THE OUTCOME OF MINIMAL LESION NEPHROTIC S YNDROME - A SURVEY AMONG 77PATIENTS WITH A MEAN FOLLOW-UP OF 15 YEARS

From a total of 90 patients in whom minimal lesion nephrotic syndrome (MLNS) was diagnosed before the age of 18, 77 who had follow-up of mor e than five years were selected for inclusion. The survey was conducte d through home terminals of a French telecommunications system and res ponses to questionnaires were made by telephone or letter. Among the 9 0 patients : 82.2 % had corticosteroid sensitive MLNS (SSMLNS) and 17. 8 % steroid resistant MLNS (SRMLNS). Mean follow-up was 15 years (rang e, 5-30). Of the SSMLNS, 21 % never relapsed, 41 % had infrequent rela pses (IR) and 38 % were steroid-dependent (SD). The mean number of rel apses per child was 6.1 (range, 2-17); 8.5 in the SD group and 4.1 in the IR group. The annual number of relapses per child decreased with t he duration of the disease, from 1.9 during the first year to 0.4 in t he fifth. The time between relapses (in the IR group) was 17 months (r ange, 6 months-7.5 years). At the end of the observation period 68.9 % of the SSNhad been in remission for more than two years without treat ment; 26,2 % for less than two years (of whom 70 % were still on treat ment). At the end of the followup, five patients with SRMLNS were in r emission, three still had mild proteinuria, and eight had developped e nd-stage renal failure (ESRF). Of these, six had been transplanted one had died and the other was on hemodialysis. The mean duration of neph rosis before ESRF was 7.7 years (range, 2.7-18). This survey confirms the good prognosis of SSMLNS (the number of permanent remissions incre ases with time) and the bad prognosis of SRMLNS (the number of nephros es progressing to ESRF increases with the length of follow-up).