effect of long-term steroid therapy on linear growth of nephrotic children

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. 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. 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 [Ht[SDS]] 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 [Ht[SDS]] -1.63; final [Ht[SDS]] -0.08; P<0.001] and subgroup B, that showed growth retardation, included 85 children [initial [Ht[SDS]] -0.13; final [Ht[SDS]] -1.59; P<0.001]. 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

estimation of steroid responsiveness of idiopathic nephrotic syndrome in Iranian children

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. 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. 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. Our study results showed that 81.5% of all patients, 96.2% of MCL and 32% of FSGS patients initially responded to steroid therapy

Fungal peritonitis in Iranian children on continuous ambulatory peritoneal dialysis

Fungal peritonitis [FP], causing catheter obstruction, dialysis failure, and peritoneal dysfunction, is a rare but serious complication of peritoneal dialysis. In this study, the frequency and risk factors of FP are evaluated in children who underwent peritoneal dialysis. A retrospective multicenter study was performed at the 5 pediatric peritoneal dialysis centers in Iran from 1971 to 2006, and FP episodes among 93 children were reviewed. Risk ratios were calculated for the clinical and demographic variables to determine the risk factors of FP. Ninety-three children aged 39 months on average were included in study. Sixteen out of 155 episodes of peritonitis were fungi infections, all by Candida albicans. The risk of FP was higher in those with relapsing bacterial peritonitis [P = .009]. Also, all of the patients had received antibiotics within the 1 month prior to the development of FP. Catheters were removed in all patients after 1 to 7 days of developing FP. Six out of 12 patients had catheter obstruction and peritoneal loss after the treatment and 5 died due to infection. Fungal peritonitis, accompanied by high morbidity and mortality in children should be reduced by prevention of bacterial peritonitis. Early removal of catheter after recognition of FP should be considered

Prevalence and symptoms of idiopathic hypercalciuria in primary school children of Tehran

Determining prevalence of idiopathic hypercalciuria [IH] in primary school children of Tehran. We evaluated 778 primary school children [age 6-11, mean 9.1 years] in two steps: first [Screening test], we measured urine calcium to urine creatinine ratio [UCa/UCr] and in the second step [Definitive test], for those children who had UCa/UCr ratio more than 0.21 mg/mg we measured 24 hours urine calcium excretion. Children with secondary forms of hypercalciuria were excluded from the study. Children with idiopathic hypercalciuria were evaluated for manifestations of IH. Among 778 children, 195 [25.1%] had UCa/UCr ratio more than 0.21 mg/mg, but from these 195 children only 128 children delivered 24 hours urine samples. Among these 128 children for whom 24 hours urine calcium measurements were done 28 children excreted more than 4mg/kg/day calcium without hypercalcemia or any other known causes of hypercalciuria and we defined them as having idiopathic hypercalciuria. If all 195 suspicious cases of IH had delivered 24-hrs urine samples we would have 42 cases of IH. Prevalence of IH in our children was 5.4% and its manifestations were: hematuria, dysuria, recurrent abdominal pain, urinary incontinence, urgency, urinary tract infections and urolithiasis

Thoracic ectopic kidney with diaphragmatic hernia

Congenital thoracic ectopic kidney is a very rare developmental anomaly and the rarest form of all ectopic kidneys .it is usually asymptomatic and discovered incidentally in routine chest radiography. We report an 18-month old boy with right congenital diaphragmatic hernia with thoracic ectopic kidney and positional respiratory symptoms. Chest X- ray revealed opacity at the base of right lung. Dimercaptosuccinic acid [DMSA] scan showed right thoracic kidney. Hereby we discuss the features of congenital right thoracic ectopic kidney and review the literature in this regard

Low dose prednisolone with increase in dosage interval in frequent relapsing nephrotic syndrome

In order to avoid using cytotoxic drugs and to minimize prednisolone side effects in frequent relapsing or steroid-dependent idiopathic nephrotic syndrome, 35 patients, 3 to 15 [mean= 8.1] years of age, were studied. While in remission for at least 6 weeks, the dose of prednisolone was reduced to 0.1-0.37 [mean= 0.23] mg/kg/day as a single dose for 12 to 72 [mean= 27.6] months. It was followed by a tapering method, with gradual increase in the interval instead of decrease in the dose for about 10 to 12 months, and about 18.4 months follow-up without treatment. About 54.3% of the patients had no relapse during the treatment period and the relapse rate per patient per year was 3.94 during the preceding 12 months before the study, 0.51 during treatment [p<0.001] and 0.23 after discontinuation of the drug. Each relapse was treated by a standard dose of prednisolone for 2 months and then the low dose regimen was resumed. The patients tolerated the drug well with minimal side effects. It is concluded that long term, low-dose daily prednisolone therapy followed by gradual increase in the interval is a safe, well-tolerated and effective method of maintaining prolonged remission in most children with frequent relapsing idiopathic nephrotic syndrome