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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. MATERIALS AND METHODS Major databases were searched, including Medline, Web of Knowledge, Google Scholar, Scopus, Cochrane, and the Iranian Scientific Information Database for all eligible studies in accordance with specific keywords. The inclusion criteria for the retrieved studies were determination of graft survival, patient survival, and reasons for graft failure. The exclusion criteria were as follows: (1) a lack of clear results; (2) non-English or non-Persian language format; (3) lack of access to the full-text manuscripts; and (4) case reports, case series, and review papers. A total of 115 studies were initially assessed based on the keywords; of these, 8 met inclusion criteria and were considered for final analysis; these were published between 2005 and 2017. RESULTS According to our results, 1-year graft survival rates were overall 89.7%, and 5-year graft survival rates were 65.4%. The 1-year patient survival rates were estimated to be 97.1%, and 5-year patient survival rates were estimated to be 89.8%. Acute rejection, dialysis status before transplant, and inappropriate immunosuppression were the main risk factors. CONCLUSIONS Our systematic review and meta-analysis indicated a high success rate of childhood kidney transplant in Iran according to long-term graft and patient survival rates.

Introduction: Since mid-April 2020, infection with coronavirus disease of 2019 (COVID-19) has affected more than 2 million people worldwide. Little is known about the effects of COVID-19 infection on kidney transplant patients treated with immunosuppressive (IS) drugs. Case Presentation: Here, we report three Iranian pediatric kidney transplantation cases who acquired COVID-19 infection and were admitted to Hazrat Ali Asghar Hospital in Tehran, Iran, in March-April 2020 (first wave). They were treated with prednisolone, cy-closporine, and cellcept. All of them had gastrointestinal symptoms, and one patient also had respiratory symptoms. In two patients who had only gastrointestinal symptoms, chest radiographies were considered normal. They did not need the intensive care unit (ICU), ventilator respiratory support, and dialysis due to a temporary increase in serum creatinine and no change in urine volume. Cellcept was stopped and other medications continued. The patient, who had pulmonary and gastrointestinal symptoms, expressed more severe manifestations that led to ICU admission and dialysis. Prednisolone was increased to a stress dose and the other drugs were discontinued. Conclusions: Pulmonary manifestations significantly worsen the prognosis compared to gastrointestinal manifestations in pediatric kidney transplants with COVID-19. © 2021, Author(s).

Background and Objectives: Serum amylase increases and its excretion fraction decreases in patients with renal failure. Excretion fraction can be used as a diagnostic way in individuals with acute renal transplant rejection. In this study, the association between amylase excretion fraction and acute renal transplant rejection, was investigated in pediatrics. Methods: in this case - control study, serum and urine amylase and serum and urine creatinine, were measured in children without (30 cases) and with (30 cases) acute renal transplant rejection, and their amylase excretion fraction was determined. Sonography of kidney and urinary tract, was used to rule out the obstruction due to surgical manipulation. To rule out cyclosporine intoxication and urinary tract infection (differential diagnostic factors of acute renal transplant rejection), serum cyclosporine and urine CRP were assessed, respectively. Data analysis was performed using t-test and logistic regression test. Results: In children with acute renal transplant rejection, variables of urine creatinine (20.1±5.5 vs 15.9±4.7, p=0.002), urine amylase (744.9±376.6 vs 584.8±218.3, p=0.049), and amylase fraction excretion (238.7±118.4 vs 96.112±2.5, p

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. We identified likely causal variants in 20 out of 71 families screened. Fifteen families segregated variants in known deafness-associated genes. Eight families segregated variants in novel candidate genes for HL: DBH, TOP3A, COX18, USP31, TCF19, SCP2, TENM1, and CARMIL1. In the three of these families, intrafamilial locus heterogeneity was observed with variants in both known and novel candidate genes. In aggregate, we were able to identify the underlying genetic cause of HL in nearly 30% of our study cohort using ES. This study corroborates the observation that high-throughput DNA sequencing in populations with high rates of consanguineous marriages represents a more appropriate strategy to elucidate the genetic etiology of heterogeneous conditions such as HL.

Background: 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. Methods: Databases of PubMed, Embase, and Web of Science were searched systematically to find the papers on neurological involvement in HUS pts. Two researchers independently assessed the papers’ quality and extracted data. CMA v. 2.2.064. was used for data analysis. Heterogeneity was evaluated using the I-squared (I2) test, and a fixed/random-effects model was used when appropriate. Results: In this review, 21 studies including 2,189 participants with a median age between 1.3-40-year-old, entered the meta-analysis. The meta-analysis in D+HUS patients indicated 27.0% with neurological complications (95% CI, 22.0%-32.6%), 25.5% of symptoms weren’t categorized (95% CI, 15.9%-38.3%), 20.8% of them developed the seizures (95% CI, 2.3%-74.4%). In D-HUS pts, 20.8% of them were presented neurological symptoms (95% CI, 17.9%-24.0%), of which 29.0% weren’t categorized (95% CI, 19.2%-41.2%), 17.5% of pts got into coma (95% CI, 9.6%-29.7%), 5.6 % showed hemiparesis (95% CI, 2.8%-10.9%), 17.2% experienced lethargy (95% CI, 5.2%-44.1%), 30.5% developed the seizures (95% CI, 18.2%-46.2%), 7.4% manifested speech abnormalities (95% CI, 0.2%-7.22%), 6.4% of D-HUS pts presented visual-disturbances (95% CI, 3.4%-11.6%). Conclusion: This systematic review and meta-analysis indicated more than one-fourth of both D+HUS and D-HUS patients were presented with neurological symptoms, and the most prevalent symptoms were seizures, which can lead to an epilepsy sequel.

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. Case presentation Here, we report on a 19-year-old female with the clinical diagnosis of aHUS, who has unaffected consanguineous parents and an older sibling who was deceased from aHUS when she was seven months old. We performed whole exome sequencing (WES) followed by evaluation of detected variants for functional significance, using several online prediction tools. Next, in order to confirm the detected pathogenic variant in proband and segregation analysis in her family, Sanger sequencing was done. The novel variant was analyzed in terms of its impact on the protein 3-dimensional structure by computational structural modeling. The results revealed that the proband carried a novel homozygous missense variant in DGKE located in exon 6 of the gene (NM_003647.3, c.942C > G [p.Asn314Lys]), and in silico analysis anticipated it as damaging. Protein computational study confirmed the influence of potential pathogenic variant on structural stability and protein function. Conclusion We suggest that some variations in the catalytic domain of DGKE like p.Asn314Lys which can cause alterations in secondary and 3-D structure of protein, might lead to aHUS.

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. Methods The proband (index patient) had polyuria, polydipsia and chronic kidney disease and was clinically suspected of NPHP. After the collection of blood sample from proband and her parents, whole exome sequencing (WES) was performed to identify the possible variants in the proband from a consanguineous marriage. The functional importance of variants was estimated by bioinformatic analysis. In the affected proband and her parents, Sanger sequencing was conducted for variants’ confirmation and segregation analysis. Results Clinical and paraclinical investigations of the patient was not informative. Using WES, we could detect a novel homozygous frameshift mutation in XPNPEP3 (NM_022098.2: c.719_720insA; p. Q241Tfs*13), and by Sanger sequencing, we demonstrated an insertion in XPNPEP3. Conclusion The homozygous genotype of the novel p.Q241Tfs*31 variant in XPNPEP3 may cause NPHP in the early childhood age.

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.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).The mean age of patients was 6.22 ± 3.5 years (range 2-12 years). Of patients, 23.3% had abnormal findings in perfusion scan suggestive of pulmonary emboli despite absence of any respiratory manifestations. Median plasma albumin and anti-thrombin III levels in patients with asymptomatic pulmonary embolism were lower than in patients without pulmonary embolism. Also, median fibrinogen and D-dimer levels in patients with asymptomatic pulmonary embolism were higher than in patients without pulmonary embolism, with no statistically significant differences between sex, age, hemoglobin and hematocrit of patients and lung perfusion scan results.Patients with abnormal blood levels of albumin (3.5 g/dl), anti-thrombin III (80 ml/dl), fibrinogen (400 ml/dl) and D-dimer (0.5 μg/dl) underwent CXR/Q scan and were treated with heparin if there was pulmonary embolism.

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. World Health Organization criteria were used for comparing Z-score BMI for age in our cases. Overweight was defined as Z-score BMI >+1 SD (standard deviation) and obesity as >+2 SD. At the time of transplantation, the mean age was 10.8 ± 3 years (5-16 years) and based on BMIZ-score, the patients were found to be thin (BMIZs +1 SD to +2 SD) in 9.9%, and obese (BMIZs >+2 SD) in 5.6%.The mean follow-up duration after transplantation was 3.57 ± 1.68 years (1-7 years) and at the time of reevaluation after transplant, their mean age was 14.4 years (6-18 years). The mean BMI was 22 ± 5.3 kg/m2, and for BMI grouping, the patients were thin in 7%, normal in 54.9%, overweight in 21.1%, and obese in 17%. Pretransplant thinness (BMIZs +1 SD after transplant. Chronic continuous decrease of glomerular filtration rate (CCD/GFR) was found in 27 cases (38%); 74.1% were male (P = 0.045), hypertriglyceridemia was found in 74.1% (P = 0.023%), hypercholesterolemia in 63% (P = 0.032),and obesity in 18.5% (p = 0.5). The incidence of obesity has tripled after kidney transplantation. It was not a risk factor for graft or patient survival in our experience, whereas pretransplant obesity had some effects on long-term graft outcome.

Background: Nephrotic syndrome (NS) is a common renal disease in the pediatric population, which can be complicated with venous thromboembolic events. Objectives: In the present study, the researchers evaluated the risk factors of venous thrombotic events in children with nephrotic syndrome. Methods: In this descriptive cross sectional study, the researchers evaluated 43 cases of NS including 35 uncomplicated and eight complicated patients with venous thrombotic events, who were admitted to the nephrology ward of Ali-Asghar Children Hospital from 2011 to 2015. Two groups were matched for age onset of NS. Data were recorded on age, gender, body mass index (BMI), histopathologic varieties of NS, and serum albumin level. Results: The mean age of cases with venous thrombotic events was 7.31 ± 4.1 years. There were six females. Of eight cases with venous thrombotic events, five cases suffered of pulmonary thromboembolism (PTE) and five cases of deep vein thrombosis (DVT). The mean serum albumin level in the thrombotic group was 1.87 ± 0.4 g/dL. The histopathologic results showed two cases of membranous proliferative glomerulonephritis (MPGN), three cases of minimal change disease (MCD) and one case of focal segmental glomerulosclerosis (FSGS). The researchers found significant differences between mean serum albumin level and histopathologic results in the case and control groups. Conclusions: Venous thrombosis and pulmonary emboli are important complications in pediatric NS. The risk of VTE increases with lower serum albumin level. The risk of VTE increases with lower serum albumin level. The researchers suggest the use of anti-thrombotic agents as prophylaxis in nephrotic patients with serum albumin level of less than 2 g/dL. Copyright © 2019, Journal of Comprehensive Pediatrics.

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.This cross-sectional study was conducted on 58 patients with a diagnosis of HUS referred to 2 tertiary pediatric centers. Neurological examinations was performed on all of the patients and they were followed up between 2001 and 2015. Data including demographic variables, type of HUS, neurological symptoms, and other complications were recorded. Neurological involvements that occurred after 6 months from the acute phase of HUS were considered as chronic neurological involvement. Among 58 patients who were included in the study, 31 (53.4%) had neurological manifestations (31 with acute and 19 with chronic complications). There was no significant difference in acute neurological manifestations between typical and atypical HUS, while chronic neurological manifestations were more frequents in patients with atypical HUS (P = .05). The most common presentations were seizure and decreased level of consciousness. Chronic neurologic problems were found in follow-up visits of 11 patients with acute and 8 without acute involvement. Hypertension was associated with chronic manifestations (P = .01).According to our results, residual neurological problems were not infrequent in HUS and they were more related with atypical form of disease. Evidence of hypertension is a significant variable for persistence of neurologic problems.

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. TST and QFT were performed for all patients. QFT was negative in all patients, while TST was positive only in one case. Both tests results were negative in 30 patients, yielding an accuracy rate of 96.7% for TST to diagnose LTBI when compared to QFT. In conclusion, compared to QFT, TST is still a valuable diagnostic tool with high accuracy rate for diagnosis of LTBI in children candidates for renal transplantation and can still be used as an accurate test for screening Mycobacterium TB infection.

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. We sought to define the spectrum of autosomal recessive HHL in Iran by investigating both common and rarely diagnosed deafness-causing genes. Design Using a custom targeted genomic enrichment (TGE) panel, we simultaneously interrogated all known genetic causes of NSHL in a cohort of 302 GJB2 -negative Iranian families. Results We established a genetic diagnosis for 67% of probands and their families, with over half of all diagnoses attributable to variants in five genes: SLC26A4 , MYO15A , MYO7A , CDH23 and PCDH15 . As a reflection of the power of consanguinity mapping, 26 genes were identified as causative for NSHL in the Iranian population for the first time. In total, 179 deafness-causing variants were identified in 40 genes in 201 probands, including 110 novel single nucleotide or small insertion–deletion variants and three novel CNV. Several variants represent founder mutations. Conclusion This study attests to the power of TGE and massively parallel sequencing as a diagnostic tool for the evaluation of hearing loss in Iran, and expands on our understanding of the genetics of HHL in this country. Families negative for variants in the genes represented on this panel represent an excellent cohort for novel gene discovery.

Background There are studies indicating the relationship between interleukin 13 and pathogenesis of nephrotic syndrome. To find this relationship, we measured serum IL13 concentration in acute and remission phases in children with steroid sensitive nephrotic syndrome. Methods The serum of 15 patients was achieved for measurement of IL-13 in two phases: acute phase and remission phase. To this end, we used Biofarm ELIZA kites. Results The mean concentration of serum IL-13 was 430 ± 279 ng/mL (227 - 960 ng/mL) before starting steroid treatment. The mean concentration of serum IL-13 was 428 ± 259 ng/mL (215.6 - 960 ng/mL) after 4 weeks of steroid treatment. The difference between pre and post treatment of serum IL-13 levels was not significant (P = 0.91). Conclusions We showed serum IL13 does not significantly decrease after steroid treatment. Although the role of IL13 in increasing glomerular permeability has been shown in previous studies, the local IL13 may be more important than the systemic form. It also can be postulated that mediators other than IL13 also have primary roles in pathogenesis of proteinuria. However, we need more studies to determine the role of IL13 in the pathogenesis of nephrotic syndrome.

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. All children went on 24-hour ambulatory blood pressure monitoring (24-H ABPM). Updated classification of 24-H ABPM was used to interpret the results. RESULTS Masked hypertension was detected in 5% of cases, hypertension in 8.4%, and white coat hypertension in 11.7%. Pre-hypertension was seen in 23.3% of children. There was significant correlation between abnormal blood pressure and the severity of renal parenchymal scar (r=0.39, P value=0.004), vesicoureteral reflux (r= 0.34, P value=0.009), microalbuminuria (r= 0.39, P value=0.004), and carotid intima media thickness (r=0.41, P value=0.006). CONCLUSIONS This study revealed the utility of 24-H ABPM in early detection of hypertension and pre-hypertension in children with severe renal scars and past history of urinary tract infection.

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.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. The children were followed up for 2 years. Effectiveness was defined as remission of proteinuria in response to rituximab. Side effects of rituximab were monitored.Overall, 23 (57.1%) of the children had steroid- and cyclosporine-resistant nephrotic syndrome, of whom 8 (34.8%) revealed complete response and 3 (13%) revealed partial response. Seven children (16.7%) had late-resistant nephrotic syndrome, of whom 6 (85.7%) revealed complete response and none revealed partial response. Ten children (26.2%) had steroid- and cyclosporine-dependence all of whom revealed complete response to rituximab. Complete response rate was significantly higher in those with drug-dependent pattern than the other groups (P = .002). There was no association between response to rituximab and pathological basis of disease. Side effects were found in 4 patients as leukopenia in 2, alopecia in 1, and eosinophilia in 1.Rituximab is effective for children with nephrotic syndrome with high efficacy and well tolerability, especially in those with steroid- and cyclosporine-dependent nephrotic syndrome.

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. Knowing the exact breakpoints of his chromosomal aberrations using high resolution array CGH (aCGH) and comparison of his phenotypes with those of 24 and 59 previously published cases of 7q duplication and 13q deletion, respectively, allow us to further narrow the size of the proposed critical regions for microcephaly, thumb a-/hypoplasia and hypo/hypertonia on chromosome 13.

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. Desensitizing protocols have advantages and disadvantages. Clinical trials are required to determine suitable protocols for sensitized pediatric recipients. We performed a systematic review of these protocols in children. A massive search was done in PubMed, Embase, and the Cochrane library system. The results of these studies are compared.

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 aim of this study was to assess the management characteristics in DiN compared with developed nations [DeN]. Methods A questionnaire was sent between April 2010 and May 2011 to 87 members of the International Pediatric Nephrology Association, in 50 countries. Results A total of 213 patients were included from 41 centres in 30 nations (109 from 17 DiN and 104 from 13 DeN). 7% of DiN patients died at a median age of 5 years whereas no death was observed in DeN. DiN patients were older at the time of diagnosis. In DiN, leukocyte cystine measurement was only available in selected cases for diagnosis but never for continuous monitoring. More patients had reached end-stage renal disease in DiN (53.2 vs. 37.9%, p = 0.03), within a shorter time of evolution (8 vs. 10 yrs., p = 0.0008). The earlier the cysteamine treatment, the better the renal outcome, since the median renal survival increased up to 16.1 [12.5−/] yrs. in patients from DeN treated before the age of 2.5 years of age (p = 0.0001). However, the renal survival was not statistically different between DeN and DiN when patients initiated cysteamine after 2.5 years of age. The number of transplantations and the time from onset of ESRD to transplantation were not different in DeN and DiN. More patients were kept under maintenance dialysis in DiN (26% vs.19%, p = 0.02); 79% of patients from DiN vs. 45% in DeN underwent peritoneal dialysis. Conclusions Major discrepancies between DiN and DeN in the management of nephropathic cystinosis remain a current concern for many patients living in countries with limited financial resources. Electronic supplementary material The online version of this article (doi:10.1186/s12882-017-0633-3) contains supplementary material, which is available to authorized users.

Rahimzadeh N, Otukesh H, Hoseini R, Sorkhi H, Otukesh M, Hoseini S, Torkzaban M. Are serum and urine NGAL predictive of renal graft function in short term? Abstract: NGAL is a member of the lipocalin protein family that has diverse function but similar structure. The functions of NGAL are not clear, but it appears to be expressed in stress conditions and in tissues undergoing involution. Varied studies have shown increased levels of plasma or urinary NGAL in diverse renal damages. The aim of this study was the serial measurement of serum and urinary NGAL within the first week after renal transplantation in children to predict immediate and short-term graft function. A total of 27 patients were assessed. These patients were classified into those with rapid reduction in serum creatinine (more than 50% reduction in serum creatinine in the first day after transplantation) and patients with slow reduction in serum creatinine (

To evaluate the relationship between bladder volume wall index (BVWI) and the pattern of uroflowmetry in children with lower urinary tract malfunction.91 children aged 4-15 years with history of bladder dysfunction and 59 age/sex-matched healthy children with negative urine culture in previous month were enrolled. Uroflowmetry and kidney and bladder sonography were performed in all children. BVWI was measured by dividing maximum bladder volume index by mean bladder thickness. It was expressed as percentage by dividing calculated BVWI by expected BVWI, and values between 70% and 130% were presumed normal. Urodynamic study was done in symptomatic cases.The bladder was thick (70%) in 39 (28 cases, 11 controls) and thin (130%) in 35 (18 cases, 17 controls) (P0.05). Uroflowmetry was abnormal in 82 (61 cases, 21 controls) (P0.05). Severe sphincter dyssynergia was detected in 47% of cases compared with 20% of controls (P0.05).There was no relationship between BVWI and uroflowmetry in cases or in controls (P0.05). The median post-void residual urine was not statistically different between the groups (20 vs 12.3 ml) (P0.05). When both bladder sonography and uroflowmetry were abnormal, they had an association with abnormal urodynamics (P0.05).Among children with lower urinary tract dysfunction, the pattern of uroflowmetry could not be predicted from the BVWI, but in cases with combined abnormal bladder sonography and uroflowmetry results, there was a significant association with an abnormal urodynamic study.

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

Background: Myocardial dysfunction that complicates the initial stages of chronic kidney disease (CKD) has not been yet fully characterized in young patients. We aimed to assess the clinical usefulness of myocardial performance index obtained by pulsed-wave Doppler method (PWD-MPI) in predicting early disturbances of global left ventricular (LV) function in children with CKD stages 2–4. In addition, we evaluated the clinical utility of tissue Doppler imaging (TDI) as a tool for calculating MPI in comparison with the conventional method. Methods: Standard echocardiography was performed in 34 patients aged 3–18 years and for 35 age-matched healthy control subjects. PWD-MPI was calculated from Doppler spectra of mitral inflow and LV outflow. To obtain TDI-MPI, time intervals were measured from mitral annulus. Results: The mean values of both PWD-MPI and TDI-MPI of the patients were significantly different from those of the control subjects. Using receiver operating characteristics curve analysis, TDI-MPI yielded a better predictive discrimination for separating patients with versus those without myocardial dysfunction than PWD-MPI. Using a PWD-MPI >0.36 as the cutoff value, myocardial dysfunction was found with a sensitivity of 64.7% and specificity of 97%. The sensitivity and specificity of TDI-MPI >0.34 in identification of LV dysfunction were 91% and 82%, respectively. TDI-MPI was correlated with that measured by PWD (P < 0.004, r = 0.57). Conclusions: Subtle abnormalities of LV function develop early when renal insufficiency is mild to moderate. MPI, measuring by PWD and TDI, are appropriate indicators of overall LV function in young patients with CKD. (Echocardiography 2011;28:97-103)

Objective Several factors have been identified as predictive of future renal function in children with posterior urethral valves (PUV). Our aim was to analyse upper and lower urinary tract outcome in patients with PUV, and determine any factors from the period of early management that were predictive of future renal function. Methods The charts of 67 boys (mean age 2.4 years) diagnosed with PUV were reviewed. The most common presenting symptom was dribbling in 43.2% and UTI in 28.3%. Twenty-three (34.8%) patients developed end-stage renal disease aged 1–15 years. The mean time of renal survival was calculated as 7.8 (SEM = 0.73) years. Results Incontinence in patients over 5 years old, lower urinary tract dysfunction, serum creatinine level in first year or at the time of diagnosis, and presence of vesicoureteral reflux and high-grade bilateral reflux were significant risk factors for occurrence of renal failure in the future. Lower tract dysfunction was seen in 58.6% of patients. Postnatal diagnosis and presence of high-grade reflux were significant risk factors for the future occurrence of lower urinary tract dysfunction. Conclusion It is important to recognize that PUV have consequences not only during childhood or before treatment, but also during or after the treatment period. Long-term assessment and follow-up of upper and lower urinary tract functions after valve ablation is necessary.

Otukesh H, Hosein R, Fereshtehnejad S-M, Riahifard A, Basiri A, Simforoosh N, Chalian M, Jazayeri S, Chalian H, Safarzadeh AE, Sharifian M, Hoseini S. Short-term and long-term effects of slow graft function on graft survival in pediatric live donor renal transplantation. Pediatr Transplantation 2010:14:196–202. © 2009 John Wiley & Sons A/S. Abstract: SGF generally has early and long-term consequences for allograft survival. Limited studies have been performed on SGF and its complications in pediatric renal transplantation. Therefore, 230 children who received transplants between 1985 and 2005 in Labafi Nejad hospital were included in this study. SGF was defined if the serum creatinine level increased, remained unchanged, or decreased by

There is controversy about the outcome of renal transplantation in patients with PUV. The objective of this study was to analyze the outcome of renal transplantation in children with PUV. Fifteen patients had a history of PUV as the etiology of their ESRD. Forty-five patients comprised a control group without lower urinary tract anomalies. Mean age and follow-up duration were not significantly different between the case and the control group (p = 0.1). The immunosuppressive protocol and the year of transplantation were similar in these two groups (p = 0.2, 0.4, respectively). Among patients with PUV, 37.5% had acute rejection; and 56.2% had chronic rejection. Among the controls, 22.2% had acute rejection and 28.8% had chronic rejection. None of these differences was significant. Mean survival time was seven yr in affected patients and 6.2 yr in the control group (p = 0.9). Among patients with PUV, the rate of graft survival in the first year after transplantation was 95%; and those in the third, fifth, and seventh yr, 91%, 65%, and 50%, respectively. For the controls, the graft survival was 83% at one yr; 80% at three yr; 71% at five yr; and 60% at seven yr after transplantation (p = 0.9). Conclusively, this study showed that a history of PUV had no effect on graft function. Graft survival was not different among these patients compared with patients free of these anomalies. We also showed that urological complications were few in these patients.

Hypertension is a late outcome of refl ux nephropathy and renal parenchymal scar secondary to urinary tract infection (UTI). We presumed that it might be detected much earlier after episodes of UTI and the associated cardiovascular risk factors assessed. Between 2009 and 2011, 85 (67 female and 18 male) children aged 5-15 years with a history of febrile UTI, followed-up for at least one year from the fi rst episode of febrile UTI, were enrolled in the study. The variables included 24-hour ambulatory blood pressure monitoring (ABPM), echocardiography, carotid sonography, renal 99mcTc-DMSA, glomerular fi ltration rate, and microalbuminuria. Masked hypertension was detected in 18.8%, hypertension in 7.1% and white coat hypertension in 9.4% of cases. Prehypertension was seen in 20% of children. Out of 85 cases, 43.5% were non-dippers. Out of 56 children with hypertensive and prehypertensive parameters on ABPM, 9.1% showed left ventricular mass index >51g/m2.7 (p>0.05). Signifi cant correlation was only recorded between abnormal blood pressure and the severity of renal parenchymal scar (p, Hipertenzija je kasni ishod refl uksne nefropatije i ožiljaka na bubrežnom parenhimu koji nastaju nakon mokraćne infekcije. Pretpostavili smo da bi se to moglo otkriti znatno ranije nakon epizoda mokraćne infekcije te procijeniti pridružene kardiovaskularne čimbenike rizika. Od 2009. do 2011. godine, u ispitivanje je uključeno 85 (67 Ž, 18 M) djece u dobi od pet do 15 godina s anamnezom febrilne mokraćne infekcije koja su bila praćena najmanje jednu godinu od prve epizode febrilne mokraćne infekcije. Praćene su sljedeće varijable: 24-satno ambulatorno praćenje krvnog tlaka (ambulatory blood pressure monitoring, ABPM), ehokardiografi ja, karotidni ultrazvuk, 99mcTc-DMSA bubrega, glomerularna stopa fi ltracije i mikroalbuminurija. Maskirana hipertenzija otkrivena je u 18,8%, hipertenzija u 7,1% i hipertenzija “bijele kute” u 9,4% slučajeva. Prehipertenzija je zabilježena u 20% djece. Od 85 slučajeva 43,5% ih nije pokazalo odgovarajući pad krvnog tlaka tijekom noći (non-dipper). Od 56 djece s hipertenzivnim i prehipertenzivnim parametrima na ABPM 9,1% ih je imalo indeks lijeve ventrikularne mase veći od 51g/m2.7 (p>0,05). Značajna korelacija zabilježena je između nenormalnog krvnog tlaka i težine ožiljka na bubrežnom parenhimu (p

Kidney transplantation is the treatment of choice for children with end-stage renal disease. In Iran, a kidney transplantation program was started in the Labfi Nejad Hospital, Tehran in 1985. From 1985 to 2003, 278 children (mean age 11.6 years, 59.7% males) received their first renal transplant. All transplants were donations from live donors (12.5% live-related donors); 30.8% of patients were preemptively transplanted. The overall 1-year patient survival rate was 92% and the 5-year survival rate 74%. The median graft survival time was 7.2 years. The rate of graft survival was 88.8% at 1 year, 77% at 3 years, 67% at 5 years, 50% at 7 years, and 43% at 10 years after transplantation. The survival rate of patients and transplants improved significantly with time (p

Background:Hearing defects are the most common sensory disorders, affecting 1 out of every 500 newborns. ATP6V1B mutations are associated with early sensorineural hearing loss, whereas ATP6V0A4 mutations are classically associated with either late-onset sensorineural hearing loss or normal hearing. ATP6V1B1 and ATP6V0A4 genetic mutations cause recessive forms of distal renal tubular acidosis.Method:Ten unrelated deaf Iranian families with distal renal tubular acidosis were referred to the Genetics Research Centre, University of Social Welfare and Rehabilitation Sciences, Tehran. All exons of the ATP6V1B1 and ATP6V0A4 genes were sequenced in affected family members.Results:We identified a previously reported ATP6V1B1 frameshift mutation (P385fsX441) in two families and a nucleotide substitution in exon 10 (P346R) in three families. In addition, one patient was homozygous for a novel nucleotide substitution in exon 3.Conclusion:ATP6V1B1 genetic mutations were detected in more than half of the families studied. Mutations in this gene therefore seem to be the most common causative factors in hearing loss associated with distal renal tubular acidosis in these families.

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 8.48 (SD = 4.8) years. VUR was unilateral in 73 and bilateral in 13 patients, comprising 86 renal refluxing units (RRUs). Pre-operative evaluation consisted of: blood biochemistry, urine analysis and culture, ultrasound scan, voiding cystourethrogram (VCUG), and dimercaptosuccinic acid (DMSA) renal isotope scan. Patients were followed using ultrasound scans at 1 month and every 3 months for the first year and then 2 years after injection. Direct radionuclide cystography with technetium pertechnetate was performed at 3 and 12 months after injection. VCUG was performed only in confirmed cases of failure and downgraded VUR at 3, 12, and 24 months after endoscopic correction. Results Sixty-nine (95%), 61 (83.4%), and 52 (71%) patients completed 3, 12, and 24 months' follow-up respectively. VUR was corrected and downgraded to G I in 81% and 3.3% of RRUs at 3 months' follow-up. The corrected and downgraded RRUs at 12 and 24 months' follow-up were 77%, 10%, and 77%, 11% respectively. De novo contralateral G I VUR was demonstrated in 8.6% of normal ureters. Contralateral GI VUR with normal DMSA isotope renal scans was resolved in 71% of RRUs. Febrile urinary tract infection decreased to 4.1% in the patients. Conclusion According to our study, a single Vantris injection provides a high level of efficacy and safety in the treatment of primary G I–IV VUR in young girls, at 2 years' prospective follow-up.