Abstract

Nineteen patients (12 female, 7 male) with membranoproliferative glomerulonephritis were followed from 6 months to 15 years. Twelve patients were followed longer than 5 years and 7 longer than 10. Sixteen patients had onset of disease before age 12 yrs. There was no relationship between age of onset of disease and type of clinical presentation (6 nephrotic, 5 nephritic, 8 nephrotic-nephritic). Initially all patients received daily prednisone therapy (2.2 mg/kg) followed by alternate day therapy for periods from 6 months to 4 years. Fourteen patients also concurrently received cytotoxic therapy for 6-18 months. in those patients with nephrotic syndrome, therapy controlled edema, proteinuria diminished but persisted. Serial biopsies in those patients on therapy revealed less cellularity, less basement membrane splitting, and less electron dense deposits in the unsclerosed glomeruli. The 2 patients with dense deposit disease showed progression of deposits. The number of completely sclerosed glomeruli increased with duration of disease regardless of therapy. All patients who were either still on therapy or had been treated more than 3 yrs. had a serum concentration creatinine (SCr) which was normal at both five (n=3) and ten (n=2) yrs. of disease activity. In those patients treated < 3 yrs. or off therapy more than 6 yrs., values of SCr were > 4.1 mg/dl in 56% (n=9) and 80% (n=5) respectively. Serum compliment levels were not helpful in following disease activity. Younger age onset of disease (<8) was associated with a poorer prognosis at both 5 and 10 yr. follow-up.

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