| ID | 133 |
|---|---|
| Name | B. NEPHROTIC SYNDROME |
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| Introduction | Glomerular diseases presenting as nephrotic syndromes are characterized by- 1. Overt (heavy) proteinuria- usually >3.5g/24 hours 2. Hypoalbuminaemia (<30gm/l) 3. Oedema and generalised fluid retention 4. Hypercholesterolaemia (fasting serum cholesterol level > 200mg/dl). |
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| Classification | Classification of nephrotic syndromes: 1. Minimal change nephropathy or disease: This is a primary renal disorder of nephrotic syndrome (idiopathic), most commonly seen in children and occasionally in adult. Pathogenesis is unknown, .but no immune deposition. Clinical presentation is acute and often severe nephrotic syndrome. Good response to corticosteroid (prednisolone) therapy. 2. Focal segmental glomerular sclerosis (FSGS): This is usually a primary renal disorder of idiopathic nephrotic syndrome, but rarely may be secondary to some conditions e.g heroin use, norbid obesity, HIV infection etc. If the minimal change disease exists at one end of the spectrum of the idiopathic nephrotic syndrome FSGS exists at opposite end. Pathogenesis is unknown, but in some cases circulating factors increase glomerular permeability; injury to podocytes may be a common feature. No specific immune deposition is seen, but non-specific trapping in focal scars. Clinically, although it presents features of nephrotic syndrome, but also have many nephritic features. This is less responsive to corticosteroid treatment. 3. Membranous nephropathy: This is the most common cause of idiopathic primary nephrotic syndrome in the adults. There may be secondary disorder with known cause, such as hepatitis B, endocarditis, syphilis, SLE connective tissue disease, thyroditis, & certain drugs e.g gold, penicillamin, captopril. Membranous nephropathy is an immune-mediated disease with deposition of circulating IgG granular immune-complex in the subepithelial portion of glomerular capillary walls. Histologically there is thickening of GBM, progressing to increased matrix deposition & glomerular sclerosis. Approximately one-third of this nephropathy remit spontaneously, one-third remain in a nephrotic state and one-third show progressive loss of renal function. The later two may respond to short-term treatment with high doses of prednisolone or chlorambucil (an alkylating agent). 4. Membranoproliferative glomerulonephritis (MPGN): The membrano-proliferative glomerulonephritis may be primary or secondary form. Primary form is an idiopathic syndrome that can present either nephrotic (type I) or nephritic (type II) features. Patients are mostly young adult. Type I patients usually present typical features of nephrotic syndrome. Pathogenesis is unknown, but is an immune-mediated nephropathy with deposition of plentiful of circulating IgG and IgM immunoglobulin complexes and granular C3, Clq & C4 in the subendothelium. Histologically it presents a thickened GBM with immunoglobulin deposits and abnormal mesangial cell proliferation between the GBM and the endothelial cells; this gives a characteristic splitting appearance to the glomerular capillary wall. The etiology is unknown, but there may be some associated infections, such as subacute bacterial endocarditis, upper respiratory tract infections, and hepatitis B virus infection. There is no proven treatment, but corticosteroid therapy is still tried; antiplatelet drugs (e.g aspirin) are also advisable, as it is thought that platelets are playing a role in glomerular injury in membranoproliferative glomerulonephritis. Type II patients are often present with a nephritic picture, but is less common than type I. |
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