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Nephrotic syndrome in Pediatrics
Nephrotic syndrome is a kidney disorder characterized by heavy proteinuria, hypoalbuminemia, edema, and hyperlipidemia. It can be primary/idiopathic due to glomerular diseases like minimal change disease or secondary to infections, systemic diseases, toxins, or drugs. Clinically it presents with edema, weight gain, ascites, and complications include thromboembolic disorders and infections. Diagnosis involves urine tests showing proteinuria and low serum albumin. Treatment is initially corticosteroids but may require immunosuppressants for relapsing cases.
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Nephrotic syndrome in Pediatrics
- 1. NEPHROTIC SYNDROME BY DR BRIGHTRAICE SIAMUNYANGA 12/30/2018BRIGHT RAICE SIAMS
- 2. OBJECTIVES At the endof this lecture, students should be able to Define Nephrotic syndrome Outline the etiologic List the Clinical manifestations List the Complications Outline the Management 12/30/2018BRIGHT RAICE SIAMS
- 3. DEFINITION It is akidey disorder that causes the your body to excrete too much protein in urine. This is a clinical syndrome charecterised by: 1. Heavy proteinuria >40 mg/m2/hr 2. Hypoalbuminemia-↓ serum albumin < 25 g/L 3. Oedema 4. Hypercholestrolnemia 12/30/20 18 BRIGHT RAICE SIAMS
- 4. ETIOLOGY Primary orIdiopathic Secondary Congenital Nephrotic Syndrome 12/30/20 18 BRIGHT RAICE SIAMS
- 5. Idiopathic / primary (glomerulardisease- intrinsic to kidneys) Minimal Change disease ( >80 % ) Focal segmental Glomerulosclerosis (10%) Mesangial proliferation (5%) Membranous Nephropathy Membranoproliferative glomerulonephritis 12/30/20 18 BRIGHT RAICE SIAMS
- 6. Secondary causes Infectious Hepatitis(B,C) , HIV-1, Malaria (plasmodium malariae), Syphilis, Toxoplasmosis Post-infectious GN, HIV Systemic Disease SLE, HSP (Henoch- Schonlein purpura) , DM Toxins and allergen Bee sting, Food allergens Neoplastic Lymphoma, Leukemia Drugs Penicillamine, Gold, NSAIDS, Mercury, Lithium12/30/20 18 BRIGHT RAICE SIAMS
- 7. Congenital NS Appearsin the first 3 months of life Most cases are due to genetic causes Mutations in the gene encoding nephrin and those encoding podocin NS in the first 3 months of life may also be part of multisystemic syndromes such Pierson Syndrome -(congenital nephrotic syndrome and distinct ocular abnormalities like microcoria (small pupils ) Nail-patella Syndrome-Nail-patella syndrome is an autosomal dominant disorder characterized by dysplasia of finger nails, skeletal anomalies, and, frequently, renal disease. Denys- Dash syndrome -(triad of congenital nephropathy, Wilms tumor, and intersex disorders) Congenital infections such as syphilis and CMV 12/30/2018 BRIGHT RAICE SIAMS
- 8. CLINICAL MANIFESTATION Suddenonset of pitting edema - periorbital - scrotal or vulva - ankle or leg Weight gain Ascites - abdominal pain - malaise Diarrhea (due to intestinal edema) Respiratory distress (due to pulm. edema) Decreased urine output ±HTN and Hematuria 12/30/2018 BRIGHT RAICE SIAMS
- 9. COMPLICATIONS 1) Thromboembolic disorders 2)infections 3) Acute Kidney Failure 4) Pumonary Edema 5) Hypothyroidism 6) Hypocalcaemia 7) Anemia 8) PEM 9) Hypercholestremia 12/30/20 18 BRIGHT RAICE SIAMS
- 10. Lab Investigations UrineExamination Full Blood Count and differential count Renal parameters : Spot Urine Protein : Creatinine ratio Urinary protein excretion Liver Function Tests ( eg. serum albumin) Renal Function Tests 12/30/20 18 BRIGHT RAICE SIAMS
- 11. • Urinalysis -3+ to 4+ proteinuria • Renal Function Spot UPC ratio > 2.0 UPE > 40 mg/m2/hr Serum Creatinine – normal or elevated Serum albumin - < 25 g/L Serum Cholesterol/ TGA levels – elevated 12/30/20 18 BRIGHT RAICE SIAMS
- 12. Other investigations complement levels: C3, C4 Antistreptolysin O titre and throat swab Hepatitis B antigen Hepatitis C RPR Renal Biopsy 12/30/20 18 BRIGHT RAICE SIAMS
- 13. Indications for RenalBiopsy Age below 12 months Gross or persistent microscopic hematuria Low blood C3 Hypertension Impaired renal Function Failure of steroid therapy 12/30/20 18 BRIGHT RAICE SIAMS
- 14. DEFINITION FOR DX& TX OF IDIOPATHIC NS REMISSION: Urinary protein excretion < 4 mg/m2/hour or urine dipstix nil/trace for 3 consecutive days. RELAPSE: Urinary protein excretion > 40 mg/m2/hour or urine dipstix ++ or more for 3 consecutive days after treatment ceasation INFREQUENT RELAPSE 3 or less relapses per year FREQUENT RELAPSES: Two or more relapses within 6 months of initial response or four or more relapses within any 12 month period. 12/30/20 18 BRIGHT RAICE SIAMS
- 15. STEROID DEPENDENCE: Twoconsecutive relapses occurring during the period of steroid taper or within 14 days of its cessation. STEROID SENTITIVE: Normalization of proteinuria within 4 weeks after start of standard initial therapy with daily oral prednisolone STEROID RESISTANCE: Failure to achieve remission in spite of 4 weeks of standard prednisolone therapy. 12/30/20 18 BRIGHT RAICE SIAMS
- 16. Initial Episode NON MEDICAL Dietary advice; High protein diet, Salt moderation. Abulation Education MEDICAL Corticosteroid therapy with Prednisolone ( 2mg/kg per day for 6 weeks followed by 1.5 mg/kg single morning dose on alternate days for 6 weeks ) OR 60 mg/m2/day (max 80mg/day) for 6 weeks 40 mg/m2/48 hr (max 60mg/dose) for further 6 weeks 12/30/20 18 BRIGHT RAICE Treatment of infections If significant edema – diuretics can be used but cautiously SIAMS
- 17. • Symptomatic therapy: diuretic blood pressure control : ACEi (captopril, enalapril), angiotensin II receptor antagonist hyperlipidaemia • Immunosuppressive therapy: Steroids cyclophosphamide Cyclosporin, tacrolimus, mycophenolate mofetil 12/30/20 18 BRIGHT RAICE SIAMS
- 18. SIDE EFFECTS OFPREDINISIOLONE 12/30/20 18 BRIGHT RAICE SIAMS
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