1524 SYSTEMIC DISEASE IN EPIMEMBRANOUS NEPHROPATHY (EN) OF CHILDHOOD

Abstract

Approximately 2% of nephrotic children and 20% of nephrotic adults have EN. Recent reports have shown a high incidence of etiologically associated conditions for EN. At a renal referral center, 5 children had EN in a series of 422 renal biopsies over a period of 7 yrs. There were 3 boys and 2 girls ages 2-15 yrs at onset. The mean follow up is 6 yrs with a range of I to II yrs. The initial finding in each was massive proteinuria with nephrotic syndrome (NS) except for I child who had isolated hematuria at age 2 yrs associated with a family history of renal failure. This boy developed NS 3 yrs later, at which time renal biopsy showed EN, tubular fluorescence with IgG and C′3 and circulating antitubular antibodies. The two females demonstrated evidence of systemic lupus erythematosus at the time of onset of renal disease. One boy had asymptomatic hepatitis with positive Hb-s antigen and antibody (rising titer). The other boy had negative hepatitis antigen and negative anti-DNA antibody. He progressed to renal insufficiency within 2 yrs and has had a successful kidney transplant with normal GFR for 4 years. Three others had normal GFR and one had progressive renal insufficiency at the time of follow up. Our findings confirm the high incidence of primary systemic disease (3/5 in our series) in children with EN and the very low incidence of this form of renal disease in children.

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