Tehran University Medical Journal

Background: Cyclosporin A (CsA) is now commonly used in the management of children with steroid-dependent and steroid resistant nephoitic syndrome. It has been reported to be effective in maintaining remission in 70-100 percent of patients with SDNS but somewhat SRNS 0-100 percent. The aim of this study was to evaluate the efficacy of long-term (CsA) in children with refractory nephrotic syndrome (RNS) and steroid dependent nephrotic syndrome (SDNS).

Materials and Methods: The long-term effect of (CsA) in 91 Iranian children aged 3 months to 11 years (54 with RNS and 37 with SDNS) was assessed between 1984 and 1999. Eighty of 91 children received renal biopsy prior to introduction of (CsA), and the other 11 patients had not consent for kidney biopsy. If the patients did not show remission aftre receiving 3-6 months of (CsA), the medication was discontinued.

Results: All patient were treated with (CsA) in combination with low dose alternate day prednisolone. In children with RNS and SDNS, therapy with (CsA) induced, remission in 25 of 54 (46.2 percent) and 27 of 37 (73 percent) respectively (P<0.02). Of the 32 patients with minimal change disease (MCD), 23 (72 percent) responded to therapy, compared with 4 of 18 (22 percent) with focal segmental glomerulosclerosis (FSGS) (P<0.005). Twenty-four (48 percent) of 50 who entered complete remission, had relapse 1-12 months after cessation of (CsA). The duration between the onset of nephrotic syndrome (NS) and administration of (CsA) and sexuality of patients had no effect in result of treatment. Side effects occurred in 25 patients (27.4 percent). No patients exhibited raised transaminases, 8 (8.7 percent) of the children developed hirsutism, 7 (7.6 percent) hypertension, 7 (7.6 percent) gingival hyperplasia, (2.2 percent) neurological toxicity and 1 (1 percent) increase in serum creatinine.

Conclusion: Our findings suggest that (CsA) can be used to induce a complete remission in a significant proportion of patients with RNS and SDNS, and patients with SDNS have areasonable potential for remission than children with RNS. Resistant to steroid and cyclophosphamid.

Background: Childhood nephrotic syndrome is frequently characterized by a relapsing course. Due to their adverse effects, the use of corticosteroids for the management of frequently relapsing nephrotic syndrome is limited. Levamisole, a steroid sparing agent, has been found to have low toxicity. This study was conducted to evaluate the efficacy of levamisole in steroid-sensitive nephrotic syndrome (SDNS). 

Methods: In this retrospective study from January 1988 to September 2006, we included data from 305 pediatric SDNS patients at the Children's Medical Center clinics in Tehran, Iran. Nephrotic syndrome was diagnosed using classic criteria. None of the patients had any signs or symptoms of secondary causes of nephrotic syndrome. All had received prednisolone 60 mg/m²/day. After remission, prednisolone administration was reduced to every other day and the steroid was tapered over the next three months. With every recurrence, prednisolone was prescribed with the same dosage, but after remission it was continued at a lower dosage for another six months or longer if there was risk of recurrence. Levamisole was administered to all patients at a dose of 2 mg/kg every other day.         

Results: Patients ranged in age from 1 to 20 years (mean±SD: 4.84 ±3.1) and 70.8% were male. At the last follow up, 84 (27.5%) were in remission, while 220 (72.1%) patients had relapsed or needed a low dose of steroid. Levamisole was effective in reducing the prednisolone dosage and long-term remission in 68 (22.3%) and 90 (29.5%) cases, respectively. A comparison of before vs. after levamisole treatment revealed a had significant decrease in the number of relapses (2.05±0.88 vs. 1.1±1.23 P<0.0001) and the prednisolone dosage (0.74±0.39 vs. 0.32±0.38 mg/kg/day P<0.0001). Only one patient developed levamisole-induced neutropenia.

Conclusions: In childhood steroid-dependent nephrotic syndrome, levamisole is an efficacious, safe initial therapy in maintaining remission while decreasing steroid dose, in addition to reducing the rate of relapse.