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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.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.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%).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.

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 Massonʼs 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.

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.

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 diseaseThe genomic DNA of 36 Iranian patients with ADPKD was isolated using the standard salting out method. The PCR products were directly sequenced and analyzed.The frequencies of CAGGAG, ATGGTG, GTCGTA, and GTGATG 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.Our data suggests that there is potentially a common polymorphism of

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.

Nowadays, the improvement of construction materials towards sustainable construction materials had become important. Due to the hot and sunny climate in Malaysia, the consumption on electricity increases. This causes a decrease of comfort in residence. The objectives of this research was to evaluate the thermal properties of concrete containing micro steel fibres (MSF). In this study, a series of concrete cubes that consists of different percentage of MSF (0%, 0.2%, 0.4%, 0.6%, 0.8%, 1.0%) was made in order to carry out compression test. Then, a Hot Guarded Box was used to obtain the optimum percentage of MSF in the concrete for thermal conductivity. As a result, the optimum percentage of MSF obtained from the compression test was 0.8% with a reading of 25.7Mpa. While, the optimum percentage of MSF obtained for thermal conductivity 0% with a reading of 0.34 W/mk. As a conclusion, it was proven that the inclusion of MSF in a concrete matrix will increase the thermal conductivity.

BackgroundVitamin D deficiency is known as a one of the underlying causes of Idiopathic musculoskeletal pain (IMSP). This study aimed to evaluate the correlation between serum vitamin D (Cholecalciferol) status in patient with Non-specific Musculoskeletal Pain and healthy children.Materials and MethodsSeventy-seven children (aged 3–14 years), with IMSP were included as cases and 90 healthy subjects were selected for control group. Demographic characteristics and biochemical levels of vitamin D and Parathyroid hormone (PTH), were obtained. Data were analysis using SPSS version 17.0 software.ResultsResults showed that vitamin D means levels of patients and healthy children were significantly different (19.5 + 8.84 ng/mL versus 12.7 + 11.89 ng/mL), respectively (P

Background: Vesicoureteral reflux (VUR) is a common condition among children. Although, subureteral injection is a minimally invasive new method for VUR treatment, ideal bulking agent in endoscopic treatment still remains controversial. We aimed to evaluate VANTRIS subureteral injection efficacy in VUR treatment in pediatric patients. Materials and Methods: All patients who referred to Imam Khomeini hospital in Urmia-Iran, Urology ward with VUR diagnosis that had indications for open surgery, enrolled study (during Mar 2013 to Mar 2015). Prior to intervention, VUR severity, urinary tract infection (UTI) and subsequent complications determined using urine analysis and imaging. Subsequently, single injection of the VANTRIS performed for all patients and patients underwent six-month follow up including several clinical and paraclinical evaluations. Results: 31 patients with VUR diagnosis participated; of 31 patients, 18 (58.06%) children with primary UTI who had surgery indication enrolled study; of 18 patients, seven patients (38.88%) were boy and eleven patients (61.12%) were girl with mean age of 6.88 ± 2.61 years, and out of 29 refluxing rental units (RRU), 13 (44.8%) were right and 16 (55.2%) were left kidney. In current study, patients divided to two subgroups regarding their age older than five or younger than five years old and there was no significant difference between the resolution rates following VANTRIS injection in two groups (P>0.05). Eleven (38.88%) RRU detected in boy patients, nonetheless VUR resolved in all of them, postoperatively. On the other hand, of 18 RRU in female patients, complete VUR resolution observed in 16 kidneys (88.8 %), but only 2 (11.11%) kidneys had incomplete, but significant VUR resolution, where no significant difference observed. The prevalence of reflux in patients with UTI was 30% and in patients without UTI was 17%. Conclusion: The current study indicated that in all age groups of patients, the subureteral injection of the VANTRIS was an accurate and effective treatment modality for VUR

Background: Hydatid disease is a zoonotic parasitic infection that affects humans in many parts of the world, including Iran. It is caused by the larval stage of a tapeworm called Echinococcus. Human is an intermediate host infected accidentally by the ingestion of eggs excreted in the feces of dogs. This infection most commonly involves the liver and lung. Other site localizations are rare including kidney that is seen in 2% to 4% of cases. Case Presentation: Here we report a 9-year-old boy with kidney involvement in a village near Poldasht city, West Azerbaijan, Iran, admitted on Jan 2013. He referred to our hospital with a left upper quadrant (LUQ) mass without any other symptoms, which was discovered by his mother accidentally. Sonography and CT scan with and without contrast revealed large hypodense fluid containing a mass arising from the left kidney that stretched renal parenchyma. Surgery consult was done and radical nephrectomy with left adrenalectomy and retroperitoneal lymphadenectomy due to the suspicion of malignancy was performed. In addition to surgery, he received the first dose of chemotherapy for Wilms tumor and after the pathologic report, albendazole started for him. A definitive presurgery diagnosis is not always possible by radiographic evidence. Therefore, the hydatid disease should be in the differential diagnosis of renal mass. Only surgery and pathologic findings can confirm the diagnosis. In our case, due to the lack of a definitive radiologic evidence for hydatid diagnosis, we did not start albendazole presurgery and albendazole started and continued for six months after the pathologic report confirmed the hydatid disease. Conclusions: We recommend that albendazole starts in endemic areas for abdominal masses with a non-definitive diagnosis.

Background Recently the prevalence of obesity and its comorbidities has increased in children, in the worldwide. Some evidences show that there is a reverse relation between the serum level of 25-hydroxyvitamin D[25(OH)D] and Body mass index (BMI). The purpose of this study was scrutiny response to treatment vitamin D deficiency in obese/overweight children. Materials and Methods In this cross sectional study 60 children 5 -15 years old with BMI≥85% were entered. After obtaining of informed consent the calcium, phosphorous, alkaline phosphatase, 25(OH) D and parathyroid hormone (PTH) levels were measured after 8 hours of fasting. According to intensity of vitamin D deficiency, intra muscularly vitamin D3, 300,000 to 600,000 unit was prescribed. All parameters were rechecked after 1 month. Data were analyzed with SPSS- 20 software. Results Among total 60 subjects,49 children (81.7%) were overweight and 11 children (18.3%) were obese. Also, all of subjects had vitamin D deficiency. After intervention only 35% got normal level of vitamin D. Mean level of vitamin D in girls were lower than boys. Average level of vitamin D rose significantly after intervention in both overweight and obese groups (P

Respiratory distress syndrome (RDS) is a common cause of mortality and complications in the preterm neonates. Specific treatment of this disease is endotracheal surfactant administration. Some neonates need more than one dose of drug (re-dosing) so that in addition to the cost, avoidace of treatment complications and, if necessary, timely prescribtion are of high importance. In this study, the effects of the contributing factors, including prescription or non-prescription of corticosteroid to the mother, the gestational age, fetal gender, birth weight, the first and fifth minute APGAR score, respiratory distress score, time of first dose of surfactant administration, type of delivery, type of surfactant, in the case of re-dosing of surfactant in neonates with RDS, and in NICU of Motahari Maternity-Children Hospital, Urmia, Iran were compared and the positive and negative effects of each of the above-mentioned factors were examined. Few studies have been conducted on the effect of the administration of the second dose of surfactant and its subsequent doses compared with the used doses. All hospitalized neonates, who were diagnosed with RDS after birth during 2011 to 2013, were included in the study. Data was collected from a designed form and analyzed by SPSS version 22. They included 213 (60.9%) male and 137 (39.1%) females with a mean weight of 1782.03 ± 387.04 g. 66 males (31%) and 32 females (23.4%) received more than one dose of surfactant. 227 neonates (64.9%) were discharged with a recovery and 123 neonates (35.1%) died. Findings of our study showed that neonates with the low birth weight have received more than one dose of surfactant. Moreover, neonates born between 32 to 36 weeks of gestation needed re-dosing of more surfactant. Neonates with first dose of surfactant for 6 to 24 h after birth need significantly more re-dosing of surfactant. There was a significant difference between the re-dosing of surfactant and type of delivery. In addition, re-dosing of more surfactant was needed with the increasing numbers of neonate per delivery. In contrast; the need to re-dosing of surfactant in the neoantes whose mothers had received one dose of prenatal corticosteroid was greater than the neoantes whose mothers had received two doses of prenatal corticosteroid. Moreover, the type of the surfactant products (curosurf and Survanta) had no effect on the re-dosing of doses of surfactant in the studied infants. The percentage of the use of more than one dose of surfactant was higher in male neonates than the female ones. Inaddition, the first and fifth minute APGAR scores were significantly lower in re-dosing group. In this study, the need for re-dosing of surfactant was significantly greater in the neonates with the higher respiratory distress score (>8) than the neonates with the mild to moderate respiratory distress scores (≤8). Key words: Respiratory distress syndrome, surfactant, re-dosing.

Otukesh H, Hoseini R, Rahimzadeh N, Fereshtehnejad S-M, Simfroosh N, Basiri A, Sharifian M, Hashemi GH, Falahzadeh H, Derakhshan A, Fazel M, Reiesee D, Sayedzadeh A, Vazirian S, Nikibakhsh AA, Moghadam AG, Mohamadzadeh H, Naderi A, Isfahani T, Larijani F, Shorkhi H, Pasha AA, Shahbazian H, Valavi E, Mortazavi F, Gheisari A. Outcome of renal transplantation in children: A multi-center national report from Iran. Pediatr Transplantation 2011: 15: 533–538. © 2011 John Wiley & Sons A/S. Abstract: 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 (≤18 yr) 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 10 yr (n = 91) and the ones with graft survival time of equal or less than 10 yr (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

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.

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.50 unrelated pediatric cases were studied regarding M694V, V726A, M680I, and A744S mutations using ASO-PCR method.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.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: p0.001). 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.

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).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).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.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.

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.

Background. Henoch-Schnlein 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. Copyright © 2010 A. A. Nikibakhsh et al.

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.