Minimal change nephrotic syndrome in children: deaths during the first 5 to 15 years' observation. Report of the International Study of Kidney Disease in Children.

Of 521 children with a previously untreated nephrotic syndrome, as defined by proteinuria greater than or equal to 40 mg/h/m2 and serum albumin less than or equal to 2.5 g/dL, entering the International Study of Kidney Disease in Children between January 1967 and April 1976, 389 showed minimal changes on renal biopsy. Of these, seven boys and three girls died, all before July 1972. Infection was the cause of death in six patients. One child died of dural sinus thrombosis, one died as a result of cardiorespiratory failure following salt-poor albumin infusion, and another died from chronic renal failure due to focal and segmental glomerulosclerosis not apparent on initial biopsy. The mode of death in the remaining child was uncertain. Analysis according to histopathologic subgroups of minimal change disease showed no statistically significant differences in the incidence of deaths, although mesangial abnormalities and tubular atrophy were associated with higher mortality than nil disease or focal glomerular obsolescence. Nine of the ten children who died had either failed to respond to initial prednisone therapy (initial nonresponders, n = 5), or responded but relapsed during the initial 8 weeks of treatment (early relapser, n = 4), even though only one quarter of the total sample were nonresponders or early relapsers (P less than .0005). Nearly one fifth of all initial nonresponders with minimal change nephrotic syndrome died. Thus the pattern of response to initial steroid therapy in patients with minimal change nephrotic syndrome may have prognostic significance.