Minimal Change Disease I. Clinical manifestations. Minimal change disease is responsible for idiopathic nephritic syndrome in 15% of cases in adults and 75% of cases in children. The peak age at onset is 2-6 years. A recent history of viral upper respiratory infection is often present. The clinical presentation is usually that of the nephritic syndrome. The blood pressure, GFR and serum complement levels are usually normal. Urinalysis is not remarkable except for heavy protrinuria. In adults, this syndrome may be seen in association with lymphoma, particularly Hodgkin’s disease. II. Treatment and prognosis. The course is characterized by remissions and relapses. Corticosteroid therapy is usually effective, and the prognosis is good. A. Initial treatment in adults is with oral prednisone or prednisolone, 1-1.5 mg/kg/d9usually not to exceed 80 mg/d) in divided doses for 4weeks, about 90% of patients will respond to this regimen with resolution of abnormal proteinuria. The dose on alternate days for an additional 4 weeks after remissions, and the drug is then tapered and discontinued. The same regimen is used for treatment of re lapses. B. Patients who do not respond to the above regimen can be treated with a longer course of steroid therapy or a 4- to 8-week trial of cyclophosphsmide, 3 mg/kg/d, or chlorambucil, 0.2 mg/kg/d. Focal Glomerulosclerosis i. Clinical manifestations. Focal glomerulosclerosis occurs in 15-20% of adults with idiopathic nephritic syndrome. It occurs more frequently in males, with a mean age at onset of 20 years. The clinical presentation is similar to that of minimal change disease except that hypertension, hematuria, and progressive renal functional decline are more common. The disease is often confused with minimal change disease, and cases of treatment-resistant minimal change disease may actually be diagnosed later as focal glomeralulosclerosis. This histologic lesion can also be seen with the nephropathies associated with analgesic abuse, ureteral reflux, heroin abuse, sickle cell disease, sarcoidosis, and some types of cancer.
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