1575 IDIOPATHIC NEPHROTIC SYNDROME: LONG-TERM PROGNOSIS

Abstract

The clinical course of idiopathic nephrotic syndrome (NS) was studied in 65 children (mean age 3.3 yrs, range 1-14 yrs) who were either steroid responsive (61 pts) or had steroid-resistant minimal change disease (4 pts) at onset. All pts were followed for a minimum of 10 yrs (range 10-25 yrs) and follow-up was 15 yrs in 29 pts (45%).

Of the 61 steroid responsive pts, 53 had a frequently relapsing-steroid dependent course. Of these pts, 20 were treated with cytoxan which induced a permanent remission in 13 (mean duration 8.7 yrs, range 6-12 yrs) and significantly reduced the rate of relapses in 4 others. Of the 33 pts not given cytoxan, 35% continue to experience relapses 10-18 yrs after onset while the others have been in remission for the past 2-16 yrs (mean 7.6 yrs).

Of the 4 pts with steroid resistant minimal change disease, 3 were treated with cytoxan and all achieved a remission. Two of these pts have had no relapses 10 and 12 yrs after onset and 1 pt had a single relapse.

At the completion of follow-up (mean 14.4 yrs), all pts have normal renal function (S_cr < 1.5 mg/dl), 17% have BP ≥ 140/80 and 76% have a height which is < 50%tile (adjusted for sex and age) while 33% are < 25%tile for height. These findings suggest that in children with steroid responsive NS or steroid resistant minimal change disease there is: a) a favorable long-term outcome; b) the duration of relapsing disease is reduced by treatment with cytoxan and c) short stature is a significant consequence of therapy.

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