P253 De novo Inflammatory Bowel Disease following paediatric liver

Poster presentations
S202
Conclusions: IFX has been reported to induce acute liver failure
by at least three mechanisms: induction of autoimmune hepatitis,
cholestatic liver injury and direct toxicity. Considering the exclusion
of main liver diseases, the temporal correlation between infliximab
exposure and laboratorial changes, an IFX-induced hepatitis diagnosis was admitted. Current consensus guidelines recommend baseline
liver function tests with a hepatic risk factor screen (hepatitis serology, auto-antibodies) prior to onset of therapy. It is suggested screening for liver dysfunction at 4-month intervals and discontinuation
of IFX therapy if transaminase levels reach three times the upper
limit of normal. This study highlights an important and potentially
lethal complication of IFX therapy and reinforces need for caution
and increased vigilance in prescreening and ongoing surveillance for
patients on IFX
P253
De novo Inflammatory Bowel Disease following
paediatric liver transplantation: A case series of
three patients and world literature review
K. Nikaki*1, D.C. Wilson2, P. McKiernan3, C.H. Spray4
1
Birmingham Children's Hospital NHS Foundation Trust, Paediatric
Liver Unit, Birmingham, United Kingdom, 2Royal Hospital for Sick
Children, Paediatric Gastroenterology and Nutrition Department
, Edinburgh, United Kingdom, 3Birmingham Children's Hospital
“Hepatic necrosis. H&E staining, original magnification 20x”
NHS Foundation Trust, Paediatric Liver Unit , Birmingham,
United Kingdom, 4Bristol Royal Hospital for Children, Paediatric
Gastroenterology Department, Bristol, United Kingdom
Background: Inflammatory Bowel Disease (IBD) is a T-cell driven
inflammatory process due to inappropriate and enduring activation
of the enteric immune system, generally treated with immunosuppressant therapy. Following solid organ transplantation (SOT),
recurrent and de novo IBD have been described despite immunosuppressive therapy. The majority of cases in adult patients occurred
post liver transplantation (LT) (136/175) with 96/136 cases reported
having been originally transplanted for sclerosing cholangitis (SC)
or autoimmune hepatitis (AIH). In paediatrics, 14 cases have been
described post liver, heart and renal transplant. 9 cases have been
described post LT (3/9 cases transplanted for SC/AIH). Various risk
factors have been implicated in the development of post-transplant
IBD. Herein, we describe 3 cases of de novo IBD post LT for causes
other than SC/AIH.
Methods: Case 1 was the index case and the other 2 patients
were identified through an electronic search of the Birmingham
Liver Unit Paediatric Transplant database that holds the data
of 782 patients who have undergone a liver transplant between
1983 and 2014. Medline and Embase were searched for "de
novo inflammatory bowel disease" and "transplantation". The
search was extending by scanning reference lists of related
articles and free text web search. A total of 46 articles were
included in the systematic review.
Results: 3 patients (2 females) were identified with de novo IBD following LT. 2 patients were originally transplanted for a1 antitrypsin
deficiency and 1 for extra-hepatic biliary atresia. Risk factors for the
development of post LT IBD are shown in table 1.
Mean age at the time of de novo IBD diagnosis was 7.3 years (range
3-11 years) with a mean of 4.6 years post LT. Diarrhoea was the
presenting symptom in 2 patients and intermittent rectal bleeding in
1. All patients were investigated for possible IBD according to Porto
criteria. The patients underwent on average 2.6 upper and/or lower
GI endoscopies prior to diagnosis, while the time of presentation
to diagnosis varied from 3 months to 1 year. Crohn’s Disease was
diagnosed in 2 patients and Indeterminate Colitis in 1. Infliximab
was used in 1 patient while the other 2 were treated with 5-aminosalicylic acids. All patients are in clinical remission.
Conclusions: De novo IBD does occur following liver transplantation in children but is rare. De novo IBD should be
considered in the differential diagnosis of chronic diarrhoea
post-transplant.
Risk Factors for De Novo IBD and Immunosuppressive Rx before and after De Novo IBD Diagnosis
CMV mismatch (donor / recipient)
CMV infection post LTx
Acute / Chronic Rejection
Biliary stasis
PMHx of Autoimmunity
FHx of Autoimmunity
Immunosuppression at presentation with de novo IBD
Immunosuppression after diagnosis with de novo IBD
Other IBD Tx following diagnosis
Case 1: Crohn’s disease
Case 2: Indeterminate
Colitis
Case 3: Crohn’s
disease
-ve/-ve
No
No / Yes
No
No
No
Tacrolimus, Prednisolone, MMF
Tacrolimus, Prednisolone, AZA
Infliximab
+ve / -ve
No
No / No
No
No
No
Cyclosporine
MMF, Prednisolone
Mesalazine
-ve/-ve
No
No / No
No
No
No
Cyclosporine
MMF, Prednisolone
Mesalazine