Focal segmental glomerulosclerosis in children from KwaZulu/Natal, South Africa.

AIM

The aim of this paper was to describe the clinical course, histological grading, response to therapies and complications of nephrotic children with focal segmental glomerulosclerosis (FSGS) in Durban, South Africa.

METHODS

The demographic data, response to drug therapy, complications, renal function and disease status at the last hospital visit were recorded in children diagnosed as FSGS on histology. Biopsies were graded according to the degree of glomerulosclerosis and interstitial involvement.

RESULTS

Seventy-five children, 32 Indian and 43 Black were studied. FSGS as a proportion of all children seen with nephrotic syndrome, has risen between 1970 to 1995 from 1.8% to 20% in Indian children and 5% to 28% in Black children. Of those children followed for two years or more, the two racial groups were similar with respect to age at presentation, gender, histological grading, hypertension, renal outcome and response to therapies except for cushingoid features (p < 0.7), alopecia (p < 0.003) and respiratory tract infections (p < 0.001) which occurred more frequently in Indian children. Forty-three children were followed for between 2-16 years: 14 (10 Indian and 4 Black (p < 0.5)) (29%) had grade 1 changes, 24(13 Indian, 11 Black (p < 0.98)) (56%) had grade 2 changes, 3 (Indian (p < 0.26)) (7%) had grade 3 changes and 2 (Black (p < 0.17)) grade 4 changes. Over this period 27 (63%) had normal renal function, 6 (14%) died, 4 (9%) developed impaired renal function, 6 (14%) chronic renal insufficiency and 6 (14%) end-stage renal disease. Three of the latter were transplanted. At the last hospital visit, Indian children had remitted more often (p < 0.6) and Black children suffered relapse more frequently (p < 0.03). Two children had spontaneous disease remission, and 5 had sustained remission following a course of prednisolone and/or cyclophosphamide.

CONCLUSION

This, one of the largest studies of FSGS in children, shows a marked rise in this condition and some differences between Indian and Black children. The outcome in Black children was not as poor as anticipated.