The Nephrotic Syndrome The Nephrotic syndrome refers to a clinical complex that includes the following: Massive protei
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Initially - derangement in the capillary walls - resulting in increased permeability to plasma proteins. Protein to escape from the plasma into the glomerular filtrate - hypoalbuminemiaDrop in plasma colloid osmotic pressure and primary retention of salt and water by the kidney - generalized ed
The Nephrotic Syndrome The Nephrotic syndrome refers to a c...

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1. The Nephrotic Syndrome The Nephrotic syndrome refers to a clinical complex that includes the following: Massive proteinuria - 3.5 gm or more/day (24hours) Hypoalbuminemia, with plasma albumin levels less than 3 gm/dl Generalized edema ? anasarca Hyperlipidemia ? lipoprotein & cholesterol is increased 1 Dr S Chakradhar

2. Initially - derangement in the capillary walls - resulting in increased permeability to plasma proteins. Protein to escape from the plasma into the glomerular filtrate - hypoalbuminemia Drop in plasma colloid osmotic pressure and primary retention of salt and water by the kidney - generalized edema Hypoalbuminemia triggers increased synthesis of lipoproteins in the liver and impairment of peripheral breakdown of lipoproteins. The lipiduria - reflects the increased permeability of the GBM to lipoproteins. 2 Dr S Chakradhar

3. Common causes: Minimal lesion GN. Membranous GN. Focal Segmental glomerulosclerosis Membranoproliferative Mesangiocapillary GN Chronic pyelonephritis Diabetes mellitus. Amyloidosis. SLE. Drugs & Infections 3 Dr S Chakradhar

4. A. In children: Minimal lesion & Focal lesion are common. B . But in adult: Membranous lesion is more common than children. C. In adult: i) Diabetes mellitus. ii) Chronic pylonephritis. iii) Amyloidosis SLE & other systemic causes are common. Approximate prevalence of primary disease = 95% in children, 60% adults. Approximate prevalence of systemic disease = 5% in children and 40% in adults. 4 Dr S Chakradhar

5. Selectivity of Protein When inflammation in Nephron : 1. Highly selective: Only low molecular wt. 2. Non selective: Large Molecular Wt. Purpose: 1. To detect the Glomerular damage 2. Highly selective proteinuria respond to steroid 5 Dr S Chakradhar

6. Symptoms: Oedema Gradual swelling of the whole body is seen, starting from the face & gradually involving the lower parts of the body Abdominal discomfort & tightness may develop. Anorexia, Nausea & vomiting may be present Malnutrition Frequent infection & muscle wasting Infections Hypercoagulability Features of underlying cause Rash associated with SLE, or the neuropathy associated with DM 6 Dr S Chakradhar

7. Signs: Puffiness of Face with baggy eyelids. Oedema: Pitting in type & present over lower limbs, sacrum BP usually normal Evidence of Ascites may be present. In Lungs : Pleural Effusion Examination should also exclude other causes of gross edema?especially the cardiovascular and hepatic system. 7 Dr S Chakradhar

8. Heat coagulation Test (bed side test) Two thirds of a test tube is filled with urine. The upper third of the tube is heated up to boiling point over a flame. If white cloud appears, a few drops of 5% acetic acid is added and the tube is reheated. If the white cloud increases (egg white colour) it indicates the presence of albumin. But if the cloud disappears, indicates the presence of phosphate 8 Dr S Chakradhar

9. Investigation Blood TC, DC, ESR, Hb Blood Urea, Creatinine, Na, k Total Protein <6gm% Total Albumin < 3 gm % Serum Cholesterol: raised 9 Dr S Chakradhar

10. Investigation Urine (Routine & C/S) 24 hours urinary total protein : Massive proteinuria - > 3.5 g/ day ? Chest X-ray ( to see pleural effusion) According to secondary cause like DM, SLE, hepatitis etc Renal Biopsy - Confirmatory 10 Dr S Chakradhar

11. Differential Diagnosis: Heart failure Liver failure: Hepatitis/Cirrhosis of Liver Acute fluid overload 11 Dr S Chakradhar

12. General measures 1.Diet : salt restriction (No added salt), Avoidance of High protein diet 2. Control of Oedema: Diuretics- spironolactone or thiazide or frusemide 40 mg Frusemide at 8 am 40 mg at 2 pm with k supplement 3. Daily wt. of the pt. to detect progress of oedema 4. To prevent secondary infection give Antibiotics 5. Atrovastatin for hyperlipidemia 12 Dr S Chakradhar

13. Specific measures 1.Steroid therapy for minimal change nephropathy? i)In Children 60?mg/m2/day in 3 divided doses for 4 weeks followed by 40?mg/m2/day in a single dose on every alternate day for 4 weeks. ii)In Adults Prednisolone 1mg /kg/day for 8 weeks then the dose is gradually reduced & stopped in 6 months 13 Dr S Chakradhar

14. Relapses Prednisolone 2?mg/kg/day till urine becomes negative for protein. Then, 1.5?mg/kg/day for 4 weeks. Frequent relapses Cyclophosphamide or cyclosporin 2.Membranous Glomerulonephritis Treated with corticosteroids & immunosuppressive drugs 3.Treatment of the underlying disease such as SLE 4. Achieving stricter blood glucose control if diabetic. 5.Blood pressure control with ACE inhibitors are the drug of choice. Independent of their blood pressure lowering effect, they have been shown to decrease protein loss. 14 Dr S Chakradhar

15. Complications: Hypercoagubility - Venous thrombosis Hypovolaemia leads to hypovolaemic shock. Pulmonary edema Infection ? Peritonitis, CRF, RTI Effects due to steroid ? Osteoporosis, Diabetes, PUD, Growth retardation. Effects due to Cyclophosphamide - Bone marrow depression, Peripheral neuropathy 15 Dr S Chakradhar

16. Prognosis: Depends on the cause Usually good in children, MCD responds very well to steroids and does not cause chronic renal failure. Other causes such as FSG frequently lead to ESRD Factors associated with a poorer prognosis - level of proteinuria, BP control and GFR 16 Dr S Chakradhar

17. 17 Dr S Chakradhar


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