Reflux disease which cause proteinuria of nephrotic range-
Correct Answer: Focal segmental glomerulosclerosis
Description: Focal Segmental glomerulosclerosis is a type of glomerular disease and describes scarring (sclerosis) in your kidney. The scarring of FSGS only takes place in small sections of each glomerulus (filter), and only a limited number of glomeruli are damaged at first Focal segmental glomerulosclerosis Primary focal segmental glomerulosclerosis (FSGS) can occur in all age groups but is paicularly common in people of West African descent, who, compared with other ethnicities, have a much higher carriage rate of an apolipoprotein L1 (APOL1) gene variant that is associated with increased risk of FSGS. Histological analysis shows sclerosis initially limited to segments of the glomeruli, which may also show positive staining for deposits of C3 and IgM on immunofluorescence. Since FSGS is a focal process, abnormal glomeruli may not be seen on renal biopsy if only a few are sampled, leading to an initial diagnosis of minimal change nephropathy. In most cases the underlying cause is unknown (primary FSGS) and these patients typically present with abrupt onset of severe nephrotic syndrome. Primary FSGS may respond to high-dose glucocoicoid therapy (0.5-2.0 mg/ kg/day) but the response is rarely as rapid or complete as for minimal change disease. Immunosuppressive drugs, such as ciclosporin, cyclophosphamide and mycophenolate mofetil, have also been used but their efficacy is unceain. Progression to CKD is common in patients who do not respond to glucocoicoids and the disease frequently recurs after renal transplantation. FSGS may also be secondary to other diseases such as human immunodeficiency virus (HIV) renal disease (paicularly in African Americans), morbid obesity or chronic hypeension. In addition, it may reflect scarring from previous focal glomerular injury resulting from HUS, cholesterol embolism or vasculitis. Patients with secondary FSGS typically present with more modest proteinuria than those with primary disease and rarely exhibit full-blown nephrotic syndrome. Management of secondary FSGS is focused on treating the underlying cause and reducing proteinuria by inhibiting the renin-angiotensin system . Ref Harrison20th edition pg 277
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