1. Glomerulonephritis in children
Pavlyshyn H.A.

3. Definition
Acute glomerulonephritis is the inflammation of the glomeruli which causes the kidneys to malfunction
It is also called Acute Nephritis, Glomerulonephritis and Post-Streptococcal Glomerulonephritis
Predominantly affects children from ages 2 to 12
Incubation period is 2 to 3 weeks

7. Autoimmune Reactions
Some progress as either focal segmental glomerulosclerosis or
tubulointerstitial nephritis 7

8. Possible Clinical Manifestations
Proteinuria – asymptomatic
Haematuria – asymptomatic
Hypertension
Nephrotic syndrome
Nephritic syndrome
Acute renal failure
Rapidly progressive renal failure
End stage renal failure 8

9. Presentation Hematuria Oliguria Volume overload Hypertension
with Proteinuria
with Dysmorphic rbcs
with Rbc casts
Oliguria
Volume overload
Hypertension

10. Liquid Renal Biopsy

11. Urine Sediment Analysis
G4 cell

12. Other H&P findings Neurological changes Pharyngitis URI / sinusitis
Hemoptysis
Rash
Murmur
Arthritis
Edema

13. Complement Abnormalities
Ab-Ag complexes
Classical pathway
C3 convertase
Membrane attack complex
Recruitment of PMNs
Opsonization, phagocytosis
Anaphylaxis,
Chemotaxis
(C4 + C2)
(C4bC2a) C3
C3b
C3a
Microbial surfaces
(polysaccharides)
Alternative pathway
C3 convertase

14. Differential Diagnosis
Hypocomplementemia
PIGN
MPGN
SLE
Cryoglobulinemia
Bacterial Endocarditis
Shunt nephritis
Normal complement
HUS
IgAN
HSP
Alport’s / TBMD

15. b-hemolytic Streptococci
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

16. Post Infectious GN Pathogenesis Presentation
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

17. Manifestations of PIGN
Edema 85%
HTN 60-80%
Gross hematuria %
CNS (i.e. Sz) 10%
Nephrotic syndrome rare
ARF not uncommon
C3 decreased
C4 typically normal

18. Management of PIGN 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

19. Renal Biopsy

20. Histopathology Diffuse = all glomeruli
Generalized = all segments of glomeruli

21. IgG Immunofluorescence
Starry Sky Pattern

22. Electron microscopy - Normal
Basement
membrane
Foot
processes

23. Electron microscopy of PIGN
Subepithelial immune deposits (humps)
Mesangial, subendothelial, intramembranous deposits less common
Effacement of foot processes

24. Hemolytic Uremic Syndrome
2 cases/100,000 annually
Peak incidence <5yo (6/100,000)
More common June-September
Classification
D+ diarrhea associated
Strep pneumo
Atypical HUS ADAM-TS13, C1q def

25. 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

26. Microangiopathic Hemolytic Anemia

27. Henoch Schönlein Purupura

28. 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

29. Pathogenesis of Alport’s
Abnormality of type IV collagen
Disordered basement membrane
Splitting of lamina densa of GBM

30. Crescentic GN Type Serology Primary Secondary I Anti-GBM+ ANCA-
Anti-GBM disease
Goodpasture’s II
Anti-GBM- ANCA-
idiopathic
SLE, IgAN, MPGN
III
Anti-GBM- ANCA+
Microscopic polyangiitis,
Wegener’s
Drug-induced IV
Anti-GBM+ ANCA+

31. Vasculitides C-ANCA P-ANCA Anti-proteinase 3 antibodies
Anti-myeloperoxidase antibodies
75% sensitive for Wegener’s
66% sensitive for
Microscopic polyangiitis

32. Anti-GBM Disease
Silver stain
IgG immunofluorescence