Prof. Rai Muhammad Asghar Head of Paediatric Department RMC Rawalpindi

Acute Post streptococcal Glomerulonephritis

Sudden onset of Gross hematuria Edema Hypertension Renal insufficiency

Cause of AGN Post infectious (Gp A B sterp) Vasculitis HS purpura SLE Microscopic polyarteritis Polyarteritis nodosa IgA nephropathy Antiglomerular basement membrane disease

Post streptococcal Glomerulonephritis Triad of Acute nephritic syndrome Evidence of recent strep infection Low C3 level

ETIOLOGY and EPIDEMIOLOGY Infection of the throat or skin by nephrogenic strains of group A beta – hemolytic sterptococci Commonly follows streptococcal pharyngitis during cold weather months and sterptococcal skin infections or pyoderma during warm weather

Pharyngitis Pyoderma Strep type Gp A (12) Gp A (49) Climate Temperature , Cold Hot, tropical Season Wniter & spring Summer & fall Incubation period 1-2 wks 3-6 wks Sex incidence B:G 2:1 Equal

PATHOLOGY Light microscopy: Enlarged and bloodless glomeruli Diffuse mesangial proliferation with increase in mesangial matrix Polymorphonuclear leukocytes Crescent farmation and interstitial inflammation

Immunofluorescene microscopy Lumpy – Bumpy deposits of immunoglobulin and complement on GBM and in the mesangium Electron Microscopy Electron – dense deposits, or humps are observed on epithelial of the GBM

PATHOGENESIS Exact mechanism not known Morphological studies and depression in the serum complement (C3) level strongly suggest immune mediated illness

CLINICAL MANIFESTATIONS Age 5-15 year Incubation period: 1-2 weeks after pharyngitis 3-6 weeks after pyoderma Hematuria either asymptomatic microscopic with normal renal function or Gross hematuria (cola colored) with acute renal failure Oliguria Edema Hypertension Hypertensive encephalopathy

Edema results from salt and water retention and nephrotic syndrome may develop in < 5% Malaise, lethargy, abdominal or flank pain and fever are non specific features Acute subglottic edema and airway compromise Acute phase resolve within 6-8 weeks, urinary protein excretion and hypertension normalize by 4-6 weeks persistent microscopic hematuria may persist for 1-2 years

INVESTIGATIONS Urine R/E Renal function Test Serum electrolytes CXR Red blood cells Red blood cells cast Proteinuria Leukocytes Renal function Test Serum electrolytes Hyponatermia and hyperkalemia CXR

Evidence of streptococcal infection Throat culture Raised ASO titer Streptozyme Test Complement C3 Level Low Complete Blood count Hemoglobin – Low Total and differential count usually within normal limits Retics – Raised ESR- Raised

Indication for Renal Biopsy Acute renal failure Nephrotic syndrome Absence of evidence of streptococcal infection Normal complement level Hematuria, Proteinuria, diminished renal function and or a low C3 level persist more than 2 months after onset

Differential Diagnosis UTI Nephrotic syndrome CRF Liver disorders Cardiac disease Malabsorption disorder

Differential Diagnosis Membrano-proliferative glomerulonephritis IgA nephropathy Systemic lupus erythematosis Henoch-schonlein purpura Hemolytic Uremic Syndrome Infective Endocarditis

Complication Hypertension Acute renal failure Heart failure Hyperkalemia Hyperphosphatemia Hypocalcemia Acidosis Seizures Uremia

Treatment General and Supportive Care Treatment of Complications Bed Rest Antibiotics (benzyl penicillin x 10 days) Dietary Restriction Treatment of Complications Renal failure Left Ventricular failure Hypertensive

ARF Protein restriction Fluid & electrolyte balance Acid base balance Control of infections Control of seizures Anemia

Heart Failure Bed Rest Prop up O2 inhalation Digitalization Diuretics ACE inhibitors

Hypertensive Encephalopathy Labetalol 0.2 – 1.0mg/kg/dose then 0.25 – 2.0mg/kg/hour Isosorbide dinitrate (Isoket) 5µg/kg/mint Hydralazine 0.15kg/dose Furosemide ( 1-2mg/kg/dose 4-6 hrly) Captopril (0.5-5mg/kg/day 8 hrly)

Prevention Early systemic antibiotic therapy dose not eliminate the risk Family members of patients with AGN should be cultured for group A beta hemolytic streptococcus and treated if culture positive

Prognosis Complete recovery in more than 95% cases Infrequently, the acute phase be severe and lead to glomerular hyalinization and chronic renal insufficiency Recurrences are extremely rare

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