Acute glomerulonephritis in children .pptx

Acute glomerulonephritis in children

1. Acute post streptococcal glomerulonephritis Dr Sajid Noor

2. Definition It is characterized by sudden onset of gross hematuria, edema , hypertension and deranged RFTs . Most common Immune-mediated , glomerular inflammatory disorder leading to acute kidney injury (AKI ). 85 % of cases follow an infection by beta-hemolytic streptococci.

3. It is classical self-limiting illness resulting from prior pharyngeal or cutaneous infection with group beta-hemolytic streptococci . Alternative etiologies S.pneumoniae ( rare), S.aureus and other non-streptococcal bacteria. Viral infections ( e.g.EBV , parvovirus B19)

4. Epidemiology The disease burden is highest in resource-poor countries. Incidence 9.5–28.5 new cases per 100,000 person-years. Glomerulonephritis risk 5 % with pharyngitis and 25 % after skin infection of nephritogenic S. pyogenes infections.

5. Pathogenesis Exact mechanism of glomerular injury is debated It though that to be an immune-complex mediated disease . Pattern of hypocomplementemia in AGN reflects activation of the alternative complement pathway (AP): plasma C3, C5, and properdin levels are transiently decreased in the presence of preserved concentrations of C4.

6. Current hypotheses: Antibodies bind to streptococcal antigen(s) planted in the glomerular basement membrane leading to alternative complement pathway activation. Two streptococcal proteins, have been identified in the GBM of APSGN patients and proposed as pathogenic antigens. Antigen-antibody complexes are formed in the circulation and deposited in the glomeruli and may incite glomerular damage through activation of the complement system.

7. APIGN due to streptococcal pharyngitis peaks during winter and early spring D ue to Skin Infection (pyoderma) peaks in late summer and early fall and the latter is more common in tropical and subtropical regions. It occurs at any age but peaks in children ages 6–10 (2–12) years. Male to female ratio 2:1.1. Siblings are also at risk of developing subclinical nephritis.

8. Clinical Features The clinical manifestations ranges from asymptomatic microhematuria to severe disease Complications due to hypertension, renal failure, and cardiac insufficiency . Hypertension Edema Hematuria Protienuria Oliguria. Acute kidney injury.

9. Hypertension (60%) Headache, vomiting, seizures, somnolence/altered mental status, Risk of posterior reversible encephalopathy syndrome (PRES) with visual changes and focal neurological signs

10. Edema. Facial/periorbital, dependent, or generalized edema (more frequently in young children) Ascites, pleural effusion, Cardiac insufficiency (orthopnea, dyspnea, cough, pulmonary crackles/edema, and gallop rhythm)

11. Hematuria. Dark-brown urine (cola- or tea-colored) in one-third of patients; remainder microscopic hematuria.

12. Proteinuria. Mild to moderate, rarely nephrotic range (>1g/m2/day) Oliguria . Transient oliguria in 50 %, complete anuria rare

13. Acute kidney injury . Nausea, vomiting fatigue, weakness, pallor Others . Back pain and abdominal discomfort, fever, weight gain (edema)

14. Investigations Urine. Dipstick analysis Hematuria, proteinuria Urine microscopy Red blood cell (RBC) and mixed cellular casts Dysmorphic RBCs Leukocytes (sterile pyuria) Proteinuria <2 g/l in 85 % of cases (U protein/creatinine <2 g/g) Nephrotic presentation may herald poor renal outcome

15. Complement Reduced plasma C3 and CH50 in >90 % of cases Normalize within 6–12 weeks after presentation. Plasma C4 generally normal.

16. Bacterial Pharyngeal swab Index patient and siblings (opportunity of preventing spread of nephritogenic strain) Culture of beta-hemolytic streptococci Rapid streptococcal antigen test Skin swab Suspected pyoderma

17. Serology . Antistreptolysin O titer (ASOT); Elevated in 70–80 % 1–5 weeks after infection, decrease to preinfection levels after several months. Unreliable for streptococcal pyoderma or infection by other organisms Streptozyme test ; Detecting antibodies against several streptococcal antigens (streptolysin O, DNaseB , hyaluronidase, streptokinase, anti-nicotinamide adenine dinucleotidase ( NADase ) Anti- Dnase B; Elevated in 80–90 % of cases of pyoderma-associated APSGN

18. Kidney biopsy Indications (rare): Alternative diagnosis (e.g., MPGN, IgA). Normal serum C3/CH50. Persistently low serum C3 or CH50 (>12 weeks after onset). Persistent GN/deteriorating renal function.

19. Natural resolution. The acute phase usually lasts 4-10 days when urine output gradually increases; edema subsides and RFTs return to normal. Gross hematuria seldom persists beyond the weeks but microscopic hematuria may persist for 1-2 years. Low grade proteinuria may be found up to months after onset of AGN. Long-term prognosis is excellent. The incidence of CKD remains 1% after APSGN

20. General/Supportive treatment Bed rest: Patient should be preferably hospitalized for close monitoring of BP and any derangement of RFTs and treatment of any complications as they arise. Antibiotics: Dietary restrictions:

21. Treatment; Hypertension and fluid overload ; Restriction of sodium and fluid intake Loop diuretics (furosemide) Antihypertensive drugs If blood pressure is not controlled with diuretics Preferably calcium channel blocker or angiotensin- converting enzyme inhibitor ( ACEi )

22. Pulmonary edema (rare); Hypertensive urgency or emergency (with or without CSN symptoms); Loop diuretics, Oxygen therapy Oral agents (nifedipine, hydralazine, minoxidil) Intravenous agents (nitroprusside, nicardipine, labetalol)

23. Hyperkalemia; Loop diuretic K restriction, sodium polystyrene sulfonate Inhaled bronchodilator, IV calcium or bicarbonate, or insulin drip If refractory, dialysis

24. Dialysis or continuous veno -venous hemofiltration Life-threatening hyperkalemia Severe fluid overload unresponsive to diuretics Rapidly progressive GN with persistent oligoanuria

25. Antibiotics; Obtain throat cultures from patient, family members, and close contacts Treat those infected to minimize spread of nephrito - genic strain Oral penicillin V for 10 days (<25 kg = 250 mg twice daily, >25 kg 500 mg twice daily) Erythromycin (40 mg/kg for 10 days) or derivative for patients allergic to penicillin

26. Prognosis and Outcome; Short- and long-term prognosis of APSGN in children is excellent. 95 % of the patients recover renal function within 3–4 weeks. Chronic or progressive kidney disease <1 % of children (higher percentage in adults). Recurrence of APSGN extremely rare due to streptococcal type-specific, long- lasting immunity and limited number of nephritogenic GAS strains.

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