CLINICOPATHOLOGICAL STUDY OF NEPHROTIC SYNDROME IN CHILDHOOD

Abstract

Renal-biopsy specimens from 145 children with the nephrotic syndrome were placed in four morphological categories: minimal changes (111 patients), focal glomerulosclerosis (12), proliferative glomerulonephritis (20), and epimembranous nephropathy (2). These were then correlated with the clinical and laboratory findings, and the response to corticosteroid and cytotoxic therapy. Children with minimal changes, who were mainly boys and of preschool age, generally had highly selective proteinuria. 97% were steroid-responsive, though most later relapsed. Children with structural glomerular alterations, who were mainly girls and of school age, usually had moderately or poorly selective proteinuria, while hæmaturia and hypertension were additional features. Only 9% responded to steroids. Focal glomerulosclerosis, which is generally a slowly progressive lesion, may be difficult to distinguish from minimal changes in its early stage. Of the three varieties of proliferative glomerulonephritis encountered in this series, the membranoproliferative form was characterised by low serum C3 (β_1c-globulin) levels and a chronic course, while the mesangial type was associated with normal C3 levels and a favourable outcome despite resistance to conventional therapy. It seems that the likelihood of an individual responding to cytotoxic drugs can be predicted from his initial steroid response alone.