Etiology and Antimicrobial Susceptibility Pattern of Urinary Tract Infection in the First Year after Pediatric Renal Transplantation: A Preliminary Study.

Urinary tract infection (UTI) is common after pediatric renal transplantation, and the emergence of multidrug-resistant (MDR) bacteria causing UTI is a therapeutic challenge in this regard. The main purpose of this study was to determine the UTI frequency, its etiologic agents, and the antibiotic susceptibility pattern in the first year following renal transplantation in Iranian pediatric recipients. In a retrospective cohort study, all of the 81 children who had undergone renal transplantation in Hazrat Rasoul Akram Hospital between 2012 and 2017 were enrolled.

Genetic diagnosis of Bartter syndrome in Iranian patients and detection of a novel homozygous mutation.

An Iranian girl with clinical symptoms of Bartter syndrome like hypokalemia, polyuria, polydipsia, hyponatremia, and hypochloremic alkalosis was referred to us in whom the gene was genetically evaluated using Sanger sequencing. A homozygous pathogenic variant of c.1332_1335delCTCT was detected in this patient.

Gastrointestinal Manifestations of Adult Cystinosis in Iran: A Descriptive Study.

Cystinosis is a multisystemic disease caused by the accumulation of cystine crystals in the kidney and many other organs. This disease most often involves children. Recent developments in the treatment procedures have improved the chance of patients surviving as long as puberty.

Frequency of neurological involvement in patients with/without diarrhea hemolytic uremic syndrome: A Systematic review and meta-analysis.

Diarrhea-associated-hemolytic-uremic-syndrome (D+HUS) is a common from of HUS. Central-nervous-system (CNS) involvement is one of the most common extrarenal organ involvements in children with D+HUS. This systematic review and meta-analysis aim to recognize the frequency of neurological complications in pts with HUS.

Long-Term Outcome of Kidney Transplant Among Iranian Children: A Systematic Review and Meta-Analysis.

Objectives: End-stage kidney disease has dramatic health effects and life consequences in children. Presently, kidney transplant has been globally accepted as a treatment of choice for end-stage kidney disease in both children and adults, leading to better quality of life and longer patient survival. Because of lack of comprehensive information on the outcome of kidney transplant among children in Iran, we aimed to present a proper vision of pediatric kidney transplant in Iran by systematically reviewing the current literature.

Exome sequencing utility in defining the genetic landscape of hearing loss and novel-gene discovery in Iran.

Hearing loss (HL) is one of the most common sensory defects affecting more than 466 million individuals worldwide. It is clinically and genetically heterogeneous with over 120 genes causing non-syndromic HL identified to date. Here, we performed exome sequencing (ES) on a cohort of Iranian families with no disease-causing variants in known deafness-associated genes after screening with a targeted gene panel.

Determination of the value of albumin, anti-thrombin III, fibrinogen and D-dimer factors in the diagnosis of asymptomatic pulmonary embolism in patients with nephrotic syndrome.

Background: Thromboembolism is one of the most important and dangerous complications of nephrotic syndrome. This study aimed to determine the value of albumin, anti-thrombin III, fibrinogen and D-dimer factors in the prediction of asymptomatic pulmonary embolism in patients with nephrotic syndrome in non-remission period.

Methods: Plasma levels of albumin, anti-thrombin III, fibrinogen and D-dimer were assessed in 30 nephrotic children in non-remission period (including new case-patient or relapse period), and the results were compared with chest X-ray and lung perfusion scintigraphy (Q scan).

Whole exome sequencing revealed a novel homozygous variant in the DGKE catalytic domain: a case report of familial hemolytic uremic syndrome.

Background: Atypical hemolytic uremic syndrome (aHUS) is a rare disease characterized by microangiopathic hemolytic anemia caused by small vessel thrombosis, thrombocytopenia, and renal failure. The common cause of aHUS is a dysregulation in the alternative complement pathway. Mutations in none complement genes such as diacylglycerol kinase epsilon (DGKE) can also result in this syndrome.

Whole Exome Sequencing Reveals a XPNPEP3 Novel Mutation Causing Nephronophthisis in a Pediatric Patient.

Background: Nephronophthisis (NPHP) is a progressive tubulointestinal kidney condition that demonstrates an AR inheritance pattern. Up to now, more than 20 various genes have been detected for NPHP, with NPHP1 as the first one detected. X-prolyl aminopeptidase 3 (XPNPEP3) mutation is related to NPHP-like 1 nephropathy and late onset NPHP.

Changes in body mass index after pediatric renal transplantation.

Significant weight gain following renal transplantation is common in adult and pediatric recipients and mostly depends on receiving higher doses of steroids, changes in mood and feelings, as well as their level of physical activities. This study was performed to evaluate body weight and body mass index (BMI) before and after kidney transplantation in children and adolescents. In this cross-sectional study, 71 pediatric renal transplant recipients (42 boys and 29 girls) were included.

Chronic Neurological Complications in Hemolytic Uremic Syndrome in Children.

Introduction: Central nervous system (CNS) involvement is the most common extrarenal involvement in hemolytic uremic syndrome (HUS). There are limited reports on clinical cause of chronic neurologic problems in HUS. We evaluated residual neurologic involvement in children with HUS.

Efficacy and Safety of Rituximab in Children With Steroid- and Cyclosporine-resistant and Steroid- and Cyclosporine-dependent Nephrotic Syndrome.

Introduction: There is evidence of the effectiveness of rituximab in treatment of nephrotic syndrome in children. The present study aimed to assess safety and the therapeutic effectiveness of rituximab in steroid- and cyclosporine-resistant pediatric nephrotic syndrome.

Materials And Methods: Forty-three children with steroid- and cyclosporine-resistant or steroid- and cyclosporine-dependent noncongenital nephrotic syndrome were included in the study to receive intravenous rituximab, 375 mg/m2/wk, for 4 weeks.

Comparison of tuberculin skin test and interferon gamma release assay for diagnosis of latent tuberculosis infection in pediatric candidates of renal transplantation.

IGRA has been approved as an alternative in vitro test to diagnose Mycobacterium TB infection. This study aimed to assess the diagnostic value of TST in comparison with QFT assay to detect LTBI among Iranian children candidate for renal transplantation. This cross-sectional study was performed on 31 children who were candidate for renal transplantation admitted to Ali Asghar Children's Hospital and Rasoul Akram Hospital, Tehran, Iran, from 2013 to 2014.

Worldwide view of nephropathic cystinosis: results from a survey from 30 countries.

Background: Nephropathic cystinosis is a rare inherited metabolic disorder leading to progressive renal failure and extra-renal comorbidity. The prognosis relies on early adherence to cysteamine treatment and symptomatic therapies. Developing nations [DiN] experience many challenges for management of cystinosis.

The prevalence of hypertension in children with renal scars.

Background: Hypertension (HTN) is a late outcome of congenital or acquired renal scar. We used ambulatory blood pressure to assess the early blood pressure abnormalities in children with history of urinary tract infection with various degrees of renal scars.

Methods: Between 2009 and 2011, 60 (45 females, 15 males) children aged 5-15 years and height equal or more than 120 cm with previous history of febrile urinary tract infection were entered into the study.

Characterising the spectrum of autosomal recessive hereditary hearing loss in Iran.

Background: Countries with culturally accepted consanguinity provide a unique resource for the study of rare recessively inherited genetic diseases. Although hereditary hearing loss (HHL) is not uncommon, it is genetically heterogeneous, with over 85 genes causally implicated in non-syndromic hearing loss (NSHL). This heterogeneity makes many gene-specific types of NSHL exceedingly rare.

Endoscopic correction of primary VUR by using polyacrylate polyalcohol copolymer (Vantris) in young girls: 2-year follow-up.

Objective: Our aim was to evaluate the efficacy and safety of single endoscopic injection of Vantris in young girls affected by primary vesicoureteral reflux (VUR) at more than 2 years of prospective follow-up.

Material And Methods: Over the last 4 years, 73 girls with primary VUR of grades (G) I-IV underwent a single endoscopic injection of Vantris. The mean age was 8.

Serum procalcitonin level for prediction of high-grade vesicoureteral reflux in urinary tract infection.

Introduction: Procalcitonin is a reliable and specific marker of bacterial infections such as urinary tract infection. Some authors suggest measurement of serum procalcitonin as a predictor of vesicoureteral reflux (VUR). We investigated this association in children admitted because of acute pyelonephritis.

Carotid artery function in children with idiopathic nephrotic syndrome.

Background: Nephrotic patients are prone to atherosclerosis in consequence of frequent exposures to hyperlipidemia, hypertension, and immunosuppressive drugs.

Objectives: We studied the carotid parameters as early indicators of atherosclerosis in children with nephrotic syndrome.

Methods: Between 2008 and 2011, 51 children with history of nephrotic syndrome enrolled in the study.

Pretransplant Epstein-Barr virus serostatus and incidence of posttransplant lymphoproliferative disorder in pediatric renal transplants.

Objectives: Pediatric renal transplant recipients are at increased risk of Epstein-Barr virus infection which may be due to the high percentage of Epstein-Barr virus seronegative recipients at the time of transplant in the pediatric age group. We aimed to assess the Epstein-Barr virus serostatus of recipients and donors and the incidence of posttransplant lymphoproliferative disorder in pediatric renal transplant at the Labafinejad Hospital in Tehran, Iran.

Materials And Methods: We reviewed the clinical records of 183 children who had a renal transplant at the Labafinejad Hospital in Tehran, Iran, between 2003 and 2011.

Rituximab in the treatment of nephrotic syndrome: a systematic review.

Recently, the role of B cells in the pathogenesis of nephrotic syndrome is explained by some researchers. It has also been shown that the anti-CD20 antibody, rituximab, may be an option in the treatment of these patients. In this systematic review, we performed extensive search and identified studies on rituximab use in children with nephrotic syndrome.

Treatment update of sensitized pediatric kidney transplant recipients: a review.

Sensitization of recipients is an increasing problem in children. Some case series in children exist comparing the diverse desensitizing protocols. These protocols include intravenous immunoglobulin, cytomegalovirus immune globulin, plasmapheresis, and some adjunctive therapies such as rituximab.

Partial trisomy 7q and monosomy 13q in a child with disorder of sex development: phenotypic and genotypic findings.

Terminal 7q duplication and terminal 13q deletion are two conditions with variable phenotypes including microcephaly, thumb a-/hypoplasia, cortical dysplasia, microphtalmia, intellectual disability and dysmorphic features. We describe a boy born to a mother with a reciprocal t (7;13) who combines both a terminal 7q33-qter duplication and terminal 13q33-qter deletion through the inheritance of a derivative chromosome 13 (der (13)). The patient presented with developmental delay, facial and non-facial dysmorphic features, hypertonia, genital abnormality and skeletal malformation but no thumb a-/hypoplasia or microphtalmia.