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ABSTRACT: Objective: Steroids are still the mainstay of management of nephrotic syndrome (NS). It was shown that steroids could impair growth and development of children. However, other clinical studies have shown conflicting results.Methods: Hospital records of 147 children with diagnosis of NS who were followed during 1988-2008 are reviewed relating to height measurements. All patients were treated with prednisolone and had been followed for at least five years. Height measures were transformed into standard deviation score (SDS). Information on dose and duration of prednisolone therapy, histological findings of biopsy as well as concomitant use of steroid-sparing agents (SSA) were also analyzed.Findings: Mean age at onset of NS was 5.94 years and at last follow-up visit 15.08 years. All patients had normal renal function during entire duration of the study. Analysis of the whole population did not show any significant alterations in the height SDS (HtSDS) between the first and the last follow-up visit (P=0.5; –0.76±2.0 vs. –0.89±2.05 respectively). The patients were divided into two subgroups. Subgroup A, which achieved growth improvement, was composed of 62 children (initial HtSDS -1.63; final HtSDS -0.08; P<0.001) and subgroup B, that showed growth retardation, included 85 children (initial HtSDS -0.13; final HtSDS -1.59; P<0.001).Conclusion: No statistically significant retardation of linear growth was observed in the study population as a group following treatment with prednisolone according to the guidelines of ISKDC. Although about 62 subjects had growth retardation, children treated with prednisolone were not different from those who had increased growth.
Iranian Journal of Pediatrics. 01/2011;
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ABSTRACT: Objective: Idiopathic Nephrotic syndrome (INS) is the most common form of nephrotic syndrome (NS) in children with the potential of progression to end stage renal disease (ESRD). INS is steroid-responsive in most children, but not all patients respond to it. The aim of this study was to determine the rate of steroid responsiveness in children with INS that referred to Children's Medical Center since 1995 to 2007.Methods: In as a cross sectional study, the medical records of all children with INS aged 1 to 15 years who were referred to our referral hospital was reviewed. All patients with onset of disease less than 1 year of age, spontaneous remission, secondary forms of NS associated with systemic diseases, and follow up duration of less than 12 months were excluded from the study. Patients were categorized into 6 groups: Group 1 needed biopsy prior to any treatment, group 2 non-relapsing NS, group 3 infrequently relapsing NS, Group 4 frequently relapsing NS, group 5 steroid dependent NS and group 6 steroid resistant NS.Findings: A total of 238 patients were enrolled in the study. Kidney biopsy was performed in 79 cases. Minimal change lesion (MCL) was the most common (36.7%) pathological diagnosis. Steroid responsiveness was found in 81.5% of all cases including: 96% of MCL (consisting of biopsy proven cases and presumed ones), 32% of focal and segmental glomerulosclerosis, 73% of diffuse mesangial proliferation and 58% of membranoproliferative glomerulonephritis patients. During minimal follow up period of 12 months, there were 194 patients in remission, 32 patients with active NS, and 12 patients in ESRD. Conclusion: Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy.
Iranian Journal of Pediatrics. 01/2010;
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ABSTRACT: BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is one of the most common glomerulopathies in children leading to end stage renal disease (ESRD). Different values of median renal survival have been reported among different ethnicities and races. Many factors are assumed to be responsible for ESRD in these patients. In this study, we tried to determine median renal survival (MRS) and also some clinical and histopathological features predisposing FSGSpatients to ESRD in two referral hospitals in Tehran.METHODS: The study involved 103 FSGS patients (61 males and 42 females) with a mean age of 7.08 ± 0.68 years. The diagnosis was made based on kidney biopsies. All kidney biopsies were studied by light and immunofluorescent microscopes. Ocular grids (counting squares) were used as the standard method to calculate the percentage of cortical interstitial fibrosis (CIF). The percentage of glomerular sclerosis was presented as renal injury score. Glomerular filtration rate (GFR)950 ml/min was considered as renal death or the end point. Patients were followed for 1 to 15 years, until occurrence of renal death.RESULTS: The MRS was 9.04 ± 1.8 yrs. The renal survival reached 72%, 47% and 17% after 5, 10 and 15 years, respectively. Univariate analysis showed significant reverse correlation (P50%, peritubular fibrosis, periglomerular fibrosis, tubular atrophy and CIFC20%. However, multivariate analysis revealed only a reverse correlation between renal survival with CIFC20% and also hypertension (PCONCLUSIONS: In our patients, FSGS showed a rapid course towards ESRD compared with patients of western countries. Perhaps some ethnic and genetic factors such as angiotensin converting enzyme genotypes (ACE-DD) can be considered as a cause of this rapid progression. Also, we carried this study only on children and adolescents which might change the MRS results.KEY WORDS: Focal segmental glomerulosclerosis, children, renal survival, end stage renal disease.
Journal of Research in Medical Sciences. 01/2007;
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ABSTRACT: Objective: Determining prevalence of idiopathic hypercalciuria (IH) in primary school children ofTehran.Material & Methods: We evaluated 778 primary school children (age 6-11, mean 9.1 years) in twosteps: first (Screening test), we measured urine calcium to urine creatinine ratio (UCa/UCr) and in thesecond step (Definitive test), for those children who had UCa/UCr ratio more than 0.21 mg/mg wemeasured 24 hours urine calcium excretion. Children with secondary forms of hypercalciuria wereexcluded from the study. Children with idiopathic hypercalciuria were evaluated for manifestations ofIH.Findings: Among 778 children, 195 (25.1%) had UCa/UCr ratio more than 0.21 mg/mg, but fromthese 195 children only 128 children delivered 24 hours urine samples. Among these 128 children forwhom 24 hours urine calcium measurements were done 28 children excreted more than 4mg/kg/daycalcium without hypercalcemia or any other known causes of hypercalciuria and we defined them ashaving idiopathic hypercalciuria. If all 195 suspicious cases of IH had delivered 24-hrs urine sampleswe would have 42 cases of IH.Conclusion: Prevalence of IH in our children was 5.4% and its manifestations were: hematuria,dysuria, recurrent abdominal pain, urinary incontinence, urgency, urinary tract infections andurolithiasis.
Iranian Journal of Pediatrics. 01/2007;