Nephrotic Syndrome

The kidney pathology is well described and all the necessary information like the etiology pathophysiology clinical manifestation diagnosis prognosis medical management is well explained

1. NEPHROTIC SYNDROME

2. INTRODUCTION Common cause for hospitalization among children. It’s a syndrome caused by renal disease that increases the permeability across the glomerular filtration barrier.

3. DEFINITION A group of clinical findings characterized by- Proteinuria Hypoalbuminemia Hyperlipidemia Edema Sometimes – hematuria , hypertension & reduced glomerular filtration rate.

4. INCIDENCE 2-7 cases per 100,000 children per year Higher in underdeveloped countries (South east Asia) Occurs at all ages but prevalent in children between the ages 1.5-6 years. It affects more boys than girls, 2:1 ratio.

5. CLASSIFICATION Idiopathic Nephrotic Syndrome- Majority cases in childhood. Autoimmune phenomenon Two types- Minimal change NS Significant change NS- Focal segmental glomerulosclerosis Membranous glomerulonephritis Membranoproliferative glomerulonephritis Rapidly progressive glomerulonephritis

6. 2. Secondary Nephrotic Syndrome- About 10% in children of all cases. Secondary to disease condition like glomerulonephritis , DM , SLE, malaria, hepatitis B, HIV, etc.

7. 3. Congenital Nephrotic Syndrome- Rare but serious condition. Associated with congenital anomalies of kidney. Autosomal recessive disease Severe renal insufficiency and urinary infections.

8. 4. Infantile Nephrotic Syndrome- Major causes- NPHS2 Diffuse mesengial sclerosis

9. ETIOLOGY & RISK FACTORS Primary Renal cause- Minimal change nephropathy Glomerulosclerosis Acute post streptococcal glomerulonephritis Immune complex glomerulonephritis .

10. Systemic cause - Infections Toxins- Hg, Bi, Au Allergic- pollen, food allergy CVS- SCD,CHF, renal vein thrombosis Malignancies- Leukemia Others- systemic lupus erythematosus

11. PATHOPHYSIOLOGY

12. CLINICAL MANIFESTATION Periorbital puffiness Pitting edema over legs Generalised edema Ascites Hypertension Loss of appetite Weight gain

13. Pleural effusion hydrothorax Hydrocele Hematuria Weakness Fever Joint pain

14. DIAGNOSIS Palpation Urine analysis-Dipstick test for proteinuria (>3.5g/ lt /day) Blood test- Blood total serum albumin- reduced serum albumin- reduced (2.5 g/dl) Serum globulin- normal or increased Cholesterol- increased Serum creatinine - normal or elevated Serum complement level- normal or low Needle biopsy of kidney

15. Renal ultrasound ECG KUB-X ray Renal scan Intravenous urogram

16. MEDICAL MANAGEMENT Steroid therapy- Prednisolone (drug of choice) Daily dose 2mg/kg/day in 2-3 divided doses for atleast 4-6 weeks Antibiotic therapy- Penicillin antibiotics Diuretics- Furosemide (1-44 mg/kg/day in 2 divided doses)

17. Albumin infusion- 1 g/kg/day Immunosuppressive drugs- Cyclophosphamide (2mg/kg daily for 12 weeks) SURGICAL MANAGEMENT Renal transplantation

18. Dietary management- High protein High carbohydrate Sodium restricted Water restricted occasionally.

19. COMPLICATION Acute renal failure Renal vein thrombosis Atherosclerosis Chronic kidney disease Fluid overload Congestive heart failure Pulmonary edema infections

20. NURSING MANAGEMENT Excess fluid volume related to decreased kidney function as evidenced by pitting edema or periorbital puffiness. Imbalanced nutrition less than body requirement related to damaged metabolism as evidenced by anorexia,loss of protein,rejection of low salt diet. Fatigue related to discomfort as evidenced by lethargy, extreme edema . Risk for infection related to immunosuppressive drugs

21. PROGNOSIS Early diagnosis and appropriate treatment 80% children recover 10-15% becomes complicated with chronic renal failure 2-4% cases may have fatal outcome

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