Idiopathic nephrotic syndrome is associated with the following except:
Correct Answer: Mesangioproliferative glomerulonephritis
Description: Ans. D. Mesangioproliferative glomerulonephritis. (Ref. Robbin's Pathology8th/pg. Table 14-2)Membranoproliferative GN (MPGN) is manifested histologically by alterations in the GBM and mesangium and by proliferation of glomerular cells. It accounts for 5% to 10% of cases of idiopathic nephrotic syndrome in children and adults. Some individuals present only with hematuria or proteinuria in the non-nephrotic range; others have a combined nephrotic-nephritic picture. Two major types of MPGN (I and II) are recognized on the basis of distinct ultrastructural, immunofluorescence microscopic, and pathogenic findings. Of the two types, type I is far more common (~ 80% of cases).The Nephrotic Syndrome# The nephrotic syndrome is characterized by proteinuria, which results in hypoalbuminemia and edema. Podocyte injury is an underlying mechanism of proteinuria, and may be the result of nonimmune causes (as in MCD and FSGS) or immune mechanisms (Membranoproliferative variety).# Minimal change disease (MCD) is the most frequent cause of nephrotic syndrome in children; it is manifested by proteinuria and effacement of glomerular foot processes without antibody deposits; the pathogenesis is unknown; the disease responds well to steroid therapy.# Focal and segmental glomerulosclerosis (FSGS) may be primary (podocyte injury by unknown mechanisms) or secondary (e.g. as a consequence of prior glomerulonephritis, hypertension or infection such as HIV); glomeruli show focal obliteration of capillary lumens, hyaline deposits and loss of foot processes; the disease is often resistant to therapy and may progress to end stage renal disease.# Membranous nephropathy (MN) is caused by an autoimmune response against an unknown renal antigen; it is characterized by granular subepithelial deposits of antibodies with GBM thickening and loss of foot processes but little or no inflammation; the disease is often resistant to steroid therapy.Causes of Nephrotic Syndrome: CauseChildren-Prevalence (%)Adults-Prevalence (%)A.Primary Glomerular Disease 1Membranous GN5302Minimal-change diseaseQ65103Focal segmental glomerulosclerosisQ10354Membranoproliferative GNQ10105IgA nephropathy and others1015B.Systemic Diseases with Renal Manifestations1Diabetes mellitusNote:Approximate prevalence of primary disease is 95% of the cases in children, 60% in adults.Approximate prevalence of systemic disease is 5% of the cases in children, 40% in adults.2Amyloidosis3Systemic lupus erythematosus4Ingestion of drugs (gold, penicillamine, "street heroin")5Infections (malaria, syphilis, hepatitis B, HIV)6Malignancy (carcinoma, melanoma)7Miscellaneous (bee-sting allergy, hereditary nephritis)
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