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Glomerulonephritis in children Pavlyshyn H.A..  Acute glomerulonephritis is the inflammation of the glomeruli which causes the kidneys to malfunction.

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Presentation on theme: "Glomerulonephritis in children Pavlyshyn H.A..  Acute glomerulonephritis is the inflammation of the glomeruli which causes the kidneys to malfunction."— Presentation transcript:

1 Glomerulonephritis in children Pavlyshyn H.A.

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3  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

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7 Some progress as either focal segmental glomerulosclerosis or tubulointerstitial nephritis

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

9 Presentation Hematuria  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 Microbial surfaces (polysaccharides) Alternative pathway C3 convertase C3 C3b C3a (C4 + C2) (C4bC2a) Membrane attack complex Recruitment of PMNs Opsonization, phagocytosis Anaphylaxis, Chemotaxis

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

15  -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 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 days after impetigo (upto 6 wks) Abrupt onset

17 Manifestations of PIGN Edema85% HTN60-80% Gross hematuria25-33% CNS (i.e. Sz)10% Nephrotic syndromerare ARFnot uncommon C3decreased C4typically 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 Foot processes Basement membrane

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 HUSADAM-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 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 TypeSerologyPrimarySecondary IAnti-GBM+ ANCA-Anti-GBM diseaseGoodpasture’s IIAnti-GBM- ANCA-idiopathicSLE, IgAN, MPGN IIIAnti-GBM- ANCA+Microscopic polyangiitis, Wegener’s Drug-induced IVAnti-GBM+ ANCA+Anti-GBM diseaseGoodpasture’s

31 Vasculitides C-ANCAP-ANCA Anti-proteinase 3 antibodiesAnti-myeloperoxidase antibodies 75% sensitive for Wegener’s66% sensitive for Microscopic polyangiitis

32 Anti-GBM Disease Silver stainIgG immunofluorescence


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