Long-term outcome of the difficult nephrotic syndrome in children

Abstract

To determine the long-term outcome of nephrotic syndrome (NS) in children, we studied 48 patients with the NS aged seven months to 12 years at onset and followed for a long period (3-9 years). Consanguinity was positive in 31.2%. Patients' history of atopy was present in 25%, while family history of allergy was present in 18 (37.5%) patients. Renal impairment at initial presentation was observed in 12.5% of the patients. Among 32 biopsied patients, 11 (34.3%) had focal segmental glomerulosclerosis and eight (25%) revealed mesangial IgM nephropathy. Outcome at two years of presentation showed 41.6% patients as frequent relapsers, 39.5% as steroid dependent and 18.7% as steroid resistant. Forty-three patients were available for follow-up after ten years of presentation, 22 (51%) patients had complete remission, 15 (34.8%) were steroid dependent, two (4.6%) developed chronic renal failure and two (4.6%) died. Two patients (4.6%) developed insulin-dependent diabetes mellitus, two (4.6%) had cataract and one (2.3%) had documented peritonitis. In conclusion, the high incidence of steroid-dependent, frequent relapses and steroid resistance in children can be explained by different factors, including consanguinity, atopy and severe presentation at onset of disease. We suggest longer initial treatment at onset for this group of patients. The low incidence of infection in this group needs to be addressed in future studies.