Familial steroid-sensitive idiopathic nephrotic syndrome: seven cases from three families in China
The seventh case was a 3-year-old boy who was the dizygotic twin of patient 6. The onset of his proteinuria and edema occurred 2 months later than that of patient 6. The 24-hr urine protein level was 2.16 g, the serum albumin concentration was 12.4 g/L, and the cholesterol level was 11.77 mmol/L. The patient was treated with prednisone at a dose of 1.8 mg/kg/day, and remission was observed 15 days after the steroid therapy was initiated. His urinary test results have remained normal for the past 2.8 years.\nPatients 6 and 7 were born to nonconsanguineous parents. Neither boy underwent a renal biopsy. The mutational screening of the Nphs2 gene using PCR and direct sequencing revealed no significant mutations in either dizygotic twin.\nSecondary causes of nephrotic syndrome, including systemic lupus erythematosus, malignancy, hepatitis B infection, and Henoch-Schonlein purpura, were excluded in all the patients. Hematuria, hypertension, and hypocomplementemia were absent in all the cases. The clinical parameters and treatment responses to prednisone/immunosuppressants of patients 1–7 are summarized in ). This study was approved by the ethics committee of The Children's Hospital of Zhejiang University School of Medicine.