Glomerulonephritis
in children
Pavlyshyn H.A.
Definition
 Acute glomerulonephritis is the
inflammation of the glomeruli which
causes the kidneys to malfunction
• It is also called Acute Nephritis,
Glomerulonephritis and PostStreptococcal Glomerulonephritis
• Predominantly affects children from ages 2
to 12
• Incubation period is 2 to 3 weeks
Autoimmune Reactions
Some progress as either focal segmental glomerulosclerosis or
tubulointerstitial nephritis
Possible Clinical Manifestations
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Proteinuria – asymptomatic
Haematuria – asymptomatic
Hypertension
Nephrotic syndrome
Nephritic syndrome
Acute renal failure
Rapidly progressive renal failure
End stage renal failure
Presentation
• Hematuria
with Proteinuria
with Dysmorphic rbcs
with Rbc casts
• Oliguria
• Volume overload
• Hypertension
Liquid Renal Biopsy
Urine Sediment Analysis
G4 cell
Other H&P findings
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Neurological changes
Pharyngitis
URI / sinusitis
Hemoptysis
Rash
Murmur
Arthritis
Edema
Complement Abnormalities
Ab-Ag complexes
C3 convertase
Classical pathway
(C4 + C2)
C3
(C4bC2a)
Membrane
attack complex
C3b
Recruitment of
PMNs
C3a
Alternative pathway
Microbial surfaces
(polysaccharides)
C3 convertase
Opsonization,
phagocytosis
Anaphylaxis,
Chemotaxis
Differential Diagnosis
Hypocomplementemia
Normal complement
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PIGN
MPGN
SLE
Cryoglobulinemia
Bacterial Endocarditis
Shunt nephritis
HUS
IgAN
HSP
Alport’s / TBMD
b-hemolytic Streptococci
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Most common organism in PIGN
20% children are asymptomatic carriers
Nephritic factor
Host susceptibility factors (HLA-DR)
Treatment of prodromal illness doesn’t
prevent nephritis
• ASO titers are NOT helpful
Post Infectious GN
Pathogenesis
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Strep antigens trigger antibodies that cross-react to glomeruli
Circulating immune complexes get filtered by glomerulus & get stuck
Immune complexes activate complement
Diffuse & generalized damage to glomeruli
↓ GFR due to inflammation, damage to BM
↓ RBF in proportion to GFR, so filtration fraction normal
Tubular function is preserved
Plasma renin and aldosterone are normal
Presentation
• 7-14 days after pharyngitis
• 14-21 days after impetigo (upto 6 wks)
• Abrupt onset
Manifestations of PIGN
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Edema
HTN
Gross hematuria
CNS (i.e. Sz)
Nephrotic syndrome
ARF
C3
C4
85%
60-80%
25-33%
10%
rare
not uncommon
decreased
typically normal
Management of PIGN
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Antibiotics do NOT prevent GN
Sodium & Fluid restriction
Antihypertensives, diuretics for HTN
Dialysis if necessary
Prognosis usually excellent
 0.5% mortality due to pulmonary edema or pneumonia
 <1% progress to CKD stage 5
• Follow-up
 Gross hematuria resolves within 2 weeks
 Complement low for 6-8 weeks
 Proteinuria remains upto 6 months
 Hematuria remains upto 2 years
Renal Biopsy
Histopathology
Diffuse = all glomeruli
Generalized = all segments of glomeruli
IgG Immunofluorescence
Starry Sky Pattern
Electron microscopy - Normal
Basement
membrane
Foot
processes
Electron microscopy of PIGN
• Subepithelial immune deposits (humps)
 Mesangial, subendothelial, intramembranous deposits less common
• Effacement of foot processes
Hemolytic Uremic Syndrome
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2 cases/100,000 annually
Peak incidence <5yo (6/100,000)
More common June-September
Classification
D+
Strep pneumo
Atypical HUS
diarrhea associated
ADAM-TS13, C1q def
Presentation of D+ HUS
• Prodromal acute gastroenteritis
 Shiga toxin producing E.coli O157:H7
 Transmission from beef, veggies, direct person-to-person, and
contaminated water all reported
 Incubation period 3-4 days
 Bloody diarrhea 2-3 days after cramping begins
 50% with emesis, afebrile or low grade fever only
• Hemolytic anemia
• Thrombocytopenia
• ARF
 Begins 2-14 days after diarrhea
• CNS disease
 Overlap with ITP in 33% HUS cases
 Somnolence, confusion, seizures, coma
Microangiopathic Hemolytic Anemia
Henoch Schönlein Purupura
Henoch Schönlein Purupura
• GI tract
Cramping, vomiting, diarrhea
• Skin rash
Lower extremities, buttocks
• Joint involvement
• HSP nephritis
Incidence 20-50%
In 80%, occurs within 4 weeks of rash & GI upset
In 15%, occurs upto 1-3 months after rash & GI
upset
Pathogenesis of Alport’s
• Abnormality of type
IV collagen
• Disordered
basement
membrane
• Splitting of lamina
densa of GBM
Crescentic GN
Type
Serology
Primary
Secondary
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Anti-GBM+ ANCA-
Anti-GBM disease
Goodpasture’s
II
Anti-GBM- ANCA-
idiopathic
SLE, IgAN, MPGN
III
Anti-GBM- ANCA+
Microscopic polyangiitis, Drug-induced
Wegener’s
IV
Anti-GBM+ ANCA+
Anti-GBM disease
Goodpasture’s
Vasculitides
C-ANCA
P-ANCA
Anti-proteinase 3 antibodies
Anti-myeloperoxidase antibodies
75% sensitive for Wegener’s
66% sensitive for
Microscopic polyangiitis
Anti-GBM Disease
Silver stain
IgG immunofluorescence