Childhood nephrotic syndrome: change in pattern and response to steroids

BACKGROUND: In our center, childhood nephrotic syndrome (NS) had been reported for over a decade to be steroid sensitive contrary to reports in other parts of Nigeria. The purpose of this study was to determine if there are changes in presentation and response to steroids, with reviews of the literature on NS. METHODS: Analysis of 28 patients seen at the University of Port Harcourt Teaching Hospital, Nigeria, from 1999-2004 with the diagnosis of NS was performed. RESULTS: There were 14 girls and 14 boys with NS. The peak age was 1-4 years. Twenty (71.4%) children had idiopathic nephrotic syndrome (INS). Four had chronic renal failure, one had sickle cell disease (HbSS), two were positive to human immunodeficiency virus (HIV) 1 and 2, and one had pulmonary tuberculosis. Anemia was found in 13 patients, while 17 had Plasmodium falciparum. Plasmodium malariae and hepatitis-B surface antigen were not isolated. Renal biopsy was performed in four patients and revealed minimal-change disease in one child, focal segmental glomerulosclerosis in two and no conclusive result in one patient. Oral prednisolone was used in INS. After one month of therapy, 16 of 20 responded, of which 12 (75%) were <5 years. The NS relapsed in 15 of 16 steroid-sensitive patients. Cyclophosphamide and levamisole were used in four and one patients with FRNS, respectively. Four (14.3%) patients died; all were secondary NS. CONCLUSION: INS remains common in our center, and the majority respond to steroid therapy