Pulse methylprednisolone therapy in children with resistant nephrotic syndrome

ABSTRACT

This study was conducted on 26 children with steroid-resistant idiopathic nephrotic syndrome [NS], 14 males and 12 females, ranging in age at the start of pulse methylprednisolone therapy from 14/12 to 9 10/12 years, admitted to Alexandria University Children's Hospital starting from 1-4-1996. All cases of idiopathic NS admitted during this period were treated by prednisone 2 mg/kg/day divided into 3-4 doses. If the child continues to have proteinuria [2+ or greater] after one month of this treatment, the nephrosis was considered steroid-resistant and renal biopsy was indicated to determine the precise etiology of the disease. All the 26 cases were still edematous with nephrotic proteinuria after one month of prednisone therapy. The diagnoses were minimal-change disease [MCD], 8 cases, diffuse mesangial proliferation [DMP], 12 cases, and focal segmental glomerulosclerosis [FSGS], 6 cases. All cases received multiple infusions of high dose intravenous methylprednisolone as described by Mendoza and Tune. Many of the children also received cyclophosphamide according to the criteria of the previous authors. The period of follow up [from the start of therapy till 30-6-99] ranged from 4 to 38 months. At the last follow up, the results were as follows In MCD, all cases responded with disappearance of edema and nephrotic-range proteinuria, 50% with complete remission and 50% with non-nephrotic proteinuria [partial response]. All maintained normal GFR. In DMP, normal GFR was maintained in 75% [9 cases]. Complete remission was found in 7 cases and partial response in 2 cases. End-stage renal failure [ESRF] occurred in one case and 2 cases died [one from septicemia and one from thromboembolic complications]. In FSGS, GFR was normal in 50% [3 cases], two cases with complete remission and one case with partial response. GFR was decreased in one case and ESRF developed in one case. One case died from septicemia. We concluded that pulse methyl prednisolone therapy with or without cyclophosphamide has better results than those reported for cyclophosphamide alone or cyclosporine. However, newer protocols are still needed to achieve better results: