Tehran University Medical Journal

Background: Nephrotic syndrome is one of the most remarkable diseases in childhood. The majority of patients have prompt response to corticosteroids.

Methods: In this study, we retrospectively evaluate the outcome of patients with steroid-responsive nephritic syndrome. Medical records from January 1996 to September 2006 were reviewed to identify all children with steroid sensitive nephrotic syndrome at the Pediatric Medical Center, Tehran, Iran. Initial steroid therapy was 60 mg/m² per day for four weeks. Levamisole, a steroid-sparing agent, was prescribed at a dose of 2.5 mg/kg on alternate days in conjunction with alternate-day prednisolone. If no benefit was observed by three months, levamisole was discontinued and immunosuppressive therapy with cyclophosphamide at a dose of 3 mg/kg daily for 8 weeks, or cyclosporin A at a dose of 3-5 mg/kg was prescribed.  

Result: Of 745 children with steroid sensitive nephrotic syndrome, 63.1% of patients were male. The most common causes were minimal change disease (98/324, 30.2%) and focal segmental glomerulosclerosis (81/324, 25%). At presentation, microscopic hematuria was found in 22.6% of the patients. During follow-up, 9.2% had no relapse at any time, while 15.8% were frequent relapsers. The remission period ranged from 3.5 to 168 months. At the last follow-up, 57.6% of the patients were in remission, 37.7% relapsed and 29 children developed chronic renal failure. The outcome of nephrotic syndrome was not associated with age or gender. The end clinical status of patients correlated with duration of remission, number of subsequent relapses and response to cytotoxic agents.

Conclusions: Steroid-responsive nephrotic syndrome in children should be followed over a long period, especially patients with early relapse. Relapse was seen in more than 90% of patients. Documentation of histopathology by renal biopsy may be helpful to identify those at increased risk for a poor outcome.

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.