Tehran University Medical Journal

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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.

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The nephrotic syndrome is the most common chronic renal disease of childhood.
Materials and Methods: In this study the clinical course, risk factors for relapse and the predictors of long-term outcome of 502 patients (median age 5 years)with primary nephrotic syndrome were followed for an average of 60 months (3.5 to 240 months) from 1981 to 2000.
Results: Among the 502 patients 5 (1%) achieved spontaneous remission and 313 children were initial responder. One hundred eighty four patients received at least 1 kidney biopsy (78 prior and 106 after initiation of treatment). Of 104 children with frequently relapsing steroid sensitive and steroid dependent nephrotic syndrome, levamisole induced prolong remission in 33 ( 31.7%) of patients. Cyclophosphamid and cyclosporine A induced prolong remission in 49 (50%) of 98 and 28 (41.3%) of 68 patients respectively. At the time of the final clinical evaluation, 73 patients (14.5%) were on remission 301 (59.9%) had relapsing 43 (8.6%) had persistent nephrotic syndrome 33 (6.6%) of patients evolving to end-stage renal disease (ESRD) and 6 (1.2%) of them with chronic renal failure died (infection and cardio respiratory were the cause of death in 5 and 1 patient respectively). Young age (1-5 y) at onset of disease and atopy were identified as an independent risk factors for relapse (P0.05). Patients with steroid dependent nephrotic syndrome (SDNS) or MCNS had better response to cyclophosphamide or cyclosporin than children with steroid resistance nephrotic syndrome (SRNS) or FSGS (P0.05). Persistent proteinuria, hypertension, microscopic or macroscopic hematuia, glucosuria were associated with progression to chronic renal failure (PO.05).
Conclusion: Steroid dependency and histopathology of MCNS in patients with nephrotic syndrome were significantly associated with good long-term prognosis. In contrast persistent proteinuria, histopatholoy of FSGS, hypertension, macroscopic or microscopic hematuria, and glucosoria were significantly correlated with unfavorable long-term outcome. Additionally our study showed a positive correlation between young age and atopy with higher rate of relapse.

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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.

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Background: Nephrotic syndrome is a kidney disorder that is identified by signs of nephrosis, severe proteinuria, hypoalbuminemia, and edema. It is a component of glomerulonephrosis, in which different degrees of proteinuria may occur. The complications of this syndrome may include blood clots, infections, and high blood pressure. Essentially, decreased protein through the kidneys (proteinuria) leads to low protein levels in the blood (hypoproteinemia including hypoalbuminemia), which causes water to be drawn into soft tissues (edema). Severe hypoalbuminemia may also lead to different secondary problems, including water in the abdominal cavity (ascites), around the heart or lung (pericardial effusion, pleural effusion), high cholesterol (hyperlipidemia) and, loss of molecules regulating coagulation (increased risk of thrombosis). Other symptoms may be weight gain, feeling tiredness, and also foamy urine. This study aimed to introduce a case of successful treatment of nephrotic syndrome in twin pregnancy.

Conclusion: Early diagnosis of nephrotic syndrome and accurate prenatal care in these patients could have optimal pregnancy outcomes, especially if it was not complicated by hypertension and renal dysfunction.