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Background: Chronic renal failure and hemodialysis affect many ECG parameters which can affect cardiac repolarization. Objective: To investigate the change in ventricular repolarization before and after kidney transplantation in children. Methods: A total of 45 children with end-stage renal disease, 45 children at least 6 months after successful renal transplantation, and 45 normal age-matched subjects were enrolled into this study. A 12-lead ECG was recorded in the 3 groups. QT dispersion, QTc dispersion, and T peak to T end (TPE) dispersion were measured. Results: In the patients before and after renal transplantation and the normal children, respectively, the mean±SD QT dispersion was 0.083±0.033, 0.056±0.029, and 0.033±0.016 (p<0.01); the mean±SD QTc dispersion was 0.104±0.038, 0.066±0.033, and 0.039±0.020 (p<0.01); the mean±SD TPE interval dispersion was 0.060±0.021, 0.045±0.021, and 0.034±0.019 (p<0.01). There was a significant correlation between left intra-ventricular diastolic diameter and QT dispersion, QTc dispersion, and TPE dispersion. The systolic velocity of the mitral valve also correlated with TPE dispersion (r=0.44, p=0.01). Conclusion: In children with chronic renal failure, indices of ventricular repolarization improve after transplantation, though they still remain longer than the normal values. [ABSTRACT FROM AUTHOR]

Minimal Change Disease (MCD) and Focal and Segmental Glomerulosclerosis (FSGS) are often studied together, because both present with heavy proteinuria and the nephrotic syndrome. The precise distinction between MCD and FSGS is sometimes difficult because of inadequate number of glomeruli for definite diagnosis. Some evidence suggests that markers of lipid peroxidation, such as Malondialdehyde (MDA) is an index of free radical mediated injury and may be involved in the pathogenesis of FSGS. In this study, we assessed the immuno-reactivity of MDA, the end product of lipid peroxidation in glomeruli of patients with idiopathic FSGS, MCD as well as normal controls (NC). Our results showed that the immunostaining level of MDA was significantly higher in patients with FSGS (mean = 1.5) than in either patients with MCD (mean = 0.16) or normal controls (mean = 0.11) with P value < 0.001. Glomerular MDA level correlated well with the degree of glomerulosclerosis in patients with idiopathic FSGS. Our data demonstrates that the glomerular level of MDA is higher in idiopathic FSGS than MCD. We suggest that MDA immunostaining can be helpful in differentiating between FSGS and MCD in problematic cases and when we do not have enough glomeruli for definite and correct diagnosis.

A 15-year-old girl, a known case of Bartter′s syndrome (BS) for 7 years, developed severe pain in her right knee and right and left ankle. Her older sister had BS and developed end-stage renal disease (ESRD) at the age of 14 years. Her serum uric acid was 12.6 mg/dL, 6 months ago, and 15.4 mg/dL in her recent lab data. Hyperuricemia and gouty arthritis are commonly seen in adults with BS, but to our knowledge there is no report of gouty arthritis in pediatric literature.

Abstract Background Arteriosclerosis and emphysema develop in individuals with Schimke immuno-osseous dysplasia (SIOD), a multisystem disorder caused by biallelic mutations in SMARCAL1 (SWI/SNF-related, matrix-associated, actin-dependent regulator of chromatin, subfamily a-like 1). However, the mechanism by which the vascular and pulmonary disease arises in SIOD remains unknown. Methods We reviewed the records of 65 patients with SMARCAL1 mutations. Molecular and immunohistochemical analyses were conducted on autopsy tissue from 4 SIOD patients. Results Thirty-two of 63 patients had signs of arteriosclerosis and 3 of 51 had signs of emphysema. The arteriosclerosis was characterized by intimal and medial hyperplasia, smooth muscle cell hyperplasia and fragmented and disorganized elastin fibers, and the pulmonary disease was characterized by panlobular enlargement of air spaces. Consistent with a cell autonomous disorder, SMARCAL1 was expressed in arterial and lung tissue, and both the aorta and lung of SIOD patients had reduced expression of elastin and alterations in the expression of regulators of elastin gene expression. Conclusions This first comprehensive study of the vascular and pulmonary complications of SIOD shows that these commonly cause morbidity and mortality and might arise from impaired elastogenesis. Additionally, the effect of SMARCAL1 deficiency on elastin expression provides a model for understanding other features of SIOD.

Introduction. The pathophysiology of urolithiasis in infancy is not well known. The aim of this study was to investigate whether infants with urolithiasis have higher serum levels of vitamin D, as a possible risk factor for urolithiasis, compared to infants without urinary calculi. Materials and Methods. In this case-control study, 36 infants with urolithiasis (age range, 2.5 to 24 months) were enrolled as well as 36 age- and sex-matched infants without urolithiasis. Random urine samples were tested for calcium, phosphorous, oxalate, citrate, uric acid, sodium, potassium, magnesium, and creatinine levels, and also nitroprusside test was done on the samples. Serum levels of potassium, urea nitrogen, creatinine, 25-hydroxyvitamin D3, parathyroid hormone, calcium, phosphorous, and uric acid were measured in all of the infants with urolithiasis. Serum levels of 25-hydroxyvitamin D3 were also measured in the control group. Results. Serum levels of 25-hydroxyvitamin D3 were significantly higher in the infants with urolithiasis than in the controls (33.85 ± 14.78 ng/mL versus 18.26 ± 7.43 ng/mL, P < .001). Nine infants in the urolithiasis group (25%) were found to have hypercalcemia; 3 of these cases also had hypervitaminosis D. Hypercalciuria was detected in 10 infants with urolithiasis (27.8%), hypocitraturia in 6 (16.7%), hypomagnesiuria in 3 (8.3%), and hyperoxaluria in 1 (2.8%). Nineteen infants with urolithiasis had at least one metabolic disorder. Conclusions. High serum levels of vitamin D may play an important role in the pathogenesis of urolithiasis in infants with hypercalcemia. We recommend evaluation of vitamin D levels in these infants. [ABSTRACT FROM AUTHOR]

Introduction. Urinary tract infection (UTI) is common after pediatric kidney transplantation. The purpose of this study was to evaluate the prevalence of UTI and its risk factors in children and adolescents with kidney transplantation in Shiraz Transplant Center. Materials and Methods. All children with kidney transplantation from 1992 to 2008 who were under regular follow-up were included in this retrospective study. Confirmed episodes of UTI after the 1st month of kidney transplantation were reviewed. Results. Of the 216 patients younger than 19 years at the time of transplantation, 138 were included. The mean age at the time of kidney transplantation was 13.6 ± 3.5 years. Urinary tract infection was documented in 24 patients (15 girls and 9 boys), of whom 12 experienced 1 episode, 4 had 2 episodes, and 8 had more than 2 episodes, during a median follow-up period of 54 months. Of the patients with UTI, 14 (58%) had urinary reflux-obstruction disorders as the primary kidney disease, 6 (25%) had suffered hereditary diseases, 3 (12.5%) had glomerular disease, and 1 (4.5%) had a urinary calculus. Occurrence of UTI was not significantly different among children with different primary kidney disease (P = .22). Despite using prophylactic antibiotics after the 1st month of kidney transplantation in all 5 patients with neurogenic bladder, they all experienced recurrent UTI. Conclusions. Despite discontinuation of antibiotic therapy, UTI was uncommon in children after the first month of transplantation. Two significant risk factors for UTI were female gender and neurogenic bladder in this transplant population. [ABSTRACT FROM AUTHOR]

Objective: Current conventional formulas do not predict the expected changes in serum sodium after administration of various fluids to correct serum sodium abnormalities. The Adrogué-Madias formula is currently the preferred and widely used fluid prescription for adult patients with dysnatremias, but its therapeutic efficacy has not been validated in pediatric patients., Methods: In this prospective study, we used the Adrogué-Madias formula for calculating the appropriate rate of various fluids administration to correct serum sodium abnormalities in 7 critically ill children with acute dysnatremias., Results: After administration of various intravenous fluids using the Adrogué-Madias formula, the anticipated as well as the achieved sodium concentrations were almost similar., Conclusions: This study demonstrates that the use of the Adrogué-Madias quantitative formula allows to calculate the appropriate rate of administration of various fluids. The calculated fluid administration resulted in the subsequent actual laboratory values and clinical changes., Competing Interests: Disclosure: The authors declare no conflict of interest., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Background: Idiopathic nephrotic syndrome (INS) is one of the chronic diseases in children and it is important to identify its related factors. The present study aimed at investigating the relationship between H. pylori infection and the incidence and recurrence of idiopathic nephrotic syndrome in children., Methods: The total number of case participants was 40 and the total number of control participants was 41. Based on the number of cases of nephrotic syndrome (NS), the same number of healthy children of the same age and gender were selected as the control group. The information and data collected include demographic characteristics of patients, duration of disease, number of recurrences, blood pressure and blood excretion in urine, height, and weight, and presence of gastrointestinal symptoms on the checklist. The data were entered into SPSS and analyzed at a significance level of 05., Results: From 81 participants in the study, 11 (13.75%) cases were H. pylori positive, of whom 7 (17.5%) cases were in the control group and 4 (10%) cases were in the patients' group. There was no significant difference between the two groups in terms of H. pylori infection rate (P = 0.863). Moreover, there were no significant differences between the patients suffering from the nephrotic syndrome in terms of height, weight, blood pressure, hematuria, duration and recurrence of the disease (p>0.05)., Conclusion: According to the evaluations performed in the present study, there was no relationship between low birth weight, blood pressure, disease duration, and disease recurrence with NS., Competing Interests: The authors declare that they have no conflict of interest.

Liver diseases in children and adolescents are a significant and arising public health issue and should be surveyed from different dimensions (clinical and para-clinical, psychological, socio-economic) and in diverse populations. Shiraz Liver Transplant Center, Shiraz, Iran is the only center for pediatric liver transplantation and its pre-operative evaluations. This provides a unique and valuable situation for studying this vulnerable population. The Shiraz Pediatric Liver Cirrhosis Cohort Study (SPLCCS) was established to assess cirrhotic children, the course of their disease, and treatment over time. This cohort study aimed to prospectively evaluate the natural course and factors that contributed to complications and death of children with chronic liver disease in the region. SPLCCS was launched in September 2018 after obtaining ethical approval; until August 2022, 370 children with end-stage liver disease were enrolled and followed every six months. Here, the cohort's features, the included population's baseline characteristics, and primary outcomes are reported., (© 2023 The Author(s). This is an open-access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.)

Background: Solid organ transplant recipients are vulnerable to various unusual infections. Visceral Leishmaniasis (VL) is a protozoal opportunistic infection, which may affect the immune-suppressed hosts and solid organ transplant recipients. The BK virus infection is an evolving challenge in kidney transplant recipients. However, there are very few reports of BK virus (BKV) nephropathy involving the native kidney in liver transplant recipients. To the best of our knowledge, this is the first report of the simultaneous occurrence of these rare infections in a liver transplant recipient., Case Report: The patient was a 9-year-old girl, a case of liver transplantation who presented with the incidental finding of proteinuria, azotemia, and cytopenia. Investigations revealed that she had concomitant BKV nephropathy and visceral leishmaniasis. Both infections were successfully treated., Conclusion: BK virus should be considered as a cause of nephropathy in liver transplant recipients. The presenting features of fever, cytopenia, and splenomegaly in a post-transplant patient should remind of unusual infections such as VL other than the common post-transplant conditions., (© 2021 Wiley Periodicals LLC.)

Rapid-onset obesity with hypoventilation, hypothalamic dysfunction, and autonomic dysregulation (ROHHAD) syndrome is a rare, life threatening disease with unknown etiology. Dysnatremia is a common finding in these patients. Here we present a 12-year-old boy with multiple admissions due to hypernatremia and was repeatedly misdiagnosed. An eventual diagnosis of ROHHAD syndrome was made by integration of the previous ignored findings of sleep apnea and obesity. The diagnosis of this rare but potentially fatal syndrome should be considered in patients with dysnatremia associated with obesity and sleep apnea disorders.

Purpose: Renal calculi are becoming more common among children. Although, extracorporeal shock wave lithotripsy (ESWL) is the first choice in this age group, minimal invasive surgeries, such as percutaneous nephrolithotomy (PCNL), are indicated for some patients. Recently, PCNL devices have become smaller in size with acceptable efficacy and lower complications. We evaluated the outcomes and complications of mini-PCNL (MPCNL) surgery in our referral training centers. Materials and Methods: Between September 2012 and January 2020, a total of 112 children under the age of 18, who had shown failure of ESWL, and/or their parents refused to do it, underwent MPCNL (15 Fr). The patients' profiles were reviewed for data collection including preoperative and stone data, operation information, and postoperative complications. Results: Of 112 patients, 69 were boys, and 43 were girls. Their mean age was 8.6 years (14 months to 18 years). Mean stone size was 20 mm (14-34 mm). Seventy-four cases had renal pelvic stone, 22 had pelvis and lower pole, and 16 had staghorn. The mean operation time was 65 min (35-100 min), and mean radiation time was 0.6 min (0.2-1.4 min). Low-grade fever was detected in 14 patients (12.5%). Four patients needed blood transfusion and two had increased creatinine, which improved with conservative management. One patient developed urosepsis that resolved with antibiotic therapy. None of the patients had kidney perforation or other organ injury or death. Early stone-free rate (SFR) after operation was 90.2% (101 patients). Six patients had residual fragment <5 mm, which passed spontaneously in 2 weeks after operation (total SFR 95.3%). Three patients underwent second-look nephroscopy, and ureteroscopy was done for two patients due to migrated stone fragments to the distal ureter. Conclusion: MPCNL is recommended as a safe alternative option for treatment of the nephrolithiasis in children with good outcome and acceptable complications., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2021 Hosseini, Irani, Altofeyli, Eslahi, Basiratnia, Haghpanah, Adib and Ahmed.)

Background: Coronavirus disease 2019 (COVID-19) is thought to cause kidney injury via a variety of mechanisms. The most common reported kidney injury following COVID-19 infection is acute tubular injury (ATI); however, the procoagulant state induced by the virus may also damage the kidneys., Case-Diagnosis/treatment: Herein, we report two cases of acute necrotizing glomerulonephritis (GN) with fibrinoid necrosis in the context of COVID-19 infection. The one with more chronic features in the kidney biopsy progressed to permanent kidney failure but the second one had an excellent response to glucocorticoid pulse therapy with subsequent normal kidney function at 2-month follow-up., Conclusions: Both reported cases had an acute presentation of kidney injury with positive nasopharyngeal PCR test for COVID-19. Based on the data review by the researchers, this is the first report of acute necrotizing GN associated with COVID-19 infection.

Introduction: Renal dysfunction is among the common and considerable complications after liver transplantation and is principally attributable to immunosuppressive medications .The purpose of this study was to define the prevalence of hypertension and renal dysfunction among pediatric liver transplant recipients., Methods: 46 pediatric liver transplant recipients were assessed for hypertension utilizing ambulatory blood pressure monitoring (ABPM), and glomerular, and tubular renal function at the transplant clinic of Shiraz University of Medical Sciences. Results were analyzed using SPSS 19 and P value < .05 was considered statistically significant., Results: The mean age of the patients was 12.2 ± 3.3 years and 24 of them were female. Considering ABPM measurements 20 patients (43.5%) were hypertensive, 37% were systolic and 36.6% were diastolic non-dippers respectively. eGFR was calculated based on different formulations and Cystatin C-based equation estimated lower GFR than Cr-based equation. Micro-albuminuria was noticed in 26.1%. Additional parameters of tubular dysfunction included hyperuricosuria (4.3%), hypercalciuria (6.5%), abnormal fractional excretion of Mg (FeMg) (43.5%), abnormal tubular reabsorption of phosphate (TRP) (4.3%), and abnormal fractional excretion of uric acid (FEUA) in 13% of the patients. We noticed a statistically significant negative correlation between hypercalciuria, microalbuminuria, FeMg (P < .05) and GFR., Conclusion: Renal function impairment and hypertension are frequent complications amongst pediatric liver transplant recipients. Using Cyctatin C instead of Cr based formula for GFR estimation, and blood pressure monitoring by ABPM and regular screening of renal function are essential measures for recognition and treatment of renal dysfunction in these patients.

Schimke immuno-osseous dysplasia (SIOD) is a rare inherited disease characterized by steroid resistant nephrotic syndrome, spondyloepiphyseal dysplasia, and T-cell immunodeficiency. Focal segmental glomerulosclerosis (FSGS) is the most frequent renal pathological finding associated with proteinuria in SIOD. In this case report, we describe a 4.5-year-old boy who presented with nephrotic syndrome and ventricular septal defect followed by tremor in the limbs after-cerebral infarction. It is emphasized that SIOD should be considered in children with wide range of presentation, from growth retardation, steroid resistant nephrotic syndrome, and bone, cardiac, and neurological abnormalities in the late childhood or even adolescence., Competing Interests: There are no conflicts of interest.

Schimke Immuno-osseous Dysplasia (SIOD) is a rare autosomal recessive disease caused by a biallelic mutation in SMARCAL1 gene. Typical findings in SIOD include spondylo-epiphyseal dysplasia, steroid resistance nephrotic syndrome, progressive renal failure, T-cell immunodeficiency, bone marrow failure, and cerebral infarction. In this case report, we describe a 9-yr-old girl who presented with failure to thrive in infancy. Nephrotic syndrome was diagnosed at the age of four years. She had three episodes of admission with cerebral stroke due to moyamoya syndrome. In the last admission at Namazi Hospital, Shiraz, southern Iran, in October 2016, she had new cerebral ischemia, developed seizure, and finally died., Competing Interests: The authors declare that there is no conflict of interest.

Introduction: Changes in cardiac structure and function are common among patients with end-stage renal disease. The aim of this study was to evaluate the effect of hemodialysis on global and segmental cardiac function in children with end-stage renal disease., Materials and Methods: Fifty-two children with ESRD who were on long-term hemodialysis at Nemazee Hospital, Shiraz, were enrolled. They underwent echocardiography (M-mode, 2-dimensional, Doppler, and speckle echocardiography) 30 minutes prior to and after hemodialysis, and the values were compared., Results: The mean age of the patients was 13.00 ± 3.53 years. There was a significant reduction in left ventricular systolic and diastolic dimensions and volume (P < .001) and the E:A ratio after dialysis (P = .03). Global and longitudinal strain and strain rate showed no significant changes before and after hemodialysis., Conclusions: Hemodialysis with volume reduction decreases left ventricular volume but not regional strain and strain rate significantly in children. This study showed preload independency of speckle echocardiography in children.

Nephropathic cystinosis is an inherited lysosomal transport disorder caused by mutations in the CTNS gene that encodes for a lysosomal membrane transporter, cystinosin. Dysfunction in this protein leads to cystine accumulation in the cells of different organs. The accumulation of cystine in the kidneys becomes apparent with renal tubular Fanconi syndrome between 6 and 12 months of age and leads to renal failure in the first decade of life. The aim of this study was to analyze the CTNS mutations in 20 Iranian patients, from 20 unrelated families, all of whom were afflicted with infantile nephropathic cystinosis. In these patients, seven different mutant alleles were found, including two new mutations, c.517T>C; p.Y173H and c.492&#95;515del, that have not been previously reported. In addition, we observed that c.681G>A, the common Middle Eastern mutation, was the most common mutation in our patients. Moreover, a new minisatellite or variable number of tandem repeat marker (KX499495) was identified at the CTNS gene. Seven different alleles were found for this marker, and its allele frequency and heterozygosity degree were calculated in cystinosis patients and healthy individuals., Competing Interests: The authors declare no conflict of interest.

Urinary tract infection (UTI) is one of the most common bacterial infections in pediatrics. Delay in diagnosis and treatment can cause significant morbidity. The physicians knowledge regarding the symptoms, microorganisms that caused UTI, and effective antibiotics in a geographical area can help them to select the appropriate antibiotics. This study was performed to determine the prevalence of bacteria that cause UTI and their susceptibility to common antibiotics as well as the common symptoms and associated factors in children of Shiraz, Southern Iran.This cross sectional study was performed among 202 children with UTI, aged 2 months to 18 years old, between August and November 2014 in pediatric medical centers of Shiraz University of Medical Sciences. Urine samples were collected using urinary catheter or suprapubic in children < 2 years and mid-stream in children over 2 years, respectively. The type of micro-organisms causing UTI was determined and evaluation of antibiotic susceptibility for each organism was assayed by the Kirby Bauer method using antibiogram test. Patient's information was collected through checking the medical documents and interview with parents.Our results showed that the frequency of UTI was significantly higher in girls (70.3%) than in boys. The most commonly discovered pathogens were Escherichia coli (E coli) (51.5%), followed by Klebsiella spp. (16.8%), and Enterococcus spp. (9.9%). Overall susceptibility test showed the highest resistance to ampicillin (81.2%) and cotrimoxazole (79.2%), and the highest sensitivity to imipenem (90.1%) and Gentamicin (65.3%). Gram negative and positive bacteria showed the highest antibiotic resistance to amoxicillin (83.8%) and clindamycin (100%), respectively. In addition, production of extended spectrum beta lactamase (ESBL) was 69.2% and 30.8% in E coli and Kelebsiella respectively.The efficacy of third generation of the cephalosporins was reduced because of the high rate of production of ESBL and drug resistance. These results inform the physician as to which antibiotics are appropriate to prescribe for the patient, as well as urine culture reports and following the patient's clinical response so that high antimicrobial resistance is not developed at the community level.

Recurrence of original disease is a common threat in the field of transplantation. Recurrence of nephrotic syndrome is not common in children with congenital nephrotic syndrome (CNS). We report a case of a female child with CNS who presented with nephrotic state at first month of age and became dialysis dependent at 17 months of age. After seven months of continuous ambulatory peritoneal dialysis, she received a kidney from a deceased donor. Eight months after transplantation, she presented with a full-blown feature of nephrotic syndrome. She responded well to rituximab.

Background: Protein-energy malnutrition (PEM) is a common complication in pediatric patients with chronic kidney disease (CKD). Components incorporated in the regulation of appetite and body composition appear to be of the focus in renal insufficiency and may influence the CKD-associated PEM. The purpose of this study was to investigate plasma levels of appetite-regulating hormones and their correlation with the body composition variables in a pediatric in predialysis stage of CKD., Methods: Thirty children with CKD in predialysis stage were selected and compared with 30 healthy sex- and age-matched controls. Blood samples were collected in fasting. Serum total ghrelin, leptin, and obestatin levels were measured using enzyme immunometric assay methods. Anthropometric parameters measurement and body composition analysis were done using the bioelectric impedance analysis (BIA) method., Results: Patients showed insignificant elevated total ghrelin (105.40±30.83 ng/l), leptin (5.32±1.17 ng/ml) and obestatin (5.07±1.09 ng/ml) levels in comparison with healthy participants. By using BIA, patients had significantly different Dry Lean Weight (P=0.048), Extra Cellular Water (P=0.045), Body Cell Mass (BCM) (P=0.021), Basal Metabolic Rate (P=0.033) and Body Mass Index (P=0.029) compared with controls. Furthermore, the total body water was slightly and the ECW was significantly higher in CKD participants. There were significant negative correlation between obestatin and BCM (r=-0.40, P=0.03) and fat free mass index (FFMI) (r=-0.40, P=0.029) in patients., Conclusion: It seems that our results are insufficient to clarify the role of appetite-regulating hormones in PEM in CKD patients. It is apparent that there are still many unknown parameters related to both appetite regulating and CKD-associated PEM.

Ictal asystole is a rare, probably underestimated manifestation of epileptic seizures whose pathophysiology is still debated. This report describes two patients who had cardiac asystole at the end of their seizure. The first patient was a 13-year-old boy with complex partial seizures.. His MRI showed symmetrical signal abnormality in the bilateral parietooccipital lobe accompanied by mild gliosis and volume loss. During a 3-day long-term video-EEG monitoring, he had cardiac arrest at the end of one of his seizures that was secondarily generalized. The second one was a 42-year-old veteran with penetrating head trauma in the left frontal lobe due to shell injury. During long-term video-EEG monitoring, he had one generalized tonic-clonic seizure accompanied by bradycardia and cardiac asystole. Asystoles could have a role in the incidence of sudden unexpected death in epilepsy (SUDEP), meaning that the presence of ictal bradycardia is a risk factor for SUDEP. In cases of epileptic cardiac dysrhythmia, prolonged simultaneous EEG/ECG monitoring may be required. Cardiological investigation should be included in epilepsy management.

Pediatric kidney transplant recipients are at increased risk of CVD. Exercise test is a good method to evaluate exercise capacity, cardiorespiratory fitness, and risk of potential CVDs. The aim of this study was to assess the exercise capacity in this population and determine its relationship with their cardiac function using conventional and tissue Doppler echocardiography. Exercise test, conventional and tissue Doppler echocardiography were performed on 44 kidney transplant children (age ranging 11-20, 59% male) with acceptable renal function, and the results were compared with their normal healthy counterparts. Our transplant patients achieved significantly lower maximal heart rate, maximal heart rate ratio, total energy expenditure during the exercise, and maximal O2 consumption (Max VO2 ) than the normal group (p < 0.05). No correlation was found between hemoglobin (Hb) level, dialysis duration, kidney function, and the exercise test parameters. Kidney transplant patients had preserved systolic despite diminished diastolic cardiac function compared to the normal children. Our pediatric renal transplant recipients had severely impaired diastolic dysfunction and significantly reduced MaxVO2 compared with their healthy counterparts. No correlation was found between MaxVO2 and measured indices of systolic and diastolic cardiac function., (© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Objective: Cyanotic congenital heart diseases (CCHDs) are a series of cardiac anomalies that have long been recognized as a potential cause of nephropathy. There have been few reports on renal impairment in patients with CCHD before and after corrective cardiac surgery. The aim of this study was to evaluate the prevalence of renal dysfunction before and after cardiac surgery and the impact of some risk factors on final renal outcome., Methods: Thirty children with CCHD who had done corrective cardiac surgery in the previous 6 months were enrolled in this study. All data prior to surgery were collected from the charts. Post-operation data including blood and spot urine samples were taken simultaneously for CBC, Cr, and uric acid and 24 hour urine was collected for microalbumin and Cr during the follow up visits. Pre- and post-operation parameters were compared to study the impact of cardiac surgery on renal function. Findings : Pre- and post-operative GFRs were not significantly different. Final GFR was significantly and inversely associated with pre- and post-operation age (P=0.008 r=-0.48, P=0.03 r=-0.38). Three (10%) patients had microalbuminuria. The prevalence of microalbuminuria in children older than 10 years was 30%. There was no link between microalbuminuria and age, GFR, and hematocrit (P=0.1, P=0.3, P=0.3, respectively). Patients with preoperation hematocrit >45 had a significantly lower final GFR compared to children with HCT <45 (83.7±6.5 vs 111.10.2, P=0.001). The mean uric acid fraction (FEua) excretion was 8.21±4.75. Pre-operative HCT was inversely associated to FEua (P=0.01, r=-0.44). There was no relationship between FEua and age, serum uric acid, and GFR (P=0.7, P=0.4, P=0.2)., Conclusion: Children with CCHD are at increased risk of renal injury which is related more to the duration of cyanosis and higher degree of hematocrit level. To lower the risk, corrective cardiac surgery is recommended to be done as soon as possible to improve renal function and stop more renal impairment.

Introduction: Mutations in podocin (NPHS2) gene have the key role in the pathogenesis of steroid-resistant nephrotic syndrome (SRNS) in children, but data is scarce regarding their prevalence and natural course among different all ethnic groups. This study was aimed to demonstrate the spectrum of NPHS2 mutations in children with SRNS and to compare the clinical course of disease in patients with and without mutation., Materials and Methods: All 8 exons of NPHS2 were sequenced in 99 children, including 49 with SRNS and 50 with steroid-sensitive nephrotic syndrome (control group) by DNA sequencing., Results: The prevalence rates of NPHS2 gene mutation among children with SRNS and SSNS were 31% and 4%, respectively. The prevalence rates of mutation among familial and sporadic forms were 57% and 26%, respectively. Thirty-three percent of the children experienced recurrence of primary disease after kidney transplantation, none of whom had a mutation. The clinical response to treatment was poorer in children with mutation in comparison with patients without mutation (12% versus 32%, respectively; odds ratio, 3.29, 95% confidence interval, 0.40 to 25.64). Patients with and without mutation could not be differentiated by demographic and histological features, glomerular filtration rate at onset, hypertension, progression to end-stage renal disease, and proteinuria., Conclusions: Mutations of NPHS2 gene are frequent among Iranian children with SRNS. Regarding similar clinical features in patients with and without mutation and poor response to pharmacotherapy in patients with mutation, a molecular approach might be necessary for different treatment plans and prediction of prognosis.

Introduction: There is still controversy regarding the possible role of chronic functional constipation in disorders of the urinary tract, specifically urinary tract infection (UTI) and enuresis. The aim of this study was to investigate the frequency of (UTI) and enuresis in children with chronic functional constipation., Materials and Methods: We included 120 children (60.8% girls) with chronic functional constipation based on the Rome III criteria. Detailed history of UTI and enuresis or symptoms pointing to these diagnoses was obtained. Urinalysis, urine culture, and abdominal ultrasonography were performed for all of the participants., Results: The mean age of the patients was 7.4 ± 3.2 years. Seventy-five percent of the patients had constipation for more than 1 year. The most common urinary symptoms were dysuria (16.7%), urinary frequency (12.5%), and dribbling (4.2%). The frequencies of nocturnal and daytime enuresis were 22.5% and 3.3%, respectively. Pyuria was seen in 10 girls (8.3%). Overall, 7 patients (5.8 %) had a positive urine culture, of whom all were girls and 6 had pyuria. Urinary tract ultrasonography was normal in these patients., Conclusions: Urinary symptoms, especially nocturnal enuresis, were found in a significant number of children who had chronic functional constipation, but UTI was not so common in the present study. Therefore, we suggest that nocturnal enuresis be considered in children with chronic functional constipation, but screening for UTI is not recommended in these patients.

Introduction: Obesity is a growing problem worldwide and is likely a major cause of the increased prevalence of high blood pressure in children. The aim of this study was to investigate the frequency of pediatric obesity and its association with hypertension in a sample of children and adolescents in Fars province (south of Iran)., Materials and Methods: This cross-sectional study was carried out in Shiraz during a period from 2010 to 2011. A total of 2000 healthy students aged 11 to 17 years were included. Data on weight, height, systolic and diastolic blood pressure (measured 3 times with 5-minute intervals), and parental history of hypertension and educational level were obtained. The 95th percentile body mass index for age and sex was considered obesity., Results: Overall, 7% and 11.8% of the students were obese and hypertensive, respectively. Blood pressure was associated with body mass index. Maternal education level was not associated with hypertension in the child., Conclusions: In this study, obesity was an important risk factor for hypertension. Our data showed that prevalence of obesity has not been changed in the recent 5 years in Iran, but that of hypertension has risen significantly. The high prevalence of hypertension in overweight and obese children emphasizes the need for prevention and control of childhood obesity and hypertension in early stages.