Diseases List

ID 137
Name NEPHROTIC SYNDROME
Cause
Signs Symptoms
Diagnosis Essentials of diagnosis:1 1. Proteinuria > 3.5gm/1.73m2 per day 2. Hypoalbuminaemia (serum albumin< 3gm/dl). 3. Hypercholesterolaemia (fasting serum cholesterol level > 200mg/dl). 4. Peripheral oedema (or anasarca with ascites)
Investigations Investigations: 1. Blood: a. Urea usually normal b. Total protein less than 5gm %. c. Albumin less than 3gm %. d. Serum cholesterol raised, above 300mg %, e. E.S.R, raised. 2. Urine: Colour straw yellow, albumin coagulates on bioling & hyalline cast present but no R.B.C.
Management Management: 1. Relief of oedema & ascites: a. Rest in bed b. Salt restriction, c. High protein diet (lgm/kg/day)- if blood urea is not raised (but, now a day usually not advices), d. Diuretics: Mild oedema- bendrofluazide 5-10mg/day. Or, metolazone 5-10mg/ day. Moderate to severe oedema- frusemide 80-120mg/day. Or, bumetanide 2-3mg/day. Or/with spironolactone 100-200mg/day. 2. Treatment of proteinurea: by steroid therapy. Before starting steroid, confirm the followings- a. no haematuria b. no hypertension, c. renal function is not impaired Steroid therapy in minimal changed nephropathy: Prednisolone 60mg/m /day or Img/kg/day once daily for 4-6 weeks or until proteinuria disappears. The children respond excellent with this therapy, but about 10% of patients become steroid-resistant after 4-6 weeks. Adult patients respond less than the children and may require steroid therapy as long as 16 weeks to achieve a response. It is usually needed to continue me treatment for several weeks after complete remission of proteinuria; the cessation of treatment should be done gradually by tailing off doses over 4 weeks. Unexpectedly, a significant number of patients may relapse and may require further treatment with steroid. In case of frequent relapse and steroid resistance cyclophosphamide or chlorambucil may be given to induce subsequent remission. In case of minimal change nephropathy progression to end-stage renal disease is rare, but complications due to prolonged steroid therapy are usual consequence. N.B: For further information see under paediatric section 3. Antibiotic to prevent secondary infection. 4. Advise on discharge from hospital: Urine test for every 15 days or 1 month interval to detect the recurrence. If recurrence then give steroid again in full dose. In steroid resistant cases cytotoxic drugs like cyclophosphamide 2mg/kg per day & continued for 2 weeks after remission. 5. Treatment of complications (if any). 6. Treatment of cause (if any).
Introduction It is a clinical syndrome characterised by massive proteinurea, hypoproteinaemia, generalised oedema and hyperlipidaemia.
History
Etiology Etiology:2 Glomerular lesions resulting in nephrotic syndrome: A. Primary (or idiopathic) glomerulonephritis (GN), such as: 1. Minimal change nephropathy 2. Focal & segmental glomerular sclerosis 3. Membranous GN 4. Membranoproliferative GN or Mesangiocapillary GN (mesangial proliferative, focal proliferative) B. Secondary glomerulonephritis (GN), such as: Associated with systemic disease: 1. Systemic lupus erythematosus 2. Polyarteritis nodosa; microscopic polyartertis 3. Amyloidosis 4. Diabetes mellitus Associated with infection- 1. Bacterial endocarditis 2. Malaria 3. Hepatitis B 4. Syphilis Associated with tumours- 1. Carcinoma 2. Hodgkin’s disease 3. Chronic lymphatic leukaemia Associated with drugs- 1. Penicillamine 2. Captopril 3. Gold & mercury 4. Trimethadione 5. Phenindione 6. Heroin (contaminated).
Clinical Features Clinical features: Symptoms: 1. Gradual swelling of the whole body is seen, starting from the face & gradually involving the lower part of the body. 2. Scanty micturition. 3. Abdominal discomfort and tightness may develop. 4. Anorexia, nausea & vomiting. Signs: 1. Facies- puffiness of face with baggy eye lids. 2. Oedema present & pitting in type. 3. Blood pressure: usually normal. 4. Evidences of ascites may be present
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