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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.
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3. Common causes:
Minimal lesion GN.
Focal Segmental glomerulosclerosis
Drugs & Infections
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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.
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.
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5. Selectivity of Protein
When inflammation in Nephron :
1. Highly selective: Only low molecular wt.
2. Non selective: Large Molecular Wt.
1. To detect the Glomerular damage
2. Highly selective proteinuria respond to steroid
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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
Frequent infection & muscle wasting
Features of underlying cause
Rash associated with SLE, or the neuropathy associated with DM
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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.
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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
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TC, DC, ESR, Hb
Blood Urea, Creatinine, Na, k
Total Protein <6gm%
Total Albumin < 3 gm %
Serum Cholesterol: raised
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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
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11. Differential Diagnosis:
Liver failure: Hepatitis/Cirrhosis of Liver
Acute fluid overload
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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
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13. Specific measures
1.Steroid therapy for minimal change nephropathy?
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.
Prednisolone 1mg /kg/day for 8 weeks then the dose is gradually reduced & stopped in 6 months
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Prednisolone 2?mg/kg/day till urine becomes negative for protein. Then, 1.5?mg/kg/day for 4 weeks.
Cyclophosphamide or cyclosporin
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.
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Hypercoagubility - Venous thrombosis
Hypovolaemia leads to hypovolaemic shock.
Infection ? Peritonitis, CRF, RTI
Effects due to steroid ? Osteoporosis, Diabetes, PUD, Growth retardation.
Effects due to Cyclophosphamide - Bone marrow depression, Peripheral neuropathy
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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
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