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Glomerulonephritis in children Pavlyshyn H.A. Slide 2 Slide 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 Slide 4 Slide 5 Slide 6 Slide 7 Some progress as either focal segmental glomerulosclerosis or tubulointerstitial nephritis Slide 8 8 Proteinuria asymptomatic Haematuria asymptomatic Hypertension Nephrotic syndrome Nephritic syndrome Acute renal failure Rapidly progressive renal failure End stage renal failure Slide 9 Presentation Hematuria with Proteinuria with Dysmorphic rbcs with Rbc casts Oliguria Volume overload Hypertension Slide 10 Liquid Renal Biopsy Slide 11 Urine Sediment Analysis G4 cell Slide 12 Other H&P findings Neurological changes Pharyngitis URI / sinusitis Hemoptysis Rash Murmur Arthritis Edema Slide 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 Slide 14 Differential Diagnosis Hypocomplementemia PIGN MPGN SLE Cryoglobulinemia Bacterial Endocarditis Shunt nephritis Normal complement HUS IgAN HSP Alports / TBMD Slide 15 -hemolytic Streptococci Most common organism in PIGN 20% children are asymptomatic carriers Nephritic factor Host susceptibility factors (HLA-DR) Treatment of prodromal illness doesnt prevent nephritis ASO titers are NOT helpful Slide 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 14-21 days after impetigo (upto 6 wks) Abrupt onset Slide 17 Manifestations of PIGN Edema85% HTN60-80% Gross hematuria25-33% CNS (i.e. Sz)10% Nephrotic syndromerare ARFnot uncommon C3decreased C4typically normal Slide 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