Clinical course and outcome of children with steroid-sensitive nephrotic syndrome.

We conducted a retrospective study on children with primary nephrotic syndrome (NS) to evaluate the clinical course and outcome of children with steroid-sensitive NS (SSNS). The medical records of 226 children, median 3.46 years (min 1.00, max 15.08) who referred to our clinics with SSNS between January 1978 and September 2005 were reviewed and entered into the study. Minimum duration of follow-up was 5 years and maximum 20 years (median 7.25 years). Of 226 patients who were treated with corticosteroids, 38 (16.8%) had no relapse but the remaining 188 (83.2%) patients experienced several relapses of which 128 patients (56.6%) required additional immunosuppressive agents for the remission. Of these, 122 (95%) were treated with levamisole, 22 (17%) with cyclosporine, 36 (28%) with cyclophosphamide, and ten (7.8 %) treated with mycophenolate mofetil. Several patients had to switch from one medication to others due to lack of response. On the last follow-up visit, 64(28.3%) patients were still under treatment, some patients had taken all of the above-mentioned drugs but still had multiple recurrences. Only 103 (45.5%) patients were in remission off the drug more than 3 years. This study shows that nearly one-third of pediatric patients with SSNS experience frequent relapses despite the combination of multiple immunosuppressive medications, which may continue until adulthood.

Prevalence of IgA deficiency in children with juvenile rheumatoid arthritis.

The purpose of this study was to investigate any association between IgA deficiency (IgAD) and juvenile rheumatoid arthritis (JRA) among Iranian children.This case-control study was carried out on 83 children who were diagnosed as JRA according to American College of Rheumatology (ACR) criteria; Patients were admitted at the rheumatology clinic of Children's Medical Center, (Tehran). Serum immunoglobulins concentrations were determined by nephelometry method. Control group was 112 healthy children who were matched for age and gender. Informed consent obtained from all parents.Selective IgA deficiency (sIgAD) was found only in a boy (1.2%) among JRA children; however, partial IgA deficiency was found in 6(7.1%) of patients with JRA and in 12(10.7%) of control subjects, this difference was not statistically significant (p=0.46). Immunoglobulins levels in patients with JRA (IgM: 126.7±57.2, IgG: 1182.3±351 and IgA:169.3±98) were significantly higher than their controls (IgM: 104±52, IgG:802±220 and IgA: 94.6±47) (p<0.05). Patients with growth failure had higher IgM, IgG and IgA levels in comparison with patients without growth failure; however, this difference was significant about IgM and IgG levels (p<0.05).In contrast to other similar studies, the number of IgAD did not differ significantly between JRA patients and their control counterpart; this might be partly due to the high rate of consanguineous marriages in Iran that resulted in increased prevalence of clinically undiagnosed partial IgAD in general population. Hence, future epidemiological studies are warranted to make it clear.

Outcome of lupus nephritis in Iranian children: prognostic significance of certain features.

The objective of this study was to determine the clinical and histopathological features and outcome of children with lupus nephritis (LN). Of 84 children with systemic lupus erythematosus (SLE), we retrospectively studied 58 children (69%) under 15 years of age with biopsy-proven LN who had been followed between October 1989 and January 2005. The mean age at diagnosis or initial referral was 10.6 +/- 2.25 years, and the mean follow-up was 5.3 +/- 4.1 years. Class IV LN was observed in 34 (58.6%) patients. The 5-year patient and renal survival rates were 82.5 and 78.5%, respectively, in the total group, and 75 and 85.8%, respectively, in patients with Class IV LN. No independent predictor of unfavorable outcome, including renal histology, was detected by multivariate analysis. The mid-term patient and the renal survival rates of Iranian children with biopsy-proven LN are high. Within 5 years of follow-up, renal histology was not a predictor for survival.