GLOMERULONEPHRITIS in children - Pediatrics

GN in children, causes, clinical features and management

1. GLOMERULONEPHRITIS (Acute and Chronic) Dr Vinod Reddy P MBBS, MD, FIAP Assistant Professor Dept of Pediatrics

2. ACUTE GLOMERULONEPHRITIS

3. Acute Nephritic Syndrome This is a syndrome of acute glomerular injury/ inflammation characterized by fever , abrupt onset of haematuria , proteinuria , high blood pressure and oliguria . This can also be referred to as Acute Glomerulonephritis

4. Signs and Symptoms Historically , nephritic syndrome has been described to present with The classical triad of; Hematuria Hypertension Edema

5. Nephritic syndrome is also characterized by PHAROH P: Proteinuria (Proteins in the urine): < 3.5g/day H: Hematuria (Blood in the urine) slight giving the urine smoky appearance A: Azotemia (Elevated blood Urea and Creatinine ): Due to retention of waste products and variable renal insufficiency. R: RBC casts present in the urine. O: Oliguria : Low urine output less than 400ml/day. H: Hypertension: High blood pressure

6. Other signs and symptoms of nephritic syndrome may include: Nocturia Fatigue Loss of appetite Nausea and vomiting Blurred vision: This is a symptom of high blood pressure .

7. Post streptococcal Glomerulo nephritis

8. Poststreptococcal Glomerulonephritis Acute GN following infection by group A beta-hemolytic streptococci is a common disorder . Streptococcal infection of the throat or skin precedes the onset of nephritis by 1 to 4 weeks .

9. Pathophysiology

10. Clinical Manifestations Poststreptococcal GN involves school-age children, more commonly boys uncommon below 3 yr. The onset is rapid

11. Clinical Manifestations Onset: 10–20 days after pharyngitis, up to 6 weeks post skin infection Oliguria , hematuria Edema (periorbital, generalized) Hypertension Malaise , GI symptoms, headache, fever

12. Diagnostic Evaluation - Urinalysis: oliguria, hematuria, proteinuria, casts - Blood: ↑BUN, ↑Creatinine, ↑ASO titer, ↓C3/C4 - Imaging: Chest X-ray (cardiomegaly, congestion), ECG

13. Management Patients with mild oliguria and normal blood pressure can be managed at home. Once acute GN has occurred , treatment with penicillin has no effect on the course of the disease, but may be given if active pharyngitis or pyoderma is present.

14. Management Diet. The intake of Na, K and fluids should be restricted Overhydration is a dangerous complication as it may increase hypertension and precipitate left ventricular failure. Patients with azotemia require accurate measurement of urine output and daily weight restriction of fluid intake to an amount equal to insensible losses and 24 hr urine output .

15. Management Diuretics. oral frusemide at a dose of 1-3 mg/kg Therapy with IV frusemide (2-4 mg/kg) is necessary in subjects with pulmonary edema. Hypertension. restriction of salt and water intake. Amlodipine , nifedipine or diuretics. Beta-blockers and ACE inhibitors carry risk of hyperkalemia For hypertensive emergencies IV nitroprusside or labetalol .

16. Management Left ventricular failure. HTN should be controlled IV frusemide given to induce diuresis If diuresis is not noted, dialysis is initiated. Prolonged oliguria . Dialysis is required in children with severe renal failure and prolonged oligoanuria , fluid overload and lifethreatening electrolyte disturbance

17. Outcome and Prognosis Excellent prognosis in childhood . The symptoms begin to resolve in the first week with loss of edema and fall in blood pressure. Gross hematuria and significant proteinuria disappear within 2-weeks , although microscopic hematuria and slight proteinuria may persist for several months. HTN subsides within 2-3 weeks .

18. Crescentic Glomerulonephritis or Rapidly progressive Glomerlonephritis

19. Crescentic Glomerulonephritis Rapidly progressive GN (RPGN) is defined as an acute nephritic illness accompanied by rapid loss of renal function over days to weeks.

20. Types of RPGN The chief forms of RPGN are: (i) immune complex crescentic GN ( Ig and C3 deposits) (ii) pauci -immune crescentic GN (related to small vessel vasculitis ; positive anca antibodies) (iii) antiglomerular basement membrane GN (with anti-GBM antibodies; linear IgG deposits).

21. Management Renal biopsy should be performed in all patients with severe nephritic features, which do not resolve within 1-2 weeks. HPE: presence of crescents ( crescentic GN) involving 50% or more glomeruli suggesting severe glomerular injury. Rx: IV and oral corticosteroids and IV cyclophosphamide , followed by maintenance immunosuppression . Plasmapheresis in pauci -immune crescentic GN and Goodpasture syndrome.

22. Nephritis in Henoch-Schonlein Purpura

23. Nephritis in Henoch-Schonlein Purpura HSP is the most common vasculitis in children Serum IgA levels may be elevated. Renal Bx : shows mesangial proliferation with deposition of IgA . Most patients recover without any specific treatment. Severe cases: combination of oral/IV corticosteroids and cyclophosphamide initially , followed by maintenance steroids and azathioprine is recommended .

24. lmmunoglobulin A Nephropathy Predominant deposition of IgA in the glomeruli C/F : recurrent episodes of gross hematuria following upper respiratory infections ; each episode lasts for 2-5 days. In between these episodes, microscopic hematuria and mild proteinuria may persist. Renal histology : mesangial proliferation of varying severity. Rx: ACE inhibitors . Corticosteroids and alkylating agents in patients with nephrotic range proteinuria .

25. Lupus Nephritis C/F : Asymptomatic proteinuria and/ or hematuria Rarely RPGN. Investigations: Renal Bx : normal glomeruli / focal or diffuse proliferative GN / membranous nephropathy. IgG and C3 deposits ANA and ds DNA autoantibodies are positive C3 levels are reduced . Rx: Corticosteroids cytotoxic agents ( cyclophosphamide , mycophenolate mofetil and azathioprine ), calcineurin inhibitors ( cyclosporine, tacrolimus ) monoclonal antibodies

26. CHRONIC GLOMERULONEPHRITIS

27. CHRONIC GLOMERULONEPHRITIS It is advanced stages of several forms of GN. Renal biopsy shows several patterns in early stages, nonspecific changes in later stages. Most glomeruli are sclerosed with corresponding tubular , interstitial and vascular changes . Poststreptococcal GN seldom leads to chronic GN.

28. CHRONIC GLOMERULONEPHRITIS Clinical Features asymptomatic detected on routine urine examination. Diagnosis CUE: proteinuria , hematuria , white cells and casts. RFT: Blood urea and creatinine levels are raised, eGFR < 30 ml/min/1.73 m 2 USG shows small kidneys with regular outline. Management There is no specific treatment for chronic GN. Treatment with steroids and immunosuppressive drugs does not offer any benefit. The blood pressure should be controlled and infections treated. CKD Rx if required