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Purpose: Our aim was to determine association of vesicoureteral reflux (VUR) and idiopathic hypercalciuria in children with recurrent and single episode of urinary tract infection (UTI). Materials and Methods: The study group consisted of 45 children with VUR and recurrent UTI, and 2 control groups: 45 normal healthy children (control group 1) and 45 children with VUR and single episode of UTI (control group 2). Idiopathic hypercalciuria was defined as urine calcium to creatinine ratio more than 0.8 (mg/mg) in infants younger than 1 year old, and more than 0.2 (mg/mg) in older children (without any detectable causes for hypercalciuria). Results: The study group consisted of 26 (57.8%) girls and 19 (42.2%) boys, with the mean age of 41.14 +/- 22.1 months. Nine (20%) subjects had hypercalciuria. The control group 1 composed of 22 (48.9%) girls and 23 (51.1%) boys, with the mean age of 43.98 +/- 16.23 months. In this group, 6 subjects (13.3%) with hypercalciuria were detected. The control group 2 composed of 23 (51.1%) girls and 22 (48.9%) boys, with the mean age of 39.96 +/- 24.2 months. In group 2, 7 subjects (15.6%) with hypercalciuria were detected. Conclusion: Comparison between such results was not statistically significant. Despite reports of different studies about accompanying of hypercalciuria with recurrent UTI with or without anatomical abnormalities, according to the present study, idiopathic hypercalciuria is not a major contributing factor to recurrent UTI in children with VUR. [ABSTRACT FROM AUTHOR]

Renal fibrosis is a major cause of renal failure in diabetic nephropathy. Tropisetron is an antagonist of the 5HT3 receptor that exhibits anti-fibrosis effects. The present research aimed to investigate the protected role of tropisetron against renal fibrosis of diabetic nephropathy and its molecular mechanisms. For this purpose, male Wistar rats were allocated into 5 groups of control, tropisetron, diabetes, tropisetron + diabetes, and glibenclamide + diabetes (n = 7). After induction of type 1 diabetes with a single injection of STZ, tropisetron (3 mg/kg) and glibenclamide (1 mg/kg) were given to the rats daily by intraperitoneal injection for 2 weeks. The obtained data revealed that the treatment of diabetic rats with tropisetron led to a significant decrease in the elevated blood glucose, serum cystatin c, and urinary total protein (UTP) level, indicating the improvement of the impaired kidney function. Moreover, the results of Masson's trichrome staining showed that fibrosis attenuated in the kidney of diabetic rats after tropisetron treatment. RT-PCR and Western blotting revealed that TGF-β1, the apoptotic mediator, and p53 were considerably declined in the kidney of diabetic rats in response to tropisetron treatment. Meanwhile, the expressions of matrix metalloproteinase-9 (MMP-9) and matrix metalloproteinase-2 (MMP-2) were increased. These notable effects were equipotent with glibenclamide, as a standard drug, suggesting that tropisetron can alleviate renal fibrosis in diabetic nephropathy. Our data indicate that tropisetron could improve kidney function and attenuate renal fibrosis through regulation of TGF-β1, p53, and expression of extracellular matrix metalloproteinases., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Purpose: Knowing the epidemiological aspects of chronic kidney disease (CKD) in children is crucial for early recognition, identification of reversible causes, and prognosis. Here, we report the epidemiological characteristics of childhood CKD in Iran., Materials and Methods: This cross-sectional study was conducted during 1991 - 2009. The data were collected using the information in the Iranian Pediatric Registry of Chronic Kidney Disease (IPRCKD) core dataset., Results: A total of 1247 children were registered. The mean age of the children at registration was 0.69 ± 4.72 years (range, 0.25 -18 years), 7.79 ± 3.18 years for hemodialysis (HD), 4.24 ± 1.86 years for continuous ambulatory peritoneal dialysis (CAPD), and 3.4±1.95 years for the children who underwent the renal transplantation (RT) (P < .001). The mean year of follow-up was 7.19 ± 4.65 years. The mean annual incidence of CKD 2-5 stages was 3.34 per million age-related population (pmarp). The mean prevalence of CKD 2-5 stages was 21.95 (pmarp). The cumulative 1-, 5-, and 10-year patients' survival rates were 98.3%, 90.7%, and 84.8%, respectively. The etiology of the CKD included the congenital anomalies of the kidney and urinary tract (CAKUT) (40.01%), glomerulopathy (19.00%), unknown cause (18.28%), and cystic/hereditary/congenital disease (11.14%)., Conclusion: The incidence and prevalence rate of pediatric CKD in Iran is relatively lower than those reported in Europe and other similar studies. CAKUT was the main cause of the CKD. Appropriate management of CAKUT including early urological intervention is required to preserve the renal function. Herein, the long-term survival rate was higher among the children with CKD than the literature.

Background: A link between vitamin D deficiency and susceptibility to bacterial and viral infections has recently been suggested., Aim: To investigate a possible association between vitamin D deficiency and urinary tract infection (UTI)., Methods: A case-control study was undertaken comprising 75 children aged 2-7 years with UTI (cases) compared with 75 healthy controls in terms of serum 25 hydroxyvitamin D [25(OH)D] levels. Serum 25(OH)D levels were measured using a chemiluminescence assay. For cases, dimercaptosuccinic acid (DMSA) renal scan was used as the gold standard to distinguish between acute lower UTI (cystitis) and acute pyelonephritis., Results: Median (IQR) 25(OH)D levels were lower in the UTI group [14.5 ng/mL (9.4-18.8)] than in the controls [27 ng/mL (22.4-39.0)] ( p < 0.001). In addition, the prevalence of 25(OH)D levels <20 ng/mL was higher in the children with UTI than in the controls (68% vs 18%) ( p < 0.001). There was a statistically significant difference between the cystitis and pyelonephritis groups in mean (SD) serum 25(OH)D levels-18.76 (9.35) ng/mL vs 13.94 (6.97) ng/mL, p < 0.05, respectively., Conclusion: Low serum vitamin D is associated with UTI and supports the hypothesis that children with low vitamin D levels could be at greater risk of UTI.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is a delayed-onset renal disorder that results from a mutation in the PKD1 or PKD2 genes. Autosomal dominant polycystic kidney disease results in end-stage renal disease due to renal cystic dysplasia. The aim of this study was to evaluate, by exon sequencing, the disease-causing variants of PKD2 (exons 4, 6, and 8) in Iranian ADPKD patients., Methods: Genomic DNA was extracted from 3-5 ml of peripheral blood by the salting-out method. PKD2 exons 4, 6, and 8 were PCR-amplified and sequenced., Results: Three disease-causing PKD2 variants were identified; all three were missense mutations in exon 4. The mutations were AGC → ACC (c.893G>C, cDNA.959G>C, S298T), TAC → TTC (c.1043A>T, cDNA.1109 A>T, Y348F), and GAA → GAT (c.1059A>T, cDNA.1125 A>T, E353D. These novel pathogenic variants may cause loss of the normal protein function., Conclusion: Our results suggest that AGC → ACC (c.893G>C, cDNA.959G>C, S298T), TAC → TTC (c.1043A>T, cDNA.1109 A>T, Y348F), and GAA → GAT (c.1059A>T, cDNA.1125 A>T, E353D variants are common in Iranian ADPKD patients. These mutations modify the transmembrane domain and likely influence PC2 function.

Background: Autosomal dominant polycystic kidney disease (ADPKD) is a highly prevalent life-threatening monogenic disorder with high morbidity and mortality. Roughly 1:400-1000 individuals are affected with this disease worldwide. The development of ADPKD is largely attributed to mutations in the polycystic kidney disease (PKD) 1 and PKD2 genes. However, the pathogenicity of the different polymorphisms in PDK1 in the development of ADPKD remains unclear. The aim of this study was to further elucidate the role of the polymorphisms in exon 25 of the PDK1 gene in relation to the pathogenesis of ADPKD in Iranian patients., Methods: The genomic DNA of 36 Iranian patients with ADPKD was isolated using the standard salting out method. The PCR products were directly sequenced and analyzed., Results: The frequencies of CAG>GAG, ATG>GTG, GTC>GTA, and GTG>ATG polymorphisms in exon 25 of the PKD1 gene were 34 (94.44%), 33 (91.67%), 26 (72.22%), and 5 (13.89%), respectively. The most frequent polymorphism associated with ADPKD was the homozygous CAG→GAG which causes an amino acid change of Q[Gln] to E[Glu] at codon 3005., Conclusion: Our data suggests that there is potentially a common polymorphism of PDK1 among the Iranian population with ADPKD. This may aid in the diagnosis and genetic screening of at-risk patients for ADPKD.

Henoch-Schonlein purpura (HSP) is the most common childhood vasculitis. Renal involvement in HSP is one of the major causes of chronic renal failure in children. It is important to start effective and relatively safe medication to prevent end-stage renal disease (ESRD). Mycophenolate mofetil (MMF) appears to be a promising therapeutic agent in many autoimmune diseases such as lupus nephritis and vasculitis. Herein, we describe the treatment with MMF of three patients with HSP nephritis. In two cases with rapidly progressive glomerulonephritis without response to steroid, after treatment with MMF, significant improvement in kidney function and proteinuria were observed. In another patient with HSP nephritic-nephrotic syndrome who showed resistance to steroid, MMF offered a favorable effect. MMF seems to be a promising therapeutic agent in the treatment of the severe HSP nephritis.

Objective: Intractable epilepsy is a major difficulty in child neurology, because the numbers of drugs that are available for treatment are limited and new treatments such as diets must be tried. Now there are some diets available for treating patients with intractable epilepsy. The oldest diet is the classic ketogenic diet and one of the newest diets is the modified Atkins diet. Patients have a harder time accepting the classic ketogenic diet than the Atkins diet, which is easier to accept because the food tastes better. This study compares the efficacy of the ketogenic diet and the Atkins diet for intractable epilepsy in children., Materials & Methods: This study is a clinical trial survey with sample size of 40 children with refractory epilepsy who were patients at Mofid hospital in Tehran, Iran. Initially, from Jan 2005-Oct 2007, 20 children were treated with the Atkins diet, and then from Oct 2007-March 2010, the other group was treated with the classic ketogenic diet and the results were compared., Results: In this study, response to treatment was greater than a 50% reduction in seizures and at the end of first, second, and third months for the ketogenic diet were 55%, 30%, and 70% and for the Atkins diet were 50%, 65%, and 70%, respectively., Conclusion: The results of this study show that there is no significant difference between the classic Ketogenic diet and the Atkins diet at the end of first, second, and third months and both had similar responses to the treatments.

Objective: Peritoneal dialysis remains the only available option for patients which need immediate dialysis and it could be a bridge between end-stage renal failure (ESRD) and transplantation. There is a paucity of published experience of children with immediate use of permanent Tenckhoff Catheter for peritoneal dialysis from developing countries. In this study we report our experience on immediate use of permanent peritoneal access and continued peritoneal dialysis for a prolonged time., Methods: Fifty six patients were studied including 30 males and 26 females within the age range of 1 month to 14 years with mean age of 6.5 years in Urmia, Northwest Iran., Findings: No operative morbidity was seen. During a total of 499.5 continuous ambulatory peritoneal dialysis months, 16 patients had 28 episodes of peritonitis, which means a overall result of one episode per 17.8 months. There were 3 patients (5.35%) with catheter site leakage, 12 (21.4%) catheter obstructions (which led to omentectomy), 4 (7.2%) exit site infections (2 patients in the early postoperative period and 2 patients in during follow up). Death due to catheter related complications occurred in 1 per 56 patients and due to non-catheter related causes in 10 per 56 patients., Conclusion: Present results indicate that catheter-related complications were not higher than those previously reported and peritoneal dialysis could be initiated immediately after catheter implantation and could be a safe bridge between end-stage renal failure (ESRD) and transplantation.

Introduction: Henoch-Schönlein purpura (HSP) is the most common systemic vasculitis in children. Several risk factors play important role in pathogenesis of HSP. We aimed to study the MEFV gene mutations (M694V, V726A, M680I, and A744S) in Iranian children with HSP., Material and Methods: 50 unrelated pediatric cases were studied regarding M694V, V726A, M680I, and A744S mutations using ASO-PCR method., Results: 24% of cases had a mutation. 22% of cases had M694V mutations. One out of 50 (2%) patients had V726A mutation. In 76% of cases no mutation was determined. In other hand, 13 out of 100 alleles (13%) were carrier for one mutation. 12 out of 100 alleles had M694V mutations (% 12) and I out of 100 alleles had V726A mutation (%1). In 87 out of 100 alleles no mutation was detected. M680I and A744S mutations were not found in tested group. Mutation study and analysis demonstrated that the most frequent mutation was M694V (22%). Frequency of alleles were 0.12, 0.01,0,0,0.13, and 0.87 regarding M694V, V726A, M680I, A744S, total mutation, and wild type alleles, respectively. Our findings imply that M694V was dominant mutation., Conclusions: This report as the first investigation of its kind in Iranian Azeri Turkish patients implying that M694V mutations are more frequent in tested group in comparison with general population. So it is suggested that investigation of M694V mutations should be considered as genetic test for diagnosis of HSP among Iranian Azeri Turkish patients.

The outcome of pediatric renal transplantation was previously reported by a single-center study at the year 2006. Therefore, we aimed to evaluate and report the characteristics and outcome of renal pediatric renal transplantation in a multi-center nationwide study. In this nationwide report, medical records of 907 children (≤18yr) with renal transplantation in eight major pediatric transplant centers of Iran were recorded. These 907 patients received a total of 922 transplants. All children who failed to follow-up were excluded. Rather than baseline characteristics, graft and patient outcomes were considered for survival analysis. For further analysis, they were divided into two groups: patients who had graft survival time more than 10yr (n=91) and the ones with graft survival time of equal or less than 10yr (n=831). Of 922 recipients, 515 (55.8%) were boys and 407 (44.2%) were girls with the mean age of 13.10 (s.d.=3.54) yr. DGF and AR were occurred in 10% and 39.5% of the transplanted children, respectively. Transplantation year, dialyzing status before transplantation, DGF, and AR were significant enough to predict graft survival in cox regression model (overall model: p<0.001). Nowadays, there is a successful live donor pediatric renal transplantation in Iran. Graft survival has improved in our recipients and now the graft survival rates are near to international standards., (© 2011 John Wiley & Sons A/S.)

Steroid-resistant nephrotic syndrome (SRNS) in children carries a significant risk of progression to end-stage renal failure (ESRF). We report a two-step protocol adapted in children with SRNS. Thirty-seven SRNS were treated with cyclosporine A (CyA) in association with prednisolone (alternate day) for 6 months (first-step treatment). Twelve patients (32.4%) went into complete remission, and 2 (5.4%) got partial remission. The other 23 cases who were steroid and CyA resistant entered a second-step treatment with withdrawing steroids, with CyA (5 mg/kg/day) in association with mycophenolate mofetil (MMF) 30 mg/kg/day for 6 months. Complete remission was observed in 11 cases (47.82%) and partial remission in 2 cases (8.7%). After two steps of treatment, 27/37 children went into total remission. In steroid and CyA-resistant INS, the association of MMF with CyA was able to induce remission in about half cases without relevant side effects.

Background. Henoch-Schönlein purpura (HSP) is the most common childhood vasculitis with an incidence of approximately 10 per 100 000 children. There is some evidence to support steroid therapy in the treatment of severe abdominal pain, severe nephritis, and central nervous system involvement. However, the routine use of corticosteroids is controversial. Frequent relapses, lack of response to steroid, steroid dependency, and steroid side effects may occur in some patients. Mycophenolate mofetil (MMF) gains increasing popularity in the treatment of autoimmune disorders, but hitherto, the available evidence to support the use of MMF in HSP is limited to some case study reports. Case Presentation. We report six children with HSP who failed to respond to systemic steroid therapy, whereas MMF successfully treated the manifestations of the disease. Conclusion. The manifestations of HSP disappeared mainly during the first week of treatment with MMF and all the patients were in a complete remission at the end and after discontinuation of the therapy. In our experience, MMF appeared to be safe and effective for the maintenance of remission in the HSP patients.

Introduction: The aim of this study was to evaluate the accuracy of Technetium Tc 99m dimercaptosuccinic acid (99mTc-DMSA) renal scintigraphy in the diagnosis of urinary tract infection (UTI) in children with suspected infection but with a negative urine culture., Materials and Methods: The records of all children with suspected or definite diagnosis of UTI presented during a 2-year period were reviewed in this study. Abnormal findings on renal scintigraphy, voiding cystourethrography (VCUG), and ultrasonography were evaluated and compared between the patients with the definite diagnosis of UTI and those with suspected UTI and negative urine cultures., Results: Of 210 patients, 86 had a definite diagnosis of UTI (group 1) and 124 had suspected UTI without a positive culture (group 2). Abnormal findings on DMSA scans were seen in 76 patients (88.4%) in group 1 and 84 (67.7%) in group 2. Vesicoureteral reflux was detected by VCUG in 50% and 32.3% of the patients in groups 1 and 2, respectively. In group 2, vesicoureteral reflux was seen in 40.5% of the patients with abnormal DMSA scan. Ultrasonography findings were abnormal in 51.3% and 39.8% of the patients with abnormal DMSA scan findings in groups 1 and 2, respectively., Conclusion: According to our findings, in children with a negative urine culture and abnormal urinalysis, 99mTc-DMSA renal scintigraphy is helpful in diagnosing UTI and vesicoureteral reflux; we recommend VCUG when DMSA scan supports UTI despite a negative urine culture and a normal ultrasonography.