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Glomerulonephritis in children

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 Post-Streptococcal Glomerulonephritis

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Glomerulonephritis in children

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  1. Glomerulonephritis in children Pavlyshyn H.A.

  2. 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

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

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

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

  6. Liquid Renal Biopsy

  7. Urine Sediment Analysis G4 cell

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

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

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

  11. 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

  12. Post Infectious GN Pathogenesis • 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

  13. Manifestations of PIGN • Edema 85% • HTN 60-80% • Gross hematuria 25-33% • CNS (i.e. Sz) 10% • Nephrotic syndrome rare • ARF not uncommon • C3 decreased • C4 typically normal

  14. 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

  15. Renal Biopsy

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

  17. IgG Immunofluorescence Starry Sky Pattern

  18. Basement membrane Foot processes Electron microscopy - Normal

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

  20. 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

  21. 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

  22. Microangiopathic Hemolytic Anemia

  23. Henoch Schönlein Purupura

  24. 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

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

  26. Crescentic GN

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

  28. Anti-GBM Disease Silver stain IgG immunofluorescence

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