: In this cross-sectional study of pediatric residents, anonymous surveys
evaluated personal assessment of involvement, comfort and leadership in codes before (PRE) and one
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year after (POST) a monthly mock code curriculum was implemented. The survey measured residents’
involvement in actual and mock codes (ranges) as well as self-reported levels of anxiety, knowledge and
leadership ability (Likert scale of 1-5) when faced with coding patients. In comparing PRE and POST
results, we use frequencies of ranges for objective answers and odds ratios for subjective responses.
RESULTS and CONCLUSIONS: After instituting a mock code program, residents participated in more
mock codes (p<0.001) and reported being more comfortable with their knowledge in codes (OR 2.5; 95%
CI 1.2-5.2). There was no significant change in the number of actual codes attended or led by residents, or
in anxiety levels or perceived capability to run a code. As resident comfort and experience increases with
several years of mock code experience, feelings of anxiety may decrease and involvement and leadership
in actual codes may increase.
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A REVIEW OF COCHLEAR IMPLANT WEBSITES
C Buxton S Stuart
Studies have reported that parents utilize media materials, including the Internet, to find out about
cochlear implants. The aim of this study is to evaluate materials about cochlear implants available on the
Internet and to provide a list of relevant resources for parents to use for their own research. Websites will
be reviewed to assess authority, accuracy, objectivity, currency, readability, and cultural relevance.
Conclusions will be made from a professional point of view regarding the relevance of these websites.
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NUTRITION, PHYSICAL ACTIVITY AND MANAGING OVERWEIGHT
CHILDREN AND ADOLESCENTS
NS Long
With overweight and obesity increasing rapidly in children and adolescents, there have been many efforts
and studies trying to determine how to best manage this pediatric problem. In conjunction with ongoing i
npatient research on childhood obesity led by R Carlisle, M.D. and P Lewis, M. D. at Holy Cross
Hospital, this REACH project attempts to consolidate recent information for primary care providers in
Montgomery County, Maryland to help manage overweight and obese pediatric patients in the outpatient
setting. In 2007, a committee convened by the AMA, HRSA and CDC released their “Expert Committee
Recommendations on the Assessment, Prevention and Treatment of Child and Adolescent Overweight
and Obesity”1. This project used these current guidelines and combined them with local resources and
various handout materials to produce a resource folder for primary care providers to use in their practices.
The final product of this project was a consolidated resource folder that was distributed in March 2008 to
multiple outpatient pediatricians who are affiliated with Holy Cross Hospital and work in Montgomery
County, Maryland.
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IMPROVING PATIENT SATISFACTION & ACCESS: NEUROLOGY
CLINIC
B Wiley
Patient satisfaction is an important aspect to providing quality, comprehensive health care services to
patients and their families. In an effort to improve satisfaction with the appointment scheduling process in
the Neurology Clinic, the phone-tree was redesigned to increase access to schedulers by decreasing the
amount of time it took to reach a live voice. Satisfaction with the new phone system was measured using
a validated, anonymous patient survey. The survey results demonstrate that further changes are necessary
and the phone system is not yet adequately meeting the needs of the patients of the Neurology Clinic.
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INTEGRATION OF WELLNESS AND DISABILITY EDUCATION IN
UNDERGRADUATE HEALTH PROFESSIONS CURRICULA
O Zavadska JB Riley
The number of persons in the US living with a disability continues to increase. As medical technology
advances and the baby-boomer population continues to age, this number is expected to grow even more.
Even so, health and wellness needs of persons with disability continue to be inadequately met. These
individuals continue to face attitudinal and structural barriers to accessing quality healthcare services. To
respond to the principles and goals of the 2005 Surgeon General’s Call to Action: Advocating for the
Health and Wellness of Persons with Disabilities, the authors developed a two-hour course for future
health professionals. The purpose of the course is to increase the capacity of future healthcare
professionals to deliver culturally sensitive and quality healthcare services to children, adolescents, and
adults with disability and to promote their holistic health and wellness. Initial evaluation showed that
students reported increased awareness about the holistic health and wellness needs of persons with
disabilities. Students reported increased knowledge about removing barriers to access and increased
communication skills.
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INTEGRATING YOGA INTO A PEDIATRIC PHYSICAL THERAPY
SESSION: OBSERVATIONS FROM THE FIELD
CH Wong
This project shows the holistic benefits gained from a comprehensive Yoga Practice that was designed to
be integrated into a pediatric Physical Therapy (PT) session for a 17 year-old patient with multiple
orthopedic diagnoses, obesity and chronic pain. The Yoga Practice for this project was designed to
include modified Yoga postures, Yoga breathing techniques and Yoga concepts. The Yoga postures were
selected and modified based on the PT evaluation to meet the goals for the patient and provide a fun and
challenging routine. The Yoga Practice was done over a four month period and showed improvements in
strength, endurance and range of motion. Additional benefits from this Yoga Practice extended into the
areas of pain reduction, home exercise compliance and participation in the community to reflect back that
the progress this patient made was holistic. This single-subject study showed that a Yoga Practice that
combines movement practices with mind-body practices is an effective complementary treatment
technique and is an appropriate therapeutic choice for this patient within a pediatric PT session. More
research is needed to bring the practices of Yoga postures and other mind-body practices within Yoga to a
larger population within Physical Therapy.
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RE-ENGINEERING THE CLINICAL RESEARCH ENTERPRISE: A
PROTOTYPE OF INTERDISCIPLINARY RESEARCH
NC Robinson CE Guzzetta SL Feetham PA Broadnax M Lee L Hardy E Dawson N Jairath
Nurse scientists in Washington, DC have formed the Washington Regional Nursing Research Consortium
(WRNRC) to address the integration of scientific evidence to inform nursing practice and improve
patient/family outcomes. WRNRC’s vision is to strengthen regional nursing research by fostering
collaboration between students, clinicians, and researchers. Our mission is to promote a nursing culture of
research and an environment conducive to inquiry/scholarship to advance clinical and translational
research. Our primary goal is to create a regional forum to improve patient care and healthcare quality and
increase the visibility/perceived value of nursing scientists. WRNRC membership includes researchers
from all DC regional nursing schools and healthcare systems. An outcome of WRNRC activities is to
better position nurse researchers to be integrated within the newly formed Washington Regional Institute
of Clinical and Translational Science (WRICTS) involving seven academic and clinical institutions.
Surveys responses from nurse researchers from participating WRICTS and WRNRC institutions have
identified their research interests, expertise, methodological specialties, funding sources, and research
mentors. This information will be placed in a regional research repository intended for identification of
knowledgeable multidisciplinary investigators, collaboration, and multi-center research and encourage the
development of strategies to enhance the conduct of clinical bio-psycho-social-spiritual research.
WRNRC activities include increasing access to clinical research by university faculty, establishing
mentors for researchers, providing clinicians with resources to serve as co-investigators, and facilitating
opportunities for collaboration and data sharing. In addition, we will develop a core research education
framework normally not emphasized in biomedical research. The WRNRC will lead this initiative to
bring disciplines together to generate a roadmap of bio-psycho-social-spiritual research. The described
process can be used as a prototype for integrating nursing science into NIH’s roadmap and developing
new organizational models of team science to re-engineer clinical and translational research and influence
the nursing research agenda of the future.
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REDUCING EMERGENCY DEPARTMENT THROUGHPUT TIMES
SD Doyle JH Hinrichs JM McKenna KB Brown CG Guzetta
Overcrowding in the Emergency Department (ED) results in treatment delays and families who leave
without being seen (LWBS) which increases the potential risk for negative patient outcomes. Patients
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who leave the ED without being seen (LWBS) usually do so because of long wait times (Kronfol et.al.,
2006). While LWBS patients are usually found to have a lower acuity and are more likely to receive
treatment elsewhere, there is a subset of that group that is ill enough to require admission to a hospital
(Goldman et.al., 2005). By implementing a more systematic, consistent assessment tool, the initial triage
process could be improved with resulting improvement in ED efficiency. The purpose of this study was to
determine if implementing a rapid triage Emergency Severity Index (ESI) assessment would decrease
LWBS rates, decrease arrival-to-triage times, and improve fast track utilization. The sample included all
ED visits March 2006 to December 2006 and all visits March 2007 to December 2007, a sample size of
approximately 120,000 patients. We measured the percent of patients leaving without being seen, average
arrival to triage time, and percent of patients utilizing fast track. Data collection for this study has been
completed and data analysis is in progress. It is expected that study result will show a decrease in arrival
to triage times from an average of approximately 30 minutes to less than 10 minutes, an overall decrease
in LWBS, and an increase in fast track utilization from approximately 20 percent to approximately 35
percent.
PROVIDER ATTITUDES TOWARD FAMILY PRESENCE DURING
INVASIVE PROCEDURES AND RESUSCITATION EVENTS IN THE
PICU/CICU
169
L Sanders G Gilmore H Greenlick B Pastor D Sawyer S Smallwood-Mason B Slavin J Walker
Family Presence is defined as the attendance of family in a location that affords visual or physical contact
with the patient during invasive procedures (IP) and resuscitation events (RE). Family presence, however,
remains a controversial practice in most critical care units. Multiple studies over the past 10 years
document the many benefits of family presence for families including removing their doubt about their
loved one’s situation and allowing them to see everything possible was done (Halm, 2005). In >600
family presence cases, no disruption of patient care by family members has been documented (ENA,
2007). Researchers, professional organizations, and consensus conferences recommend implementing a
written family presence policy based on national guidelines (Henderson, 2006) yet Children’s has no
policy in our PICU/CICU for families or staff. Our aims were to describe PICU/CICU provider attitudes
toward family presence during IP and RE and determine the level of support for a family presence policy.
A voluntary, anonymous, 15-question Family Presence Self-Assessment Survey incorporating a 4-point
Likert scale was used to measure the agreement with items that reflected attitudes about family presence.
Of 249 surveys distributed, 138 were returned (55% response rate). Most respondents were nurses (n=87,
63%) and physicians (n=18, 13%); the majority had > 3 years of critical care experience (51%) and many
had experienced families at the bedside during IP (41%) and RE (48%). Overall, providers had favorable
attitudes toward family presence during IP (72%) and during RE (84%). More than Вѕ reported they would
support a written policy for family presence during IP (77%) and RE (94%). Results of this survey
document that the majority of PICU/CICU providers favor family presence and would support a written
policy. These findings suggest a formal written policy for family presence during IP and RE should be
approved and evaluated for our PICU/CICU.
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LET'S GET MOVING
MC Birkmeier KF Lefert
It is well known that the United States has the highest obesity rates in the world. The increased incidence
of obesity is linked to the development of early onset diabetes and heart disease. With healthcare costs in
the US soaring, physical therapists can have a positive impact by promoting increased physical activity
and wellness. In honor of Physical Therapy month, the APTA Pediatric section encouraged PTs to
promote increased physical activity with their patients and families through a program called "Let's Get
Moving". The physical therapy department at CNMC expanded the idea to all CNMC employees. The
goal was to motivate and encourage increased activity levels for a 13 week period extending from
October through December. There were 317 participants with 47 individuals and 40 groups. The
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participants exercised for a total of 6947 hours. Overall, there was a positive response to the program and
further wellness programs are being developed for employees at the hospital.
171
DEVELOPING STANDARDS OF CARE FOR OBESITY PREVENTION,
SCREENING AND TREATMENT FOR CHILDREN WITH SPECIAL
HEALTH CARE NEEDS
PM O'Berry VE Schuyler SE Evans S Hassink M Weissman
The incidence of overweight and obesity in children with special health care needs (CSHCN) is greater
than that found among those who are non-disabled. (Rimmer et al 2007) A review of 100 patients with
muscular dystrophy or myelodysplasia seen in the Physical Medicine and Rehabilitation clinic at CNMC
indicated that greater than 60% were found to have a body mass index (BMI) of > 85th percentile. There
is a paucity of data in the literature regarding obesity prevention, screening and treatment of CSHCN
(Hassink, S. District of Columbia Citywide Obesity Summit, September 2007). Similarly, there is little
data regarding the associated co morbidities and their impact on the child’s primary condition. According
to Minihan et al (2007), overweight CSHCN require services and support beyond what is already in place
in the health care setting. In order to begin to meet the needs presented by these children, the Division of
Pediatric Rehabilitation, in conjunction with Children’s Obesity Institute, has joined forces with
nationally recognized child obesity researcher Dr. Sandra Hassink at Nemours/ A.I. DuPont Hospital for
Children in a collaborative project designed to: • Identify standard screening protocols for identification
and measurement of obesity in CSHCN; • Create evidence-based treatment pathways; • Develop
evidence-based prevention strategies; • Develop an obesity tool-kit for CSHCN that provides strategies
for family centered and culturally competent education at the patient, provider and community levels.
Future research will be conducted in areas identified through the project which hold potential to set the
standard for obesity prevention, screening and treatment for CSHCN.
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THE SELF REPORTED USAGE OF TOPICAL ANESTHETICS BY
REGISTERED NURSES DURING VENIPUNCTURE AT CHILDREN'S
NATIONAL MEDICAL CENTER
B Martin M Lee E Williams S Teach
A goal of Children's National Medical Center is to create a pain free environment for our families.
Children and their parents view venipuncture as a source of pain and fear in the hospital. Venipuncture is
a routine painful procedure that a child may encounter in a clinical setting (Kleiber, 2002).Research
studies have documented the perception of pain in the pediatric population and the post sequela trauma
some children experience after undergoing a painful procedure (May 1999;Paediatrics,2006). The purpose
of this pilot study is to assess nursing practice and usage of topical anesthetics such as L.M.X.4 cream and
Ethyl Chloride spray by Children's National Medcial Center Registered Nurses before venipuncture. The
overachieving goal is to develop an evidence-based nursing protocol suitable for implementation in the
General Clinical Rsearch Center. A protocol for minimizing pain during venipuncture is important to
promote family satisfaction and patient retention in clinical trials.
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CONSTRAINT INDUCED THERAPY: A PILOT STUDY
AT Turkus TN Nunnery LA Abrams CF Fox
Cerebral palsy is described as a non-progressive motor impairment caused by injury to the developing
brain. One of the most prevalent types of cerebral palsy is hemiparetic, characterized by unilateral upper
and lower extremity involvement. Constraint induced therapy (CIT) has been shown to improve the
function of the involved upper extremity in children who have hemiparetic cerebral palsy. The CIT
protocol includes 1) restraint of the non-involved upper extremity 2) intensive motor training of the
involved upper extremity. In this pilot study, we examined the efficacy of CIT when a modified treatment
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approach is used versus the previous interventions in other pediatric CIT studies. Sixteen children (8
males and 8 females; mean age 10 years 4 months; range 6-17 years) were randomized to receive either
CIT/motor learning intervention or conventional treatment with a home exercise program. After 10 weeks
of each treatment approach, the groups switched and received the opposite therapeutic intervention. In
addition, this study set out to describe guidelines that could be important for future researchers to consider
when working with children of different ages and functional abilities within the same group setting. The
protocol of this study involved restraining the uninvolved upper extremity in a fiberglass cast in which the
child had to wear during CIT sessions and during the home exercise program for a total of 17 hours a
week. A limited group of activities and games were used to help elicit specific movement patterns and to
promote improvement in various gross and fine motor skills.
TEAMWORK: IMPROVING CUSTOMER SERVICE AND NURSING
RETENTION
174
SW Weeks SR Randhawa
Teamwork is often cited as a key element for customer satisfaction, nursing retention and satisfaction, and
patient safety. Thomas, et al (2003) note that teamwork may not only serve to avoid error, but it may also
address the increasing staff shortages. Additionally, Kalisch & Begeny (2005) state that high performing
teams can improve outcomes in the areas of complex needs, patient care, staff satisfaction, enhanced
organizational effectiveness and overall healthcare. Recognizing the importance of teamwork on patient
safety, retention and safety, the Heart and Kidney Unit (HKU) planned and implement a teamwork
performance improvement project. The project included a teamwork retreat facilitated by Human
Resources staff; Children’s Core Principles were used as the guiding principles for this retreat. Pre and
post teamwork surveys were completed to evaluate the results of this project. The survey findings indicate
a 7% increase in overall teamwork results, with significant increases in specific core areas. Additionally,
since implementation, the unit has been awarded the Clinical Service Excellence award for most
improved scores in customer satisfaction and further decreased nursing staff turnover. Prior to
implementation turnover rates were 5%, post implementation turnover has decreased to 2%. The success
of this project not only highlights the importance of addressing teamwork in a nursing unit, but also
demonstrates how a structured approach to improving teamwork can influence customer satisfaction and
nursing retention.
THE EXPERIENCE OF NOVICE REGISTERED NURSES IN
RESPONDING TO MONITOR ALARMS
175
HA Walsh
Phillips (2006) maintains that clinical alarms occur to notify nurses that there has been a change in patient
status, whether to alert the nurse that a patient has experienced a vital sign outside of predetermined
parameters or that there is an equipment malfunction. Registered nurses must understand the implications
of each alarm in order to respond appropriately. Korniewicz, Clark,and David (2008) posit that nurses and
other health care providers should use alarms effectively to ensure safe clinical practice. Although many
studies have discussed clinical and safety issues related to patient monitoring, there have been no studies
that explore the experience of new graduate RNs in the process of responding to monitor alarms. A
phenomenological study explored the novice nurses’ experience in responding to monitor alarms.
SHARED NURSING LEADERSHIP AS A FORUM FOR NURSING
RESEARCH
176
EO Miller DK Morehouse DS McCoy CP Lloyd LM Williams BL Short J Paribello L Zell
Why don’t nurses do nursing research? The reasons are formidable – lack of mentors, knowledge, and
time, no allocated financial and human resources, and sheer fear. Our research journey represents an
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exemplar for the Magnet Designation Program because we successfully overcame these barriers. The
cornerstone of Magnet is its Share Nursing Leadership Councils that ensure nurses, at all levels, have a voice.
Our Neonatal Intensive Care Unit’s (NICU’s) Resource and Innovation Council became our voice in
identifying our neonatal research problem and provided the forum to begin our research journey. Our aims
were to develop a research protocol, obtain institutional approvals to conduct the study, and apply for research
funding. We knew we needed help. We sought the guidance of two nursing research mentors and our medical
NICU director and built a team of NICU nurses whose experience ranged from 3-20 years. We consulted with
our librarians, biostatisticians, physicians, and the Nursing-Research Advisory Committee. Our strong
interdisciplinary team then transformed our clinical challenge into a research problem. We dedicated over a
year to literature review, training in the protection of human subjects, homework assignments, deadlines,
discussions, debates, and editing to refine our nursing research protocol. We submitted our protocol to the NRAC (Nursing-Research Advisory Committee) and the IRB (Institutional Review Board) and obtained all
institutional approvals. We then prepared and submitted our application for a Children’s Research Institute’s
RAC grant award which provides research seed money for conducting studies. Our research journey has taught
us the impact of dedicated teamwork, importance of honoring our individual strengths and skills, and joy of
celebrating each of our milestones. Our journey continues as we look forward to completing data collection,
publishing and presenting our research findings, mentoring our NICU colleagues, and applying what we have
learned to our next study.
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A COLLABORATIVE NURSING AND PHARMACY INITIATIVE TO
IMPROVE PATIENT MEDICATION DELIVERY AT CNMC
SG Stanley BJ Simmons P Malkus S Wilson T Butler N Walsh H Toure L Nicholson A Conner B Dailey N
Hasan D McCoy L Williams-Greely L Carnegie L Talley J Cepero D Freiburg S Eades J DuVal S Sun C
Corriveau
An increase in missing dose requests generated by Nurses through the electronic medication administration
record (MAR) was evidence of a medication delivery system that had become complicated, inefficient, and
costly. With the onset of CTI, both Nursing and Pharmacy staff began using a newly implemented electronic
computer system. Unexpected work flow changes caused frustration and a lack of trust between the
departments which cascaded to delays in patient’s receiving their medications. A multidisciplinary task force
was created to identify the causes of the system problems and to implement changes for improvement. Several
methodologies were utilized to evaluate the current process: • Completed fishbone analysis of causes of
missing doses • Created a process flow and identified problem areas • Collected and analyzed data from
missing dose sheets • Interviewed nurses and pharmacists about missing dose process • Conducted detailed
analysis on the units and in the pharmacy with task force members • Completed FMEA to address process
issues identified on the process flow • Prioritized key findings and recommendations At the completion of the
analysis, an education steering committee of nursing and pharmacy staff was formed to design and implement
a comprehensive education program. Key problem areas identified in the analysis were targeted by the
education team. In addition, process flow in the Pharmacy was enhanced with new ADT reports, more frequent
batch reports, and recommendations to increase IV medication delivery to twice a day. Refrigerators on the
inpatient units were upgraded to accommodate more patient medications. The overall impact of the analysis,
education, and process flow improvements has decreased the number of credited doses and the requests for
missing doses by nurses. The cost for lost and credited doses has decreased significantly as well as the requests
for missing doses by nurses.
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EDUCATING AND ESTABLISHING GOOD TECHNIQUE AMONG ALL
RESUSCIATION PARTICIPANTS DURING CODES IN THE PEDIATRIC
INTENSIVE CARE UNIT
JL Walker B Russell T Hamilton E Marcelo E Felder A Wratney H Herrera CE Guzzetta
Resuscitation of a child in a critical care setting is a frightening and disturbing event. According to
Hamilton (2005) “Poor knowledge and skill retention following cardiopulmonary resuscitation training
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for nursing and medical staff has been documented over the past 20 years.” Implementation of mock
codes can improve a unit’s emergency response performance. A multidisciplinary team was formed
within the Pediatric Intensive Care Unit consisting of a PICU attending, fellow, respiratory therapist,
registered nurse, PALS instructor, patient care technician and the nurse manager. Ineffective
communication, poor role delineation amongst code participants, lack of code experience and lack of
individual confidence were areas that were identified as potential problems. As a team, it was decided to
implement random unannounced MOCK codes in the PICU setting. The purpose of having mock codes in
the ICU is to empower our staff to conquer codes with confidence and to improve our resuscitation
techniques. Currently we have one mock code per month. During the mock sessions the code blue bottom
is pressed. When the PICU team enters the room they are confronted with a simulation mannequin and are
presented with a patient scenario. This computerized mannequin can demonstrate signs of cardiac arrest.
The team that responds to the code is asked to proceed in the treatment plan as though this is a real event.
After each mock code, surveys were distributed to determine if the identified goals were met. Results of
this survey documented that the majority of PICU participants felt that having standardized mock codes
helped increase their confidence level. Findings suggest that conducting mock codes within the critical
care units results in improved confidence of individual team members, enhances teamwork, and leads to
effective personnel utilization during resuscitation.
IMPROVING EARLY RECOGNITION USING THE PEDIATRIC EARLY
WARNING SCORE
179
LH Hall SR Randhawa RC Cross
Early detection of the deteriorating patient has been linked to improved patient outcomes. According to
Tume & Bullock (2004) early warning scores have been used successfully in adult populations to
optimize patient outcomes, improve quality of life and decrease lengths of stay in ICU's. In 2001, the
Pediatric Early Warning Score (PEWS) was developed in England to address the growing concerns
surrounding patients at risk for deterioration. Since the development of the PEWS it has been successfully
used to improve detection of deteriorating pediatric patients. In a retrospective study, Duncan et. al (2006)
found that application of the PEWS tools would have identified more than 75% of the hospital’s code
blues if it had been implemented. Based on these findings, and the need to provide nurses with a
structured approach to detection and escalation, PEWS was implemented on the Heart and Kidney Unit
(HKU). Prior to implementation, nursing and medical staff were trained using SBAR communication and
educated on how to correctly use and interpret the PEWS tool and algorithm. Prior to implementation of
PEWS, the frequency of code blues during FY07 on HKU was 0.98/1000 patient days; the number of
codes since implementation has been reduced to 0.3/1000 patient days. Additionally, nursing staff have
increased the number of times patient care was escalated through the use of the rapid response team, also
known as the Clinical Assessment and Triage Team (CAT Team). In FY07, the CAT Team was activated
a total of 10 times, whereas current FY08 data highlights that the CAT Team has already been activated
14 times. Implementation of the PEWS has proven to be an effective tool for detection and escalation of
the deteriorating pediatric patient. Further investigation of the data will be necessary to determine if
PEWS has improved patient outcomes on HKU.
UPDATES IN TORTICOLLIS MANAGEMENT
180
AM Conway L Phillips SH Evans
Congenital muscular torticollis is described as idiopathic shortening of the sternocleidomastoid muscle
which results in a posture of varying degrees of severity of lateral flexion of the neck to the ipsilateral
side and rotation to the contralateral side. Clinical presentation and outcome is dependent on further
classification into three distinctly different categories. The sternomastoid tumor group (SMT) which
presents with a sternomastoid tumor. The muscular torticollis group (MT)presenting with tightness of the
sternocleidomastoid muscle but with no clinical tumor. And the postural torticollis (POST) used to
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describe individuals with all of the clinical features of torticollis but without demonstrable tightness or
tumor of the sternomastoid muscle (Macdonald,1969, Cheng, 2000). Classification is key to identifying
the clinical treatment pathway for successful outcome as different emphasis on further diagnostic workup,
specific therapeutic intervention and varied timelines are applied. Since 2004, the Department of
Rehabilitative Services at CNMC has sponsored a Torticollis Clinic providing comprehensive diagnostics
and coordination of clinical therapy services. The clinic is run by a pediatric nurse practioner and physical
therapist with consultative services of a pediatric physiatrist. Emphasis is on early identification, specific
classification and clinical intervention.
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NEONATAL AND PEDIATRIC WOUND CARE: FILLING THE HOLES IN
KNOWLEDGE AND PRACTICE
J Amling
There is no doubt there is a need for data to drive systemic quality improvement in the areas of neonatal
and pediatric wound care. National registries are needed to identify and quantify wound and skin care
issues in this population. It's not just about pressure ulcers but surgical dehiscence, diaper dermatitis, IV
extravasations, generalized skin rashes, etc. that lack the research to guide clinical practice. In a concerted
efffort at our hospital, an APS was devoted in the last fiscal year to examine the clinical practice, collect
data, and implement changes to the existing care. The data for each patient included in vs. outpatient; site;
wound etiology; hospital acquired or not; wound treatment; new patient or follow up; and amount of time
at each APS visit. An average of 15 patients were seen daily by the APS. Preliminary data shows that not
only was there heightened awareness to this vulnerable population but there was a reduction of the
hospital acquired "serious" wounds during this time period. Several strategies were implemented to
improve the clinical outcomes: a baseline prevalence study; implementation of a skin assessment tool;
standardized wound documentation; a Wound and Skin multidisciplinary team; weekly wound rounds:
new practice guidelines; new products; hotline available for suspected surgical site infections; in house
availability of specialty beds; and an education week dedicated to wound and skin care isues each spring.
This was a multidisciplinary effort to yield success despite all the services and personnel involved.
Commmunication was key to make sure the team was aware of the most current care plan for each
patient. Another prevalence study will be done to compare results from two years ago matched by the
APS's daily data collection. Above all, new and improved patient care practices are in place at our
institution.
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CELEBRATING 35 YEARS OF NURSING RESEARCH AT CNMC
J Pryor S Stanley S Feetham C May
Under the leadership of Lillian B. Williams, RN, MS, nurses at CNMC have participated in research since
1973. During the late 1960s and early 1970s, Lillian B. Williams, Director of Nursing, played a leading
role in the vision and design for the new Children’s Hospital to be built on Michigan Ave. Ms. Williams
had the vision to initiate steps to assure state of the art nursing practice at Children’s, and to become a
national leader in Pediatric Nursing. Ms. Williams implemented Primary Nursing as the care delivery
system in 1972 after months of research and planning. The new design was a pilot project on Main 3.
After 6 months, the members of the health care team endorsed Primary Care Nursing. In order to prepare
for a house wide initiation, Ms. Williams hired Geraldene Felton, RN, Ed.D, FAAN, to conduct an
experimental evaluation research study for Primary Nursing. She utilized 3 instruments to test the quality
of nursing care using the Primary Care model. • Quality patient Care Scale (QualPaCS) • Slater Nursing
Competencies Scale • Phaneuf Nursing Audit As primary nursing was implemented and evaluated,
recruitment increased, job satisfaction rose, and the turnover rate dropped. In the late 1970s, 75% of staff
were professional nurses with a baccalaureate degree and by the mid 1980s, 37 master prepared nurses
were functioning in management, education, and advanced practice roles. Today, CNMC nurses are
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actively involved in nursing research and evidence-based practice. A goal for the nursing research
program is to advance scientific knowledge and to improve the quality and safety of care for children and
families. Currently there are 12 active nursing research studies at CNMC.
PRE-OPERATIVE CARE CENTER TRANSFORMING CHILDREN'S
HEALTH THROUGH COLLABORATION WITH MAGNET FORCE
183
E Kassner M Coker K Thomson
Pre-Operative Care Center Surgical cases completed at Children’s National Medical Center are increasing
in complexity and case volume. Obtaining knowledge of the patient’s overall health is required to
successfully manage their perioperative care. As a joint venture between nursing and anesthesia, the PreOperative Care Center (POCC) has been developed to be a central point of coordinating multidisciplinary
services. The collected information enhances patient care, patient safety and surgical flow, increasing
satisfaction of families, patients and staff. The review process is primarily completed via phone by a
registered nurse or nurse practitioner under the supervision of an anesthesiologist. The screening is
divided into two categories- generally healthy patients and medically complex patients. The evaluation
includes a review of previous medical records and a comprehensive interview of the caregivers and/or
patient. The clinic coordinates with the child’s treating physicians, interdisciplinary specialists and assists
with coordination and review of additional testing (Echo’s, laboratory tests, x-rays, etc.), thus developing
a plan of care for the perioperative period. Optimizing the physical and emotional condition of medically
complex children prior to anesthesia and surgery can minimize health care cost and improve patient
outcomes. Review of preoperative information is completed within 24 hours of surgery by the chart
coordinator. Components include a surgical plan of care, consent, history, physical exam, consults as
needed and relevant laboratory data. Assembling this information prior to day of surgery, helps with
patient safety and JCAHO compliance. The nurse practitioners are available to assist with patient
throughput problems as they arise. Additionally they complete cardiovascular patient anesthesia
assessments and pre-operative teaching. Proposed additional services to be provided by the POCC include
pre-anesthesia visits for children requiring spinal fusion surgery, complex orthopedic procedures or any
medically complex/fragile child. Other areas of growth include evaluation of in-patients and patients at
CNMC ambulatory surgical centers.
IMPROVING ASTHMA TREATMENT BY EXPEDITING STEROID
ADMINISTRATION IN THE ED
184
MB Burridge JH Hinrichs SD Doyle CG Guzetta
Asthma is the most prevalent diagnosis in pediatric emergency departments. CNMC Emergency
Department (ED) saw 4208 pediatric asthma patients in 2006. The need to provide evidence-based (EB)
care in order to assure positive outcomes is essential to the success of all pediatric EDs. Asthma is a
disorder of bronchial edema, and increased mucus production leading to bronchospasm. Steroids act on
the bronchial tissue to decrease edema leading to increased gas exchange and increased comfort in
breathing. Steroid action takes 60 minutes after administration to affect the edematous bronchial tissue
taking prompt control of the disease process. The National Asthma Guidelines per the National Institute
of Health: National Heart, Lung, and Blood Institute (NHLBI) recommend steroid administration within
60 minutes of entry to ED care in order to promote positive patient outcomes. In 2006 door to steroid time
for moderate to severe asthma patients was an average of 2 hours. The ED culture was to prioritize
albuteral nebulizer treatments versus steroid and many times steroid were not given. This quality
improvement initiative aimed to assure all moderate to severe asthma patients received initial steroid
doses and to decrease ED arrival-to-steroid time to achieve the NHLBI recommendations. Asthma
patients were aggregated to a specific area of the ED, an EB pathway was developed and implemented to
decrease care variance, staff were educated via in-services, posters, e-mails and 1:1 discussion to the
importance of steroid initiation and monthly data was shared with staff. The less than 60 minute goal was
EDUC/TRAINING/PROG DEV
Staff
86
achieved by August 2007. Due to the intervention’s success, the ED decreased the arrival-to-steroid goal
again to 35 minutes in October 2007. The March 2008 time is 32.5 minutes. High volume patients
populations benefit in aggregation in a specific area, implementation of a pathway decreases medical
variance and allows nursing to anticipate care.
185
TAKING CHARGE: THE IMPLEMENTATION OF AN ED CHARGE
NURSE DEVELOPMENT PROGRAM
EM Murphy
How do we reward clinically excellent nurses who possess exemplary leadership skills? We put them in
charge! The role of the charge nurse in the emergency department poses unique challenges. The EMTC
charge nurse program is a 4 hour course designed to identify conceptions of the role and to address
staffing considerations and conflict management specifically in the ED.
186
PARENTS/GUARDIANS EXPERIENCE OF SAFE PATIENT CARE
ENVIRONMENT IN A PEDIATRIC HOSPITAL
SB Bostic
Patient safety is defined as freedom from accidental injury (To Err is Human: Building a Safer Health
System, 2000) . We know that the best assessment of how well healthcare organizations are performing is
as described by the patient and/or family’s experience, preferably in real time. Few organizations receive
patient and family feedback by means other than a survey. Organizations rarely capture a patient’s
experience in real time as a way of learning proactively how safe their system is performing. Creating a
dialogue with patients and families about safety can lead to a better understanding of how organizations
can improve the delivery of healthcare. The purpose of this phenomenological study is to understand how
parents/guardians experience a safe patient care environment in a pediatric hospital setting. Healthcare
organizations benefit from detailed input from patients and families to better understand what is important
to them, to identify system failures, and to improve the delivery system.
187
EVALUATION OF FAMILY PRESENCE IN THE EMERGENCY
DEPARTMENT DURING TRAUMA AND MEDICAL ALERTS
JL Hinrichs J Edens K O'Connell K Brown
Family presence at the patient’s bedside in the resuscitation room is the essence of family support,
allowing family members to benefit from being together during crisis. The family has the opportunity to
offer each other and the patient support, alleviate the sense of helplessness, work through the reality of a
situation, and finally, having the opportunity to share the final moments of a loved one’s life. Family
presence during resuscitation, although demonstrated to be a positive option for families and staff, is not
offered in all emergency departments, due to fear of litigation, and interruption in care. Recently our
pediatric emergency department approved a new policy and procedure for Family Presence during
Invasive Procedures and Resuscitation based on the Emergency Nurses Association’s national guidelines
that encompass family screening, family preparation, and use of a family presence facilitator to guide the
family through the experience. To ensure evidence-based practice, we implemented the new policy and
procedure and evaluated the first 100 family presence events to demonstrate the effects of this practice on
patient care outcomes. This study has been completed and data analysis is in progress.
Staff
EDUC/TRAINING/PROG DEV
87
IMPLEMENTING AN EVIDENCED-BASED COMPETENCY PROGRAM
FOR THE DELIVERY OF SEDATION FOR DIAGNOSTIC AND
THERAPEUTIC PROCEDURES
EP Engh RF Kaplan C Thrasher L de Nobel T Bailey
The pediatric population requires specialized sedation services for the provision of diagnostic and
therapeutic procedures. The increased demand for sedation services, the spectrum of available drugs, the
evolving clinical therapies and the need to provide for the safest delivery of sedation provided the
initiative to review existing practice patterns and requirements. This initiative involved forming a
multidisciplinary committee to revise the scope and practice of sedation for diagnostic and therapeutic
procedures for various settings across the CNMC system. The project was designed to establish and
promote evidence based requirements and effective care delivery processes for direct care providers of
this type of sedation. Once review of literature, professional standards, local and national regulations, best
practices and delivery of care at other pediatric hospitals was completed, the committee implemented, in
phases, the updated practice of sedation throughout CNMC. The implementation began with a revised
policy and procedure to include healthcare provider competency and credentialing requirements. Multiple
educational strategies were instituted to provide for the required changes in practice. The project
established clinical quality and performance improvement indicators with the requirements of ongoing
evaluation of outcomes. For sedation for diagnostic and therapeutic procedures, the program development
continues to evolve as companion processes such as Non-Operating Room Anesthesia emerge. Similarly,
the education and training of providers and the evaluation of outcomes is ongoing for this program.
EDUC/TRAINING/PROG DEV
Staff
188
88
Projects by Keyword
Abuse, 136
Cancer 38
Constraint induced therapy 173
Access Barriers 157
Cardiology 12, 92
Coping Skills 156
Access, 164
Cardiomyopathy 9, 92
Cortico-striatal border 41
Acute Pyelonephritis, 100
Cardiopulmonary arrests 118
Craniofacial Surgery 61
Administration 118
Cardiovascular Surgery 123
Curriculum Development 145
Adolescents 103
Case Study 53
dbx1 20
Adolescents 126
Cell lysate 21
Defects 27
adolescents 58
Cell proliferation 18
Development 19, 20, 27, 31
Advance care planning 58
Cervical spine 154
Developmental Biology 25
African American Women 136
Changing practice with evidence 146
Developmental Delays 158
Airway obstruction 149
Charge nurse 185
Developmental Disabiltieis 79, 128
AMP deaminase 17
Chest Pain 97
Developmental Testing 158
Amygdala 20
Chiari I 119
Diabetes 78, 103
Anatomy 31
Child self-report 90
Diagnosis 4
Anesthetics 172
Childhood 142
Diarrhea 109
Anger Management 156
Children 76, 79, 104
Dietary Intervention 127
Antimicrobial 50
Children and Adolescents 56
Disabilities 104
Apoptosis 11
Children with Disabilities 132
Disability 57, 165
Applied Behavior Analysis 160
Children w/ Special Health Care Needs 171
Down syndrome 14
Art Therapy 159
Children with Special Needs 135
Duchenne Muscular Dystrophy 122
Asthma 1, 16, 37, 48
Cholestasis 70
Dysphagia 116
Asthma Outcomes 184
Ciliary Dyskinesia 2
Early intervention 131, 157, 158
Asthma Treatment 184
Clinical and Translational
Echocardiography 12
Astrocytes 22
Research 167
ECMO 73, 93
Autism 112
Clinical Pathology 152
Educational Design 188
Autism 131
Clinical research 120
Educational Resource 159
Autism 134
Cystic Fibrosis 150
Electrophysiology 31
Autism Spectrum Disorders 42
Cystic Fibrosis related diabetes 150
Emergency Department
Autoimmune Disease 40
Clostridium difficile 109
Autophagy 24
Cochlear Implant 155, 162
168, 185
Emergency Medicine 74,
Behavior Modification 139
Cognition 54, 68, 87
Behavioral Sleep 81
Cognitive Development 82
89, 108
Endocrinology 92
Biochemistry 50, 140
Communication 137, 143
Epidemiology 95, 96, 101
Biomarkers 64
Communication Choices 155
Epilepsy 65, 105, 124
Blood Bank 125
Community Based Program 139
Equipment 89
Bone health 55
Community Health 138
Escalation 179
Bone quality 10
Community physicians 137
Ethnography 128
Bone volume 10
Comorbidity 112
Executive Function 112
Brain 19, 46
Compassionate Use 153
Exercise 34, 66
Brain damage 22
Complementary Therapy 166
Exercise Test 97, 170
Brain development 14
Complications 96
Experience 175
Brain Malformations 119
Concussion 85, 86, 87, 90
Expression profiling 13
Brain tumors 7
Congenital cystic pulmonary
Extra Hepatic Tissues 49
Breast cancer and African American
lesions 107
Ezetimibe 102
Women 129
Congenital Heart Defects 2
Family Presence 187
Breastfeeding 99
Congenital Heart Disease 61
Family Relationships 78
89
Family Presence during
Implementing Sedation Practice
CPR 169
Family-centered care 135, 148
Individuals with Disabilities
Fatigue 86
Changes 188
Education Act 157
Methods 68
MHC class 1 17, 43
Microarray Analysis 47
Microbiology 45
Fetal imaging 107
Infectious Disease 77, 123
Migration 15
Floortime 160
Infiltrate 115
Mild Traumatic Brain Injury 85
fMRI 68, 105, 124
Inflammation 13, 28
Minority recruitment 129
Fontan Complication 149
Information 162
Mitochondrial-Associated
FSHD 51
Injury Prevention 55
Functional Outcome 57
Insulin Resistance 126
Membranes 39
Mock Codes 178
Gene expression 19
Intellectual Disability 133
Molecular biology 140
Genetic Polymorphisms 37
Interdisciplinary Collaboration 135
Molecular Targets 32
Genetics 33, 34, 35, 46, 51,
Interdisciplinary Team Science 167
Molecular Testing 125
53, 54, 94
Glioma 38, 7
Global Health 145
Interleukin-15 29
Monitor Alarms 175
International 89
International research 45
Mood 85
Morphine 62
Glycosylation 4
Intervension 181
Motor Learning 173
Growth 117
Intraclass Correlation 113
Mouse Genetics 25, 27
HCMV 44
Intraoperative Monitoring 61
Mouse Model 51
Health and Wellness 165
Invasive Group A Streptococcus 96
MRI 26
Health Belief Model 141
Iron 64
MRSA 60
Health Professions Education
KSHV 23
mTBI 71
165
Hearing Loss 155
Helicobacter pylori 63
Language 105
Mucin Genes 37, 48
Latino 127
Left Without Treatment 168
Multidisciplinary 115
Muscle 94
Hemiparetic Cerebral
Limb Girdle Muscular Dystrophy
Muscle Damage 35
Type 2B, 29
Longitudinal 82
Muscle Function 52
Heterotaxy 2
HIV 58, 76
Lopinavir 76
Muscle strength 34
HIV Screening 141
Low birth weight 66
Muscular Dystrophy 4, 9,
HIV-1 infection 8
Lysosomal Storage Disorders 33
Holistic Nursing Practice 146
Macrophage function 21
13,28, 40, 512, 171
Myelin Gene Expression 5
Hospitalist 144
MAGNET 176
Myopathy 28
Human Cytomegalovirus 39
Magnetic Resonance
Myositis 17, 24, 43
Palsy 173
Human Herpesvirus 6 7
Spectroscopy 59
Muscle Pathology 29
NAGS 49
Hypercholesterolemia 102
Massage 66
NAGS deficiency 3
Hyperglycemia 83
MDX 26
Narrative Medicine 147
Hypertension 88
MDX Mice 52
Necortical Development 18
Hyperthyroidism 106
Medicaid 130
Negative Pressure Wound
Hypothermia 75
Medical Alert 187
Hypothyroidism 106
Medical Education 147, 161
Therapy 101
Neonatal 77
Hypoxia 30
Medical informatics 84
Neonatal encephalopathy 75
IFALD 153
Medically Complex Surgical Patients 183
Neonatal intensive care unit 83
Immunizations 130
Medication Delivery 177
Neonates 60, 73, 100
Immunohistochemical staining 63
Medication safety 84
Neonatology 93
Immunology 44
Medulloblastoma 15, 114
Nephrology 8
Infant care practices 99
Metabolism 69
Neural Development 36
Infants 109, 116
Metastasis 32
Neural Dtem Cell 18
90
Neurocognitive 53
Pediatric Emergency Medicine 67
Neurodevelopment 44
Pediatric Hospitalist 91
Neurodevelopmental Disorders 31
Pediatric Imaging 154
Renal glomerular filtration 8
Neurofibromatosis 119
Pediatric Intensive
Replication Program 23
Neurofibromatosis Type 1 82
Resident Education 145
Neuroimaging 110, 114
Neuro-Oncology 32, 114
Care Unit 57, 178
Pediatric Neurology 59
Pediatric setting 186
Neuroprotection 80
Pentoxifylline 122
Resources 134
Neuropsychological
Performance Improvement 174
Resuscitation 161
Asssessment 72
Neuropsychology 69, 71, 86,
Perinatal Asphyxia 80
Resuscitation 178
Perioperative Efficiency 183
Peripheral IV 115
Revised hyperglycemia
87, 90, 124
Neuroscience 25, 36
Neurosurgery 65
Relationship Development
Internvention 160
Resident Training 152, 161
Resource Guide 98
Pharmacokinetics 62
Pharmacy 177
protocol 83
Revised Sedation Practice 188
Rh genetics 125
NF-kB screening 40
Phenomenology 152
Rigid bronchoscopy 149
Normative 59
Phone-tree 164
Risk Assessment 141
Novice Registered Nurses 175
Phyiscal Activity 78
Scanning Success Rates 110
Nursing 174, 177, 179
Physical Medicine and
SCFE 95
Nursing Research 138, 167,
Rehabilitation 180
Physical Therapy 166, 170
Screening Tool 88
Plagiocephaly 180
Sedation Training 91
Nutrition 117, 142
Platelets 47
SensorMedics 113
Obesity 126, 127, 142, 163, 171
Pneumococcus 45
Sepsis 47, 77
OCCUPATIONAL HEALTH
Post-Concussive Symptoms 71
Serial Casting 121
Postpartum Depression 136
Severe gastritis 63
Oligodendrocytes 30
Practice Variation 74
Shared Nursing Leadership 176
Omega-3 Fatty Acids 153
Pre-clinical drug testing 9
Siblings 132
one lung ventilation 151
Prednisone 43
Sickle cell disease 62
Oral Glucose Tolerance Test 150
Prenatal Education 133
Signal Transduction 5, 16
Orientation 185
Pre-operative screening 183
Situs Inversus 12
Orthopedic 35, 94
Preterm Infant 70
Skeletel Muscle 26
Otitis Media 6
Preterm infants 22
Sleep Debt 111
Outcome 60, 75, 14
Prevention 108
Sleep Duration 111
Overweight 163
Primary Care 88, 163
Sleep Medicine 42
Pacifier use 99
Progenitor Cells 36
Sleep Problems 56, 111
Pain 172
Pro-Inflammatory Cytokines 6
Spatial Analysis 130
Palliative Care 98, 148
Protein Trafficking 39
Spealized Teams 143
Parenting 139
Proteomics 38, 46
Stat6 16
Parents/guardians 186
Provider Attitudes toward
Steroids 184
Pathogen 50
Family Presenc 169
Rad-path correlation 107
Stress Test 97
Patient /family outcomes 120
Patient Awareness 156
Randomized clinical trial 122
Patient Safety 67, 84, 186
Rapid Response Teams 118
of enzymes 3
Support Groups 132
Patient Satisfaction 164, 168
Reactive Oxygen Species 1
Support Staff 143
Patient-Family-Centered Care 146,
169
PECARN 67
Recognition 179
Surgery 137
Recovery curves 72
Surgical Site Infections 123
Pediatric 98, 102, 110
Renal disease 64
Teamwork 174
Pediatric Dialysis 117
Renal function 73, 93
Telencephalon 41
176, 182
Nursing Research History 182
AND WELLNESS 138
Sedation Practice 91
Structures and function
91
Television 79
Therapeutic Hypothermia 80
Therapeutics 11
Therapy 33
Tobacco Smoke 6
Tobacco Smoke Pollution 1
Trauma 154, 187
Traumatic Brain Injury 72,74
Treatment 121
Toe Walking 121
Torticollis 180
Total Parenteral Nutrition 70
TRAIL 24
Transcriptional regulation 48
Triangulation research
design 129
Ts65Dn mouse model 14
Tumor immunology 21
Type 1 Diabetes 81
Ultima 113
Unilateral Eisenmenger's
Syndrome 151
Unilateral pulmonary artery
stenosis 151
Urea Cycle Disorder 3, 49, 69
Ureagenesis 140
Urinary Tract Infection 100
Venipuncture 172
Viral Myocarditis 11
Virology 23
Viscocity 116
Vit.D deficiency 95
Washington, DC 134
Websites 162
Weight 106
Wellness 170
White Matter 30, 54
White matter Development 5
Whole-genome analysis 10
Wound Care 181
Wound management 101
Yoga 104, 166
Young children 131
Youth Violence 108
92
Projects by Program
AAAAAAAAAAAAAAAAAAAAA AAAAAAAAAAAAAAAAAAAAAA A
Acronym
Program
CFP
Cystic Fibrosis Program
150
CINRG
The Cooperative International Neuromuscular Group 122
COOLKids
Combating Obesity and Overweight in Latino Kids 127
CPCCRN
Collaborative Pediatric Critical Care Research Network 57
GCRC
General Clinical Research Center 172
IBS
Institutional for Biomedical Science 38
LEND
Leadership Education in Neurodevelopmental Disabilities
79, 98, 104, 116, 128, 132, 133, 134, 135, 154, 155, 157, 159, 160, 162, 164, 165
MTLDP
Master Teacher Leadership Development Program 147, 175, 186
PANDA
Pediatric Advanced NeeDs Assessment Team 148
Pediatric Clinical Research Scholar Program 62
PCRS
PECARN
Pediatric Emergency Care Applied Research Network 67, 74
REACH
Research, Education, Advocacy, and Child Healthcare
88, 92, 96, 99, 100, 103, 106, 108, 114, 130, 163
SCORE
Safe Concussion Outcome, Recovery & Eduction 86, 90
93
Projects by Department
AAAAAAAAAAAAAAAAAAAAA AAAAAAAAAAAAAAAAAAAAAA A
Academic Services Department 104, 132, 133, 134, 135, 153, 157, 159, 160, 164, 165
Adolescent and Young Adult Medicine 58
Allergy, Pulmonary, and Sleep Medicine 150
Anesthesiology and Pain Medicine 61, 143, 149, 151, 188
Cardiology Department 102
Center for Autism Spectrum Disorders
112
Center for Cancer and Blood Disorders 62, 148
Center for Cancer and Immunology Research 8, 11, 15, 21, 23, 32, 39, 44, 64
Center for Community and Clinical Research 55, 76, 136, 172
Center for Heart, Lung and Kidney Disease 12, 97
Center for Hospital Based Specialties 73, 74, 77, 96, 100, 168, 184, 187
Center for Neuroscience and Behavioral Medicine 5, 14, 18, 19, 20, 22, 25, 27, 30, 31, 36,
41, 65, 68, 92, 105, 110, 111, 119
Children's National Heart Institute 2
Critical Care Nursing 181
Critical Care Medicine 57, 178, 118
Emergency Medicine and Trauma Center 67, 89, 161, 185
Endocrinology and Diabetes 103
Epidemiology 123
General Pediatrics 127, 139, 142
General Pediatrics and Adolescent Medicine 113
General Surgery, Division of Trauma and Burn Services
Genetics and Metabolism 33
Hearing and Speech 116, 155, 162
Heart and Kidney Unit 174
Hospitalist Medicine 91, 144
Infectious Diseases 60, 109
Laboratory Medicine 125, 152
Medical Education 130, 147
Neonatology 66, 70, 75,80, 93
Nephrology 117
Neurology and Neuroscience 7, 54, 59, 114
Neuropsychology 53, 69, 71, 72, 85, 86, 87,90, 124
Nursing 115, 141, 146, 169, 175, 177, 179, 182
Occupational Health 138
Orthopaedic Surgery and Sports Medicine 10, 34, 95, 101
Patient Services 120, 129, 167, 186
Pediatric Rehabilitation 171
Pediatric Residency 45, 88, 92, 99, 106, 108, 128, 145, 163
Perioperative Services 183
Pharmacy Services 83, 84
Physical Medicine and Rehabilitation 121, 173, 180
Physical Therapy 166, 170
94
Psychiatry 56, 78, 156
Psychology 42, 79, 81, 98, 131, 158
Radiology 107, 154
Research Center for Genetic Medicine 1, 3, 4, 6, 9, 13, 16, 17, 24, 26, 28, 29, 35, 37, 38, 40,
43, 46, 47, 48, 49, 50, 51, 52, 94 122, 126, 140
The Joseph E. Robert Center for Surgical Care 63
95
Projects by First Author
AAAAAAAAAAAAAAAAAAAAA AAAAAAAAAAAAAAAAAAAAAA A
32
TJ
AbouAntoun
IMATINIB MESYLATE(GLEEVEC): ATTACKING THE
CULPRIT(PDGFR) AND THE ACCOMPLICE (EGFR) IN CHILDHOOD
MEDULLOBLASTOMAS
34
K
Adham
GENETIC VARIANTS ARE ASSOCIATED WITH BASELINE MUSCLE
STRENGTH AND SUBCUTANEOUS FAT VOLUME
56
CA
Alfano
SLEEP PROBLEMS AND THEIR RELATION TO COGNITIVE
FACTORS, ANXIETY, AND DEPRESSIVE SYMPTOMS IN CHILDREN
AND ADOLESCENTS
181
J.
Amling
NEONATAL AND PEDIATRIC WOUND CARE: FILLING THE HOLES
IN KNOWLEDGE AND PRACTICE
97
S
Anwar
EXERCISE TESTING IN CHILDREN WITH CHEST PAIN
74
SM
Atabaki
PRACTICE PATTERN VARIATION IN HEAD CT USE IN CHILDREN
WITH MINOR BLUNT HEAD TRAUMA IN THE ED: IS THERE AN
ASSOCIATION WITH PHYSICIAN TRAINING?
156
RE
Bannerman
COGNITIVE ADOLESCENT ANGER MANAGEMENT PROJECT
136
A
Barber
EFFECTS OF ABUSE ON MATERNAL MOOD DISORDERS AND
TREATMENT-SEEKING BEHAVIORS: PRELIMINARY ANALYSES OF
A COMMUNITY SAMPLE OF AFRICAN AMERICAN MOTHERS
40
AR
Baudy
AN IN VITRO SCREENING ASSAY TO IDENTIFY POTENTIAL NF-ОєB
INHIBITORS TO TREAT AUTOIMMUNE AND GENETIC MUSCLE
DISEASES
47
AS
Benton
A CROSS-SPECIES ANALYSIS OF PLATELET GENE EXPRESSION IN
SEPSIS SHOWS DYSREGULATION OF THE INNATE IMMUNE
CYTOKINES IL-1Гџ AND TNFa
57
JT
Berger
DEVELOPMENT OF A QUANTITATIVE FUNCTIONAL STATUS
SCALE
68
MM
Berl
THE ROLE OF MIDDLE FRONTAL GYRUS IN VERBAL WORKING
MEMORY
42
H
Bhatt
PARENT REPORTED SLEEP COMPLAINTS IN YOUTH DIAGNOSED
WITH AUTISM SPECTRUM DISORDERS
170
MC
Birkmeier
LET'S GET MOVING
96
93
LA
Bone
COMPARISON OF THE EFFECT OF VENOARTERIAL VERSUS
VENOVENOUS ECMO ON RENAL FUNCTION
132
BR
Bordonaro
DEVELOPING A RESOURCE GUIDE FOR SIBLINGS OF CHILDREN
WITH DISABILITIES
186
SB
Bostic
PARENTS/GUARDIANS EXPERIENCE OF SAFE PATIENT CARE
ENVIRONMENT IN A PEDIATRIC HOSPITAL
119
TC
Bouton
BRAIN MALFORMATIONS IN PATIENTS WITH
NEUROFIBROMATOSIS 1
101
C
Brandoli
NEGATIVE PRESSURE WOUND THERAPY IN CHILDREN:
EXPANDED INDICATIONS
129
PA
Broadnax
HEALTH LOCUS OF CONTROL AND FACTORS THAT INFLUENCE
AFRICAN AMERICAN WOMEN'S BREAST CANCER EXPERIENCE
92
JB
Brown
HYPOCALCEMIC RICKETS AND DILATED CARDIOMYOPATHY:
CASE REPORTS AND REVIEW OF LITERATURE
184
MB
Burridge
IMPROVING ASTHMA TREATMENT BY EXPEDITING STEROID
ADMINISTRATION IN THE ED
162
C
Buxton
A REVIEW OF COCHLEAR IMPLANT WEBSITES
51
JF
Cabotage
CHARACTERIZATION OF A TET-REPRESSIBLE MUSCLE-SPECIFIC
PITX1 TRANSGENIC MOUSE AS A MODEL OF FSHD
50
J
CabreraLuque
ANTIMICROBIALS THAT TARGET NOVEL MODES OF ARGININE
BIOSYNTHESIS
19
RS
Carney
DIFFERENTIAL REGULATION OF TELENCEPHALIC PALLIALSUBPALLIAL BOUNDARY PATTERNING BY PAX6 AND GSH2.
14
L
Chakrabarti
MECHANISMS OF ALTERED FOREBRAIN DEVELOPMENT IN THE
TS65DN MOUSE MODEL OF DOWN SYNDROME
67
JM
Chamberlain
INCIDENT REPORTS AND MEDICAL ERRORS IN PEDIATRIC
EMERGENCY DEPARTMENTS
75
T
Chang
OUTCOMES OF PATIENTS WITH NEONATAL ENCEPHALOPATHY
TREATED WITH WHOLE BODY HYPOTHERMIA
97
84
M
Chehab
UTILITY OF AUTOMATED TRIGGERS ON ADVERSE EVENT
DETECTION IN A PEDIATRIC HOSPITAL
48
YJ
Chen
CHROMATIN REMODELING OF THE MUC5AC PROMOTER DURING
DEXAMETHASONE-INDUCED GENE REPRESSION
5
LJ
Chew
P38 MAP KINASE REGULATES OLIGODENDROCYTE
DEVELOPMENT
41
LA
Cocas
DYNAMIC CELL MOVEMENTS AND THEIR RELATIONSHIP TO
FATE AT THE TELENCEPHALIC CORTICOSTRIATAL BORDER
180
AM
Conway
UPDATES IN TORTICOLLIS MANAGEMENT
7
J
Crawford
DETECTION OF HUMAN HERPESVIRUS 6 (HHV6) IN CNS TUMORS:
PREDOMINANCE OF ACTIVE VIRAL REPLICATION IN GLIAL
TUMORS.
106
MK
Crocker
THYROID STATUS HAS ONLY A MINOR EFFECT ON WEIGHT GAIN
OR LOSS IN CHILDREN
62
DS
Darbari
PHARMACOKINETIC-PHARMACODYNAMIC MODELING OF
MORPHINE IN PATIENTS WITH SICKLE CELL DISEASE:
POTENTIAL IMPLICATION FOR PAIN MANAGEMENT
49
H
Datta
Majumdar
LARGE VARIATIONS IN MRNA AND PROTEIN EXPRESSION OF
UREA CYCLE GENES IN THE LIVER AND EXTRAHEPATIC TISSUES
11
RL
DeBiasi
HEAT SHOCK PROTEINS AND G PROTEIN-COUPLED RECEPTOR
SIGNALING AS KEY DETERMINANTS OF VIRAL MYOCARDITIS
168
SD
Doyle
REDUCING EMERGENCY DEPARTMENT THROUGHPUT TIMES
159
LD
Dukes
CAPTIONS OF HOPE
82
PJ
Duquette
EXAMINING DEVELOPMENTAL NEUROPSYCHOLOGICAL
TRAJECTORIES IN CHILDREN WITH NEUROFIBROMATOSIS TYPE
1 (NF1)
154
AE
Egloff
EVALUATION OF THE PEDIATRIC CERVICAL SPINE IN TRAUMA
80
M
El-Dib
DEVELOPMENT OF SLEEP WAKE CYCLING AND PRESENCE OF
SEIZURES ON aEEG DURING WHOLE BODY HYPOTHERMIA
98
86
BM
Elliott
GENDER DIFFERENCES IN RECOVERY FROM POST-CONCUSSION
FATIGUE
135
LE
Emma
ON THE SAME PAGE: A WEB-BASED TOOL TO FACILITATE
INTERDISCIPLINARY COLLABORATION FOR A CHILD WITH
SIGNIFICANT NEURODEVELOPMENTAL DELAYS
188
EP
Engh
IMPLEMENTING AN EVIDENCED-BASED COMPETENCY
PROGRAM FOR THE DELIVERY OF SEDATION FOR DIAGNOSTIC
AND THERAPEUTIC PROCEDURES
43
V
Farajian
THE EFFECT OF PREDNISONE IN OVERALL PHENOTYPE IN CLASS
I MAJOR HISTOCOMPATIBILITY COMPLEX (MHC) – TRANSGENIC
MURINE MODEL OF MYOSITIS
120
S
Feetham
NURSING RESEARCH PROGRAM AT CNMC FROM CLINICAL
QUESTIONS TO RESEARCH TO IMPROVE CARE
144
BF
Fine
USING A PEDIATRIC HOSPITALIST ON A SUBSPECIALTY
SERVICE: UTILIZATION OUTCOMES
38
CA
Formolo
QUANTITATIVE ANALYSIS OF THE MALIGNANT GLIOMA
SECRETOME
12
LF
Frank
VENTRICULAR TWIST IN INFANT MICE WITH SITUS INVERSUS:
AN ANALYSIS BY HIGH FREQUENCY ULTRASOUND IMAGING
1
RJ
Freishtat
TIMP-1-NUCLEATES A CRITICAL BRONCHIAL EPITHELIAL
RESPONSE NETWORK TO TOBACCO SMOKE IN ASTHMA
161
D
Friedman
PEDIATRIC MOCK CODES: IMPROVING RESIDENT
RESUSCITATION
150
C
George
IMPROVED SCREENING FOR CYSTIC FIBROSIS RELATED
DIABETES IN THE PEDIATRIC AND ADULT CYSTIC FIBROSIS
PROGRAMS
29
R
Gernapudi
EFFECTS OF IL-15 ON MUSCLE PATHOLOGY AND FUNCTION IN A
MOUSE MODEL OF LIMB GIRDLE MUSCULAR DYSTROPHY TYPE
2B (LGMD2B)
71
GA
Gioia
PSYCHOMETRIC PROPERTIES OF THE PARENT AND TEACHER
POST-CONCUSSION SYMPTOMS INVENTORY (PCSI) FOR
CHILDREN AND ADOLESCENTS
54
AL
Gropman
DIFFUSION TENSOR IMAGING DETECTS AREAS OF ABNORMAL
WHITE MATTER MICROSTRUCTURE IN PATIENTS WITH PARTIAL
ORNITHINE TRANSCARBAMYLASE DEFICIENCY (OTCD)
26
D
Guerron
USE OF MAGNETIC RESONANCE IMAGING (MRI) TO DETECT
SKELETAL MUSCLE CHANGES IN MDX MOUSE MODELS OF
MUSCULAR DYSTROPHY.
99
146
CE
Guzzetta
EXPERT MENTORING TO CHANGE PRACTICE WITH EVIDENCE:
FAMILY PRESENCE DURING CARDIOPULMONARY
RESUSCITATION
44
JH
Haile
HUMAN NEURAL PRECURSOR CELL SUSCEPTIBILITY TO HUMAN
CYTOMEGALOVIRUS
98
SE
Hall
DC PEDIATRIC PALLIATIVE CARE COLLABORATION PARENT
AND CHILD RESOURCE MANUALS
179
LH
Hall
IMPROVING EARLY RECOGNITION USING THE PEDIATRIC EARLY
WARNING SCORE
140
N
Haskins
PROTEIN-PROTEIN INTERACTIONS BETWEEN NACETYLGLUTAMATE SYNTHASE (NAGS) AND
CARBAMYLPHOSPHATE SYNTHETASE I (CPSI) IN THE
REGULATION OF UREA CYCLE
187
JL
Hinrichs
EVALUATION OF FAMILY PRESENCE IN THE EMERGENCY
DEPARTMENT DURING TRAUMA AND MEDICAL ALERTS
20
T
Hirata
EMBRYONIC ORIGINS OF EXCITATORY AND INHIBITORY CELLS
IN THE AMYGDALA
137
KT
Hoff
QUALITY CARE: CREATING A COMMUNICATIVE ENVIRONMENT
FOR THE COMMUNITY PHYSICIANS WITH THE DIVISION OF
SURGERY
111
E
Huntley
SLEEP DURATION IN HEALTHY AFRICAN AMERICAN CHILDRENA
AND ADOLESCENTS
30
B
Jablonska
CELLULAR CHARACTERIZATION OF WHITE MATTER INJURY
AFTER PERINATAL HYPOXIA
110
KF
Jankowski
SUCCESS RATE OF FMRI SCANS IN CHILDREN WITH ASD,
EPILEPSY, ADHD, AND TYPICAL DEVELOPMENT
142
AM
Jaramillo
START EARLY, START RIGHT: CHANGING THE TRAJECTORY OF
CHILDHOOD OBESITY IN THE LATINO COMMUNITY
8
M
Jerebtsova
URINE SAMPLES FROM HIV- INFECTED CHILDRENS WITH RENAL
DISEASE INCREASE THE PERMEABILITY OF CULTURED HUMAN
RENAL GLOMERULAR ENDOTHELIAL CELLS
108
TJ
Johnson
THE ROLE OF THE EMERGENCY DEPARTMENT IN YOUTH
VIOLENCE PREVENTION: A SYSTEMATIC REVIEW
121
AJ
Joslyn
IDIOPATHIC TOE WALKING: CASE REPORT
100
183
E
Kassner
PRE-OPERATIVE CARE CENTER TRANSFORMING CHILDREN'S
HEALTH THROUGH COLLABORATION WITH MAGNET FORCE.
13
A
Kesari
DYSFERLIN-DEFICIENCY SHOWS COMPENSATORY INDUCTION
OF RAB27A/SLP2A THAT MAY CONTRIBUTE TO INFLAMMATORY
ONSET
147
T
Kind
LEARNING TO CONNECT: STUDENTS' REFLECTIONS ON DOCTORPATIENT INTERACTIONS
134
LC
King
RESOURCE GUIDE FOR FAMILIES OF CHILDREN WITH AUTISM IN
THE WASHINGTON, DC METROPOLITAN AREA
70
CJ
Klein
INCIDENCE OF PARENTERAL NUTRITION-ASSOCIATED
CHOLESTASIS IN THE NEONATAL INTENSIVE CARE UNIT AND
CHARACTERISTICS OF INFANTS
69
LS
Krivitzky
NEUROPSYCHOLOGICAL FUNCTIONING IN INDIVIDUALS WITH
UREA CYCLE DISORDERS
46
B
Kulkarni
2D GEL MAPPING OF HUMAN BRAIN SAMPLES FROM VANISHING
WHITE MATTER (VWM) PATIENTS AND CONTROL PEDIATRIC
SUBJECTS
35
JS
Larkin
GENETIC VARIANTS IN CCL2 AND CCR2 ARE ASSOCIATED WITH
MUSCLE DAMAGE
2
L
Leatherbury
A MOUSE MODEL OF PRIMARY CILIARY DYSKINESIA REVEALS
HIGH FREQUENCIES OF HETEROTAXY AND COMPLEX
CONGENITAL HEART DEFECTS
61
A
Lee
UNUSUAL MONITORING STRATEGIES DURING
CRANIOSYNOSTOSIS REPAIR IN A 15-MONTH OLD WITH
COMPLEX HEART DISEASE
77
RE
Levorson
HUMAN PARECHOVIRUS-3 INFECTIONl EMERGING PATHOGEN IN
NEONATAL SEPSIS
163
NS
Long
NUTRITION, PHYSICAL ACTIVITY AND MANAGING OVERWEIGHT
CHILDREN AND ADOLESCENTS
128
GR
Lotrecchiano
AN ETHNOGRAPHIC STUDY OF PROFESSIONAL CARE
APPROACHES AND ROLES WHEN DEALING WITH SEVERE AND
PROFOUND ILLNESSES
18
K
Loulier
BETA 1 INTEGRIN SIGNALING IS NECESSARY FOR ANCHORAGE
OF NEURAL PROGENITORS AT THE MURINE VENTRICULAR
SURFACE
58
ME
Lyon
COURAGEOUS CONVERSATIONS: FEASIBILITY &
ACCEPTABILITY OF FAMILY CENTERED ADVANCE CARE
PLANNING
101
Mackey
PARENTAL SUPPORT AND EXERCISE-RELATED CONFLICT AND
REPORTED EXERCISE IN PREADOLESCENTS WITH TYPE 1
DIABETES
GM
Many
THE PREVALENCE OF INSULIN RESISTANCE IN SEDENTARY,
OBESE AND OTHERWISE HEALTHY MINORITY ADOLESCENTS IN
THE DC METROPOLITAN AREA.
27
J
Mao
CELLULAR MECHANISM UNDERLYING THE LEFT-RIGHT
ASYMMETRY DEFECTS OF CCDC40 MUTANT MICE
133
R
Margolis
CAN PRENATAL EDUCATION PROGRAMS BE ADAPTED FOR
WOMEN WITH MILD INTELLECTUAL DISABILITY?
91
DW
Marseille
CHARACTERISTICS OF PEDIATRIC HOSPITALISTS PROVIDING
MODERATE TO DEEP SEDATION: A STUDY FROM THE PRIS
NETWORK
79
G
Martin
TELEVISION AND VIDEO EXPOSURE IN YOUNG CHILDREN AT
RISK FOR DEVELOPMENTAL DELAY
172
B
Martin
THE SELF REPORTED USAGE OF TOPICAL ANESTHETICS BY
REGISTERED NURSES DURING VENIPUNCTURE AT CHILDREN'S
NATIONAL MEDICAL CENTER
66
AN
Massaro
MASSAGE AND KINESTHETIC STIMULATION (EXERCISE)
IMPROVES WEIGHT GAIN IN VERY LOW BIRTH WEIGHT (VLBW)
PRETERM INFANTS
105
JD
Mayo
LANGUAGE LATERALIZATION IN FOCAL EPILEPSY AS
DETERMINED BY FOUR METHODS
141
DS
McCoy
THE RELATIONSHIP BETWEEN PERCEIVED SUSCEPTIBILITY AND
HIV SCREENING
160
JS
Metz
NEXT STEPS: A RESOURCE GUIDE FOR PARENTS OF CHILDREN
WITH AUTISTIC SPECTRUM DISORDER
21
S
Miles
TUMOR CELL IYSATE SUPPRESSION OF MACROPHAGE
FUNCTION
176
EO
Miller
SHARED NURSING LEADERSHIP AS A FORUM FOR NURSING
RESEARCH
127
N
Mirza
DIETARY BEHAVIOR CHANGES IN A FAMILY-CENTERED
INTERVENTION PROGRAM FOR OVERWEIGHT LATINO YOUTH
81
MC
Monaghan
SLEEP DISRUPTION IN PARENTS OF YOUNG CHILDREN WITH
TYPE 1 DIABETES
78
E
126
102
33
CP
Morgan
MIGLUSTAT IMPROVES FUNCTION IN JUVENILE GM1
GANGLIOSIDOSIS: IS THERE A PHRAMACOLOGICAL CHAPERONE
EFFECT?
185
EM
Murphy
TAKING CHARGE: THE IMPLEMENTATION OF AN ED CHARGE
NURSE DEVELOPMENT PROGRAM
130
M
Nguyen
MAPPING IMMUNIZATION RATES IN WASHINGTON, DC
96
N
Niforatos
EPIDEMIOLOGY OF INVASIVE GROUP A STREPTOCOCCUS IN
CHILDREN
171
PM
O'Berry
DEVELOPING STANDARDS OF CARE FOR OBESITY PREVENTION,
SCREENING AND TREATMENT FOR CHILDREN WITH SPECIAL
HEALTH CARE NEEDS
158
MA
O'Connell
COMPARING THE BAYLEY-II AND BAYLEY-III: ARE WE LOSING
CHILDREN IN NEED OF EARLY INTERVENTION?
31
L
Olmos
ALTERATIONS IN THE BASOLATERAL COMPLEX OF THE
AMYGDALA IN THE FMR1 MOUSE MODEL OF FRAGILE X
99
OO
Omojokun
BREASTFEEDING INTENT, PACIFIER USE, AND THE DURATION OF
BREASTFEEDING IN AN URBAN POPULATION: A PILOT STUDY
139
M
Palmer
EXAMINING KNOWLEDGE GAINED IN A LATINO PARENTING
PROGRAM
17
GS
Pandey
TYPE I AND TYPE II MUSCLE FIBERS ARE DIFFERENTIALLY
AFFECTED IN THE MAJOR HISTOCAMPATIBILTY CLASS I (MHC
CLASS I) MOUSE MODEL OF MYOSITIS
125
PP
Pary
MOLECULAR TESTING EXPLAINS THE PRODUCTION OF ANTI-D
IN AN RH POSITIVE TODDLER
94
RR
Patel
SLC30A8 VARIANT IS ASSOCIATED WITH MUSCLE SIZE AND
RESPONSE TO RESISTANCE TRAINING
59
PB
Peloquin
PEDIATRIC NORMATIVE DATABASE IN BASAL GANGLIA
MAGNETIC RESONANCE SPECTROSCOPY
83
L
Pinchevsky
IMPLEMENTATION OF A REVISED HYPERGLYCEMIA
MANAGEMENT PROTOCOL FOR BETTER CONTROL OF BLOOD
GLUCOSE IN NON-DIABETIC, CRITICALLY ILL NEONATAL
PATIENTS
88
SP
Potiny
IMPROVING THE DIAGNOSIS OF PEDIATRIC HYPERTENSION IN
THE PRIMARY CARE SETTING
103
Prasad
ALTERNATIVE RESCUE REPLICATION PROGRAM IN KAPOSI’S
SARCOMA ASSOCIATED HERPES VIRUS IN RELATION TO HOST
CELL DEATH
Preciado
CIGARETTE SMOKE ACTIVATES NF?B- MEDIATED TNF A
RELEASE FROM MOUSE MIDDLE EAR CELLS
Pryor
CELEBRATING 35 YEARS OF NURSING RESEARCH AT CNMC
Rabinowitz
SIMPLIFYING CONGENITAL CYSTIC PULMONARY LESIONS: A
SPECTRUM OF RAD-PATH CORRELATION
K
Rais-Bahrami
A TALE OF TWO BRIDGES: EFFECT OF THE BLOODLESS BRIDGE
ON BLOOD PRESSURE IN NEONATES MANAGED WITH VENOARTERIAL EXTRACORPOREAL MEMBRANE OXYGENATION
76
N
Rakhmanina
RECOMMENDED DOSE OF LOPINAVIR / RITONAVIR IS SUBOPTIMAL IN PROTEASE INHIBITOR-EXPERIENCED CHILDREN
24
R
Rawat
ROLE OF TUMOR NECROSIS FACTOR-ALPHA-RELATED
APOPTOSIS-INDUCING LIGAND (TRAIL) IN MEDIATING MUSCLE
FIBER DAMAGE IN MYOSITIS
143
RJ
Roberts
SUPPORT STAFF EXPERIENCES ON SPECIALIZED HOSPITAL
TEAMS
167
NC
Robinson
RE-ENGINEERING THE CLINICAL RESEARCH ENTERPRISE: A
PROTOTYPE OF INTERDISCIPLINARY RESEARCH
124
LR
Rosenberger
RIGHT HEMISPHERE CONTRIBUTION TO LANGUAGE AT THE
COST OF VISUALLY BASED SKILLS IN LEFT HEMISPHERE
103
M
Russell
PSYCHOLOGICAL COUNSELING IN THE ADOLESCENT DIABETIC
POPULATION IS AN IMPORTANT COMPONENT IN MAINTENANCE
OF ADEQUATE GLYCEMIC CONTROL
55
LM
Ryan
VITAMIN D INSUFFICIENCY IN AFRICAN AMERICAN CHILDREN
WITH FOREARM FRACTURES
52
AA
Sali
EFFECT OF EPIGALLOCATECHIN-3 GALLATE(EGCG) AND
PIRFENIDONE ON MDX MOUSE PHENOTYPE
65
JA
Salpekar
PSYCHIATRIC PROFILES OF PRESURGICAL PEDIATRIC EPILEPSY
PATIENTS
45
AS
Sampath
S. PNEUMONIAE DETECTION: EFFECTS OF LOW DENSITY AND
DELAY TO PROCESSING.
23
A
6
DA
182
J
107
DA
73
104
131
L
Sanchez
AUTISM RESOURCES FOR CHILDREN UNDER 3 YEARS OF AGE IN
THE DC/METRO AREA
169
L
Sanders
PROVIDER ATTITUDES TOWARD FAMILY PRESENCE DURING
INVASIVE PROCEDURES AND RESUSCITATION EVENTS IN THE
PICU/CICU
25
AA
Sarkar
CHARACTERIZATION OF THE MOLECULAR BASIS OF NEURAL
TUBE CLOSURE AND PLACENTAL DEFECTS IN THE OPENMIND
MUTANT MOUSE MODEL
123
J
Schexnayder
APPROPRIATE PROPHYLACTIC ANTIBIOTICS IN REDUCING
SURGICAL SITE INFECTION IN PEDIATRIC PATIENTS
UNDERGOING CARDIOVASCULAR SURGERY
22
TS
Schmitz
HYPEROXIA CAUSES ASTROGLIOSIS IN THE DEVELOPING BRAIN
85
JC
Schneider
PARENT AND SELF-REPORTED CHANGES OF EMOTIONAL
FUNCTIONING POST CONCUSSION
148
R
Selove
RECOMMENDATIONS FROM BEREAVED PARENTS FOR FAMILYCENTERED PEDIATRIC PALLIATIVE CARE
117
KL
Sgambat
COMPREHENSIVE ASSESSMENT AND RELATIONSHIP BETWEEN
NUTRITION AND GROWTH IN PEDIATRIC HEMODIALYSIS
PATIENTS
100
NS
Shah
NEONATAL URINARY TRACT INFECTION (UTI): VARIABILITY IN
LENGTH OF INTRAVENOUS ANTIBIOTIC THERAPY (IVAT)
145
NS
Shah
IMPLEMENTATION OF A GLOBAL CHILD HEALTH CURRICULUM
FOR PEDIATRIC TRAINEES
72
MS
Shapiro
DOCUMENTING RECOVERY TRAJECTORIES IN
CHILD/ADOLESCENT CONCUSSION
3
D
Shi
CRYSTAL STRUCTURES OF N-ACETYLGLUTAMATE SYNTHASE
PROVIDE INSIGHTS INTO CATALYTIC AND REGULATORY
MECHANISMS.
95
EJ
Silverman
SLIPPED CAPITAL FEMORAL EPIPHYSIS AT CHILDREN’S
NATIONAL MEDICAL CENTER
63
JD
Snyder
IMMUNOHISTOCHEMICAL CONFIRMATION OF THE CUASITIVE
ROLE OF H. PYLORI IN SEVERE GASTRITIS IN CHILDREN
112
JL
Sokoloff
WORKING MEMORY PROFILES IN CHILDREN WITH AUTISM
SPECTRUM DISORDERS AND ELEVATED ADHD SYMPTOMS
105
Soler-Garcia
NOVEL ROLE OF IRON AND IRON RELATED PROTEINS AS URINE
BIOMARKERS IN CHILDREN WITH HIV-ASSOCIATED RENAL
DISEASES
X
Song
CLINICAL AND ECONOMIC IMPACT OF METHICILLIN-RESISTANT
STAPHYLOCOCCUS AUREUS ON NEONATES IN INTENSIVE CARE
UNITS
4
SE
Sparks
GLYCOSYLATION DEFECTS IN MUSCULAR DYSTROPHY
155
LM
Spies
WHAT NEXT? A GUIDE FOR FAMILIES WITH CHILDREN
RECENTLY DIAGNOSED WITH HEARING LOSS.
9
CF
Spurney
P188 IMPROVES SKELETAL MUSCLE STRENGTH AND PREVENTS
TACHYCARDIA INDUCED CARDIOMYOPATHY IN DYSTROPHIN
DEFICIENT MICE
36
EK
Stancik
PROGENITOR POPULATIONS IN THE DEVELOPING VENTRICULAR
ZONE DISPLAY DISTINCT CELL CYCLE KINETICS
177
SG
Stanley
A COLLABORATIVE NURSING AND PHARMACY INITIATIVE TO
IMPROVE PATIENT MEDICATION DELIVERY AT CNMC
116
SL
Stuart
VISCOSITY IN INFANT DYSPHAGIA MANAGEMENT:
STANDARDIZING PRACTICE
104
CH
Sullivan
YOGA TO IMPROVE FITNESS IN CHILDREN WITH DISABILITIES
157
E.
Thursby
EXPLORING ACCESS BARRIERS TO EARLY INTERVENTION
SERVICES IN WASHINGTON, DC
10
LL
Tosi
DETERMINANTS OF HUMERAL BONE VOLUME IN MALES: A
GENOME-WIDE SCAN
109
TM
Tran
EPIDEMIOLOGY OF CLOSTRIDIUM DIFFICILE ASSOCIATED
DISEASE IN INFANTS YOUNGER THAN 2 YEARS OF AGE
153
AL
Trautman
COMPASSIONATE USE OF INTRAVENOUS OMEGA-3 FATTY ACID
AS AN ALTERNATIVE TREATMENT FOR CHILDREN WITH
INTESTINAL FAILURE-ASSOCIATED LIVER DISEASE
173
AT
Turkus
CONSTRAINT INDUCED THERAPY: A PILOT STUDY
151
SV
Valairucha
UNILUNG VENTILATION / LEFT THORACOTOMY / LEFT
MODIFIED BT SHUNT IN AN ADULT WITH CRITICAL LPA
STENOSIS & RIGHT EISENMENGER’S SYNDROME
64
AA
60
106
VanderMeulen
ROLE OF INFLAMMATION AND REGENERATION IN MUSCLE
WEAKNESS IN DIFFERENT MOUSE MODELS OF MYOPATHY
CV
Vaughan
INITIAL EXAMINATION OF SELF-REPORTED POST-CONCUSSION
SYMPTOMS IN NORMAL AND MTBI CHILDREN AGES 5 TO 12
149
SV
Verghese
URGENT RIGID BRONCHOSCOPY FOR REMOVAL OF PLASTIC
BRONCHIAL CASTS IN A TODDLER WITH FONTAN PHYSIOLOGY
113
S
Villavicencio
VALIDATION STUDY FOR TWO TYPES OF RESTING ENERGY
EXPENDITURE EQUIPMENT
138
L
Wagner
WALK AND TALK YOUR WAY TO HEALTH: A PILOT PROGRAM TO
INCREASE ACTIVITY AND NUTRITIONAL KNOWLEDGE
102
K
Wai
EZETIMIBE IN THE TREATMENT OF PEDIATRIC PATIENTS WITH
HYPERCHOLESTEROLEMIA
89
DM
Walker
AN INTERNATIONAL APPLICATION OF EQUIPMENT GUIDELINES
FOR PEDIATRIC EMERGENCIES
178
JL
Walker
EDUCATING AND ESTABLISHING GOOD TECHNIQUE AMONG ALL
RESUSCIATION PARTICIPANTS DURING CODES IN THE
PEDIATRIC INTENSIVE CARE UNIT
53
KS
Walsh
NEUROCOGNITIVE PROFILE IN A CASE OF MAPLE SYRUP URINE
DISEASE
175
HA
Walsh
THE EXPERIENCE OF NOVICE REGISTERED NURSES IN
RESPONDING TO MONITOR ALARMS
37
AM
Watson
A GENETIC POLYMORPHISM OF THE MUC7 MUCIN GENE IN
ASSOCIATION WITH ASTHMA AND ETHNICITY
174
SW
Weeks
TEAMWORK: IMPROVING CUSTOMER SERVICE AND NURSING
RETENTION
87
C
Wells
EXAMINATION OF THE EVERYDAY SITUATIONS SURVEY (ESS) IN
CHILDREN AND ADOLESCENTS
114
EM
Wells
NEURORADIOGRAPHIC FEATURES OF PATIENTS WITH THE
CEREBELLAR MUTISM SYNDROME: A LONGITUDINAL MRI
STUDY
164
B
Wiley
IMPROVING PATIENT SATISFACTION & ACCESS: NEUROLOGY
CLINIC
28
J
90
107
115
LW
Williams
ALL FOR ONE: REDUCING PIV INFILTRATES THROUGH A
MULTIDISCIPLINARY APPROACH
39
CD
Williamson
SORTING OF HUMAN CYTOMEGALOVIRUS UL37 PROTEINS
THROUGH THE MAM TO MITOCHONDRIA OR TO GOLGI.
152
EC
Wong
EXPERIENCES THAT CONTRIBUTE TO CLINICAL PATHOLOGY
LEARNING BY PATHOLOGY RESIDENTS
166
CH
Wong
INTEGRATING YOGA INTO A PEDIATRIC PHYSICAL THERAPY
SESSION: OBSERVATIONS FROM THE FIELD
118
KW
Woronick
PEDIATRIC RAPID RESPONSE TEAMS DECREASE
CARDIOPULMONARY ARRESTS ON ACUTE CARE UNITS
16
X
Wu
IDENTIFYING GENES AND PATHWAYS THAT LEAD TO GOBLET
CELL METAPLASIA IN AN IL13 INDUCED MURINE ALLERGIC
ASTHMA
15
LP
Yuan
THE ROLE OF RAC SIGNALING IN PDGF-MEDIATED CELL
MOTILITY IN MEDULLOBLASTOMA
165
O
Zavadska
INTEGRATION OF WELLNESS AND DISABILITY EDUCATION IN
UNDERGRADUATE HEALTH PROFESSIONS CURRICULA
122
A
Zimmerman
PENTOXIFYLLINE FAILS TO RESCUE MUSCLE STRENGTH AND
FUNCTION DETERIORATION IN PREDNISONE TREATED
DUCHENNE MUSCULAR DYSTROPHY

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