Publications by authors named "Nakysa Hooman"

44 Publications

Mortality in Children Treated With Maintenance Peritoneal Dialysis: Findings From the International Pediatric Peritoneal Dialysis Network Registry.

Am J Kidney Dis 2021 Feb 4. Epub 2021 Feb 4.

Division of Pediatric Nephrology, Heidelberg University Center for Pediatrics and Adolescent Medicine, Germany.

Rationale & Objective: Research on pediatric kidney replacement therapy (KRT) has primarily focused on Europe and North America. In this study, we aimed to describe the mortality risk of children treated with maintenance peritoneal dialysis (MPD) in different parts of the world and characterize the associated demographic and macro-economic factors.

Study Design: Prospective cohort study.

Setting & Participants: We evaluated patients aged <19 years at inclusion into the International Pediatric Peritoneal Dialysis Network registry, who initiated MPD between 1996-2017.

Exposures: The primary exposure was region (Asia, Western Europe, Eastern Europe, Latin America, North America, and Oceania). Other demographic, clinical and macro-economic (four income groups based on Gross National Income) factors were studied as exposures.

Outcomes: All-cause MPD mortality.

Analytical Approach: Patients were followed for three years. Mortality rates in different regions and income groups were calculated. Cause-specific hazards models with random effects were fit to calculate the proportional change in variance for factors that could explain variation in mortality rates.

Results: A total of 2,956 patients with a median age of 7.8 years at the start of KRT were included. After three years, the overall probability of death was 5%, ranging from 2% in North America to 9% in Eastern Europe. Mortality rates were higher in low-income countries than in high-income countries. Income category explained 50.1% of the variance in mortality risk between regions. Other explanatory factors included peritoneal dialysis modality at start (22.5%) and body mass index (11.1%).

Limitations: The interpretation of interregional survival differences as found in this study may be hampered by selection bias.

Conclusion: This study shows that the overall three-year patient survival on pediatric MPD is high, and that country income is associated with patient survival.
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http://dx.doi.org/10.1053/j.ajkd.2020.11.031DOI Listing
February 2021

Treatment and long-term outcome in primary nephrogenic diabetes insipidus.

Nephrol Dial Transplant 2020 Dec 26. Epub 2020 Dec 26.

Department of Renal Medicine, University College London, London,UK.

Background: Primary nephrogenic diabetes insipidus (NDI) is a rare disorder and little is known about treatment practices and long-term outcome.

Methods: Paediatric and adult nephrologists contacted through European professional organizations entered data in an online form.

Results: Data were collected on 315 patients (22 countries, male 84%, adults 35%). Mutation testing had been performed in 270 (86%); pathogenic variants were identified in 258 (96%). The median (range) age at diagnosis was 0.6 (0.0-60) years and at last follow-up 14.0 (0.1-70) years. In adults, height was normal with a mean (standard deviation) score of -0.39 (±1.0), yet there was increased prevalence of obesity (body mass index >30 kg/m2; 41% versus 16% European average; P < 0.001). There was also increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (32%) and adults (48%). Evidence of flow uropathy was present in 38%. A higher proportion of children than adults (85% versus 54%; P < 0.001) received medications to reduce urine output. Patients ≥25 years were less likely to have a university degree than the European average (21% versus 35%; P = 0.003) but full-time employment was similar. Mental health problems, predominantly attention-deficit hyperactivity disorder (16%), were reported in 36% of patients.

Conclusion: This large NDI cohort shows an overall favourable outcome with normal adult height and only mild to moderate CKD in most. Yet, while full-time employment was similar to the European average, educational achievement was lower, and more than half had urological and/or mental health problems.
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http://dx.doi.org/10.1093/ndt/gfaa243DOI Listing
December 2020

COVID-19 in children treated with immunosuppressive medication for kidney diseases.

Arch Dis Child 2020 Dec 21. Epub 2020 Dec 21.

Paediatric Nephrology, Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.

Background: Children are recognised as at lower risk of severe COVID-19 compared with adults, but the impact of immunosuppression is yet to be determined. This study aims to describe the clinical course of COVID-19 in children with kidney disease taking immunosuppressive medication and to assess disease severity.

Methods: Cross-sectional study hosted by the European Rare Kidney Disease Reference Network and supported by the European, Asian and International paediatric nephrology societies. Anonymised data were submitted online for any child (age <20 years) with COVID-19 taking immunosuppressive medication for a kidney condition. Study recruited for 16 weeks from 15 March 2020 to 05 July 2020. The primary outcome was severity of COVID-19.

Results: 113 children were reported in this study from 30 different countries. Median age: 13 years (49% male). Main underlying reasons for immunosuppressive therapy: kidney transplant (47%), nephrotic syndrome (27%), systemic lupus erythematosus (10%). Immunosuppressive medications used include: glucocorticoids (76%), mycophenolate mofetil (MMF) (54%), tacrolimus/ciclosporine A (58%), rituximab/ofatumumab (11%). 78% required no respiratory support during COVID-19 illness, 5% required bi-level positive airway pressure or ventilation. Four children died; all deaths reported were from low-income countries with associated comorbidities. There was no significant difference in severity of COVID-19 based on gender, dialysis status, underlying kidney condition, and type or number of immunosuppressive medications.

Conclusions: This global study shows most children with a kidney disease taking immunosuppressive medication have mild disease with SARS-CoV-2 infection. We therefore suggest that children on immunosuppressive therapy should not be more strictly isolated than children who are not on immunosuppressive therapy.
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http://dx.doi.org/10.1136/archdischild-2020-320616DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7754669PMC
December 2020

Chronic Kidney Disease in Iran: First Report of the National Registry in Children and Adolescences.

Urol J 2020 Sep 13. Epub 2020 Sep 13.

Pediatric Chronic Kidney Disease Research Center, Department of Pediatric Nephrology, Children's Medical Center, Tehran University of Medical Sciences, Tehran, Iran.

Purpose: Knowing the epidemiological aspects of chronic kidney disease (CKD) in children is crucial for early recognition, identification of reversible causes, and prognosis. Here, we report the epidemiological characteristics of childhood CKD in Iran.   Materials and Methods: This cross-sectional study was conducted during 1991 - 2009.  The data were collected using the information in the Iranian Pediatric Registry of Chronic Kidney Disease (IPRCKD) core dataset.

Results: A total of 1247 children were registered. The mean age of the children at registration was 0.69±4.72 years (range, 0.25 -18 years), 7.79±3.18 years for hemodialysis (HD), 4.24±1.86 years for continuous ambulatory peritoneal dialysis (CAPD), and 3.4±1.95 years for the children who underwent the renal transplantation (RT) (P<.001). The mean year of follow-up was 7.19±4.65 years. The mean annual incidence of CKD 2-5 stages was 3.34 per million age-related population (pmarp). The mean prevalence of CKD 2-5 stages was 21.95 (pmarp). The cumulative 1-, 5-, and 10-year patients' survival rates were 98.3%, 90.7%, and 84.8%, respectively. The etiology of the CKD included the congenital anomalies of the kidney and urinary tract (CAKUT) (40.01%), glomerulopathy (19.00%), unknown cause (18.28%), and cystic/hereditary/congenital disease (11.14%).  Conclusions: 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.
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http://dx.doi.org/10.22037/uj.v16i7.5759DOI Listing
September 2020

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

BMC Med Genet 2020 08 24;21(1):169. Epub 2020 Aug 24.

Department of Medical Genetics and Molecular Biology, school of medicine, Iran University of Medical Sciences, P.O.Box: 1449614525, Tehran, Iran.

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.
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http://dx.doi.org/10.1186/s12881-020-01097-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7446132PMC
August 2020

The Prevalence and Incidence of Hemolytic Uremic Syndrome in Iran, a Systematic Review and Meta-analysis.

Iran J Kidney Dis 2020 05;14(3):173-183

Aliasghar Clinical Research Development center, Aliasghar Children Hospital,Iran University of Medical Sciences, Tehran.

HUS is a leading cause of AKI in infants. Though new classification of HUS is based on underlying disease, it traditionally defines as diarrhea positive (typical) and negative (atypical). We have no figure of the incidence and prevalence of HUS, the underlying disease and the outcome in Iranian patients. This meta-analysis of Iranian studies deals with this matter. We used relevant medical search engines and national databases from 1985 to 2019. We searched manually to detect admissible cross references. All studies assessed for the aspects and the risk of distort by three appraisers. Metaprop package of STAT applied to calculate point prevalence, proportion, and incidence with 95% confidence intervals. A total of 27 articles and one abstract of congress containing 7084 cases met all the inclusion criteria and qualified for the final analysis. Considering 1397 patients with HUS over 33 years of study, the pooled prevalence was 28% (95% CI: 15 to 44) and 18.38 pmp (0.55 pmp/y). In children less than 15 years, the prevalence was 79.82 pmp (2.41 pmp/y). Between 1985 and 2019, atypical HUS was identified in 488 patients with the prevalence of 27.88 pmp (annual prevalence of 0.84 pmp/y of children aged less than 15 years old). The incidence was 9.4 pmp (0.28 pmp/y), contributed to 9.9% (95% CI: 3 to 20) of AKI, and 5.48% (95% CI: 3.5 to 7.9) of CKD and ESRD. The rate of HUS diagnosis was increasing during the previous four decades. HUS consists of a significant number of AKI and ESRD. It needs further prospective longitudinal study.
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May 2020

Molecular Genetic Analysis of Steroid Resistant Nephrotic Syndrome, Detection of a Novel Mutation.

Iran J Kidney Dis 2019 05;13(3):165-172

Department of Medical Genetics and Molecular Biology, Faculty of Medicine; Shahid Akbarabadi Clinical Research Development Unit (ShACRDU); Iran University of Medical Sciences, Tehran, Iran.

Introduction: Nephrotic syndrome is a heterogeneous disease in children, with nearly 10% categorized as steroid-resistant. In this study we evaluated disease related mutations within NPHS1, NPHS2 and new potential variants in other genes.

Methods: In the first phase of study, 25 patients with SRNS were analyzed by Sanger sequencing for NPHS1 (exon 2, 26) and all exons of NPHS2 genes. In the next step, whole exome sequencing was performed on 10 patients with no mutation in NPHS1 and NPHS2.

Results: WES analysis revealed a novel mutation in FAT1 (c.10570C > A; Q3524K). We identified 4 pathogenic mutations, located in exon 4 and 5 of NPHS2 gene in 20% of patients (V180M, P118L, R168C and Leu156Phe). Also our study has contributed to the description of previously known pathogenic mutations across WT1 (R205C) and SMARCAL1 (R764Q) and a novel polymorphism in CRB2.

Conclusion: It seems that NPHS2, especially exons 4 and 5, should be considered as the first step in genetic evaluation of Iranian patients. We suggest conducting WES after NPHS2 screening to identify the potential genes associated with SRNS, Further studies are required to examine more common genes in the first step and then designing native laboratory panels.
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May 2019

The Prevalence of Shiga Toxin-producing Escherichia Coli in Patients with Gastroenteritis in Iran, Systematic Review and Meta-analysis.

Iran J Kidney Dis 2019 05;13(3):139-150

Department of Epidemiology, School of Public Health, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Shiga toxin induced Escherichia Coli (STEC) is associated with chronic kidney disease or neurologic disability. The aim of this study is to determine the prevalence of STEC identified in human studies in Iran. Search engines of PubMed, EMBASE, OVID, SCOPUS, Web of Science, Google Scholar, IranMedex, MagIran, SID and ganj.irandoc were used. All human studies with stool or rectal swap samples evaluated for STEC and the outcome of HUS in Iran, which had been published between 1985 and 2017, were included. Chi-square and I2 statistic tests were applied to assess between-studies heterogeneity. Pooled prevalence and odd ratio were calculated using random effect models. A total of 30 articles containing 23379 samples were included for the final analysis. The design of study was cross sectional in 16, case control in 13 and one was cohort. The pooled prevalence of STEC was 7% (95% CI, 5 - 11; I2 = 98.3%). In subgroup analysis, the pooled prevalence was 8% (95% CI, 4 - 13; I2 = 97.55%) in children but 4% (95% CI, 2 - 7; I2 = 97.66%) in adults. The odds of patients with diarrhea having had STEC were 7.06 times the odds of healthy subjects (pooled OR = 7.06, 95% CI: 3.66-13.61). Patients with bloody diarrhea less likely to have positive STEC than patients with non-bloody diarrhea (pooled OR = 0.33, 95% CI: 0.10-1.02). STEC was prevalent in diarrheic patients and the rate increased in recent years. The highest contamination was seen in East-South of Iran. Public health intervention is mandatory to eliminate it effectively.
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May 2019

Global Variation of Nutritional Status in Children Undergoing Chronic Peritoneal Dialysis: A Longitudinal Study of the International Pediatric Peritoneal Dialysis Network.

Sci Rep 2019 03 20;9(1):4886. Epub 2019 Mar 20.

Children's Mercy Hospital, Kansas City, MO, USA.

While children approaching end-stage kidney disease (ESKD) are considered at risk of uremic anorexia and underweight they are also exposed to the global obesity epidemic. We sought to investigate the variation of nutritional status in children undergoing chronic peritoneal dialysis (CPD) around the globe. The distribution and course of body mass index (BMI) standard deviation score over time was examined prospectively in 1001 children and adolescents from 35 countries starting CPD who were followed in the International Pediatric PD Network (IPPN) Registry. The overall prevalence of underweight, and overweight/obesity at start of CPD was 8.9% and 19.7%, respectively. Underweight was most prevalent in South and Southeast Asia (20%), Central Europe (16.7%) and Turkey (15.2%), whereas overweight and obesity were most common in the Middle East (40%) and the US (33%). BMI SDS at PD initiation was associated positively with current eGFR and gastrostomy feeding prior to PD start. Over the course of PD BMI SDS tended to increase on CPD in underweight and normal weight children, whereas it decreased in initially overweight patients. In infancy, mortality risk was amplified by obesity, whereas in older children mortality was markedly increased in association with underweight. Both underweight and overweight are prevalent in pediatric ESKD, with the prevalence varying across the globe. Late dialysis start is associated with underweight, while enteral feeding can lead to obesity. Nutritional abnormalities tend to attenuate with time on dialysis. Mortality risk appears increased with obesity in infants and with underweight in older children.
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http://dx.doi.org/10.1038/s41598-018-36975-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6426856PMC
March 2019

Treatment and long-term outcome in primary distal renal tubular acidosis.

Nephrol Dial Transplant 2019 06;34(6):981-991

Department of Paediatric Nephrology, Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK.

Background: Primary distal renal tubular acidosis (dRTA) is a rare disorder, and we aimed to gather data on treatment and long-term outcome.

Methods: We contacted paediatric and adult nephrologists through European professional organizations. Responding clinicians entered demographic, biochemical, genetic and clinical data in an online form.

Results: Adequate data were collected on 340 patients (29 countries, female 52%). Mutation testing had been performed on 206 patients (61%); pathogenic mutations were identified in 170 patients (83%). The median (range) presentation age was 0.5 (0-54) years and age at last follow-up was 11.0 (0-70.0) years. Adult height was slightly below average with a mean (SD score) of -0.57 (±1.16). There was an increased prevalence of chronic kidney disease (CKD) Stage ≥2 in children (35%) and adults (82%). Nephrocalcinosis was reported in 88%. Nephrolithiasis was more common with SLC4A1 mutations (42% versus 21%). Thirty-six percent had hearing loss, particularly in ATP6V1B1 (88%). The median (interquartile range) prescribed dose of alkali (mEq/kg/day) was 1.9 (1.2-3.3). Adequate metabolic control (normal plasma bicarbonate and normocalciuria) was achieved in 158 patients (51%), more commonly in countries with higher gross domestic product (67% versus 23%), and was associated with higher height and estimated glomerular filtration rate.

Conclusion: Long-term follow-up from this large dRTA cohort shows an overall favourable outcome with normal adult height for most and no patient with CKD Stage 5. However, 82% of adult patients have CKD Stages 2-4. Importance of adequate metabolic control was highlighted by better growth and renal function but was achieved in only half of patients.
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http://dx.doi.org/10.1093/ndt/gfy409DOI Listing
June 2019

Risk Factors for Early Dialysis Dependency in Autosomal Recessive Polycystic Kidney Disease.

J Pediatr 2018 08 9;199:22-28.e6. Epub 2018 May 9.

Department of Pediatrics, University Hospital of Cologne, Cologne, Germany; Center for Molecular Medicine, University Hospital of Cologne, Cologne, Germany.

Objective: To identify prenatal, perinatal, and postnatal risk factors for dialysis within the first year of life in children with autosomal recessive polycystic kidney disease (ARPKD) as a basis for parental counseling after prenatal and perinatal diagnosis.

Study Design: A dataset comprising 385 patients from the ARegPKD international registry study was analyzed for potential risk markers for dialysis during the first year of life.

Results: Thirty-six out of 385 children (9.4%) commenced dialysis in the first year of life. According to multivariable Cox regression analysis, the presence of oligohydramnios or anhydramnios, prenatal kidney enlargement, a low Apgar score, and the need for postnatal breathing support were independently associated with an increased hazard ratio for requiring dialysis within the first year of life. The increased risk associated with Apgar score and perinatal assisted breathing was time-dependent and vanished after 5 and 8 months of life, respectively. The predicted probabilities for early dialysis varied from 1.5% (95% CI, 0.5%-4.1%) for patients with ARPKD with no prenatal sonographic abnormalities to 32.3% (95% CI, 22.2%-44.5%) in cases of documented oligohydramnios or anhydramnios, renal cysts, and enlarged kidneys.

Conclusions: This study, which identified risk factors associated with onset of dialysis in ARPKD in the first year of life, may be helpful in prenatal parental counseling in cases of suspected ARPKD.
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http://dx.doi.org/10.1016/j.jpeds.2018.03.052DOI Listing
August 2018

The prevalence of hypertension in children with renal scars.

Minerva Pediatr 2017 Jun;69(3):200-205

Department of Pediatric Nephrology, Ali-Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran.

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.
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http://dx.doi.org/10.23736/S0026-4946.16.04217-1DOI Listing
June 2017

Flushing of the vagina and the prepuce-a cause for contaminated urine cultures in children.

Pediatr Nephrol 2017 01 1;32(1):107-111. Epub 2016 Aug 1.

Department of Radiology, Great Ormond Street Hospital for Children, WC1N 3JH, London, UK.

Objective: An uncontaminated urine culture is a prerequisite for the diagnosis of a urinary tract infection. However, this may be difficult to obtain in small children. We have studied the frequency of ballooning of the prepuce in non-circumcised boys and vaginal reflux in girls during voiding as a possible cause of contaminated urine cultures.

Methods: All micturating cystourethrograms (MCUG) performed in our institution over the last 5 years in children aged 0-15 years were reviewed retrospectively for ballooning of the foreskin or vaginal reflux as a potential source of bacterial contamination. The voiding pictures were routinely done with the catheter present for the first voiding cycle and then removed on the second void.

Results: A total of 526 children (77.4 % boys, 22.6 % girls) were eligible for the study. Ballooning of the foreskin was identified on the micturition pictures of 115 (38 %) boys, with the frequency significantly higher in boys aged <12 months [odds ratio (OR) 4.1; 95 % confidence interval (CI) 2.1-7.3)] and boys with vesicoureteral reflux (OR 1.6; 95 % CI 1.06-2.4). Seventeen girls (14.3 %) showed vaginal reflux. No correlation with age or vesicoureteral reflux was found in the girls.

Conclusion: Ballooning of the prepuce or vaginal reflux was seen on a fluoroscopic MCUG in a large proportion of children during their voiding. This normal phenomenon might cause contaminated urine cultures when the urine is obtained by bag or clean catch.
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http://dx.doi.org/10.1007/s00467-016-3463-9DOI Listing
January 2017

A novel mutation pattern of kidney anion exchanger 1 gene in patients with distal renal tubular acidosis in Iran.

Iran J Kidney Dis 2015 May;9(3):230-8

Clinic of Medical Genetics, Ali Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran.

Introduction: Mutations of the anion exchanger 1 (AE1) gene encoding the kidney anion exchanger 1 can result in autosomal dominant or autosomal recessive form of distal renal tubular acidosis (DRTA). This study aimed to report deletion mutations of the AE1 and its impact on Iranian children with DRTA.

Materials And Methods: Twelve children with DRTA referred to Ali Asghar Children Hospital were investigated for all AE1 gene exons through polymerase chain reaction amplification, DNA sequencing, and bioinformatics analysis.

Results: Eleven of 12 patients (91.7%) showed an alteration in AE1 gene with a real hot spot in its exons 11 or 15. Homozygote and heterozygote deletions were confirmed in exon 15 in 5 (41.7%) and 3 (25.0%), respectively. Two patients (16.7%) showed homozygote deletions in exon 11 of AE1 gene, and 1 patient (8.3%) showed point mutation in exon 11. The 3-dimensional structures of the native and these mutant kidney AE1 proteins were determined by the multitemplate method using the Phyre and Hidden Markov Model algorithms.

Conclusions: Parents' consanguinity of these patients reveals that cousins are at a high risk for DRTA. This study is considered as a pilot study showing the importance of AE1 mutations in Iranian children with DRTA and further studies is recommended in this geographic region of the world. These models suggest that alteration in the structures leads to alteration in function and change in the current role of AE1.
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May 2015

Risk factors for loss of residual renal function in children treated with chronic peritoneal dialysis.

Kidney Int 2015 Sep 15;88(3):605-13. Epub 2015 Apr 15.

Pediatric Nephrology Division, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany.

In dialyzed patients, preservation of residual renal function is associated with better survival, lower morbidity, and greater quality of life. To analyze the evolution of residual diuresis over time, we prospectively monitored urine output in 401 pediatric patients in the global IPPN registry who commenced peritoneal dialysis (PD) with significant residual renal function. Associations of patient characteristics and time-variant covariates with daily urine output and the risk of developing oligoanuria (under 100 ml/m(2)/day) were analyzed by mixed linear modeling and Cox regression analysis including time-varying covariates. With an average loss of daily urine volume of 130 ml/m(2) per year, median time to oligoanuria was 48 months. Residual diuresis significantly subsided more rapidly in children with glomerulopathies, lower diuresis at start of PD, high ultrafiltration volume, and icodextrin use. Administration of diuretics significantly reduced oligoanuria risk, whereas the prescription of renin-angiotensin system antagonists significantly increased the risk oligoanuria. Urine output on PD was significantly associated in a negative manner with glomerulopathies (-584 ml/m(2)) and marginally with the use of icodextrin (-179 ml/m(2)) but positively associated with the use of biocompatible PD fluid (+111 ml/m(2)). Children in both Asia and North America had consistently lower urine output compared with those in Europe perhaps due to regional variances in therapy. Thus, in children undergoing PD, residual renal function depends strongly on the cause of underlying kidney disease and may be modifiable by diuretic therapy, peritoneal ultrafiltration, and choice of PD fluid.
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http://dx.doi.org/10.1038/ki.2015.108DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4558567PMC
September 2015

The cosmetic results of a simple method for repairing preputial skin defect in hypospadias.

Iran J Pediatr 2014 Aug 4;24(4):406-10. Epub 2014 Jul 4.

Department of Pediatric Nephrology, Aliasghar Pediatric Hospital, Iran University of Medical Sciences, Tehran, Iran.

Objective: Hypospadias is a common birth defect of the penis. Besides the abnormal position of the urethral opening, there is usually a ventral preputial defect with preputial redundancy in dorsal shaft. There are many flap procedures for correcting this defect. Here, we present our experience of skin coverage procedure with better cosmetic results.

Methods: It is a prospective study on patients with mid-shaft to glandular hypospadias operated from June 2008 to December 2012. The operations were performed by one surgeon in two hospitals and the cosmetic results were evaluated by the surgeon, parents, and another pediatric surgeon by a satisfaction questionnaire. In this procedure, inner prepuce was incised curvilinearly, remaining 5 mm in medial and 8 mm in lateral aspects of the inner prepuce. For skin repair, dorsal flaps were approximated in midline along median raphe.

Findings: Sixty-three patients with mean age of 25.75±8.46 (7-93) months were followed up for 7.06±3.34 (2-15( months. There were 4 complications. The overall satisfaction with penile skin coverage was 93.7% for parents and 98.4% for surgeons. Patients' age and primary site of meatus had a significant correlation with cosmetic results (P<0.05), while urethroplasty techniques and post-operative complications were not significant.

Conclusion: Reapproximation of dorsal flaps in midline is a simple method and can be used in most cases of uncomplicated primary hypospadias. By this technique a more normal appearance can be achieved.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4339564PMC
August 2014

Successful treatment of calciphylaxis with pamidronate.

Iran J Kidney Dis 2015 Jan;9(1):67-70

Department of Pediatric Nephrology, Ali-Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran.

Calciphylaxis is a complication of chronic kidney disease characterized by necrotic lesion in the skin. Histological examination reveals microcalcification of medium-sized blood vessels. We report on a 21-month-old girl with end-stage renal disease with severe calcium-phosphate imbalance. Calciphylaxis process started when she received calcium gluconate intravenously the day before the surgery to correct hypocalcemia and continued progressively despite peritoneal dialysis and forced stopping calcium-containing medication. Pamidronate, 0.5 mg/kg/d, was administered for 6 days and then once a week for 5 weeks. After 1 week, the skin lesion started to heal and circulation improved, and after 6 weeks, all skin lesions completely recovered. Pamidronate was effective to stop calciphylaxis in this case with advanced renal insufficiency and severe calcium-phosphate imbalance. Medical or surgical debridement are not suggested and lesions might recover without scar by pamidronate.
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January 2015

Correlation between serum level of fibroblast growth factor 2 and severity of reflux nephropathy.

Iran J Kidney Dis 2015 Jan;9(1):46-9

Department of Pediatric Surgery, Ali-Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran.

Introduction: Fibroblast growth factor 2 (FGF2) is a potent mitogenic factor of cortical fibroblasts and induces kidney fibrosis. We hypothesized that serum levels of FGF2 has an association with the severity of vesicoureteral reflux (VUR) and renal parenchymal scar.

Materials And Methods: Between 2007 and 2009, a total of 28 children with VUR were enrolled in this study and were compared with 52 healthy children. All children with VUR underwent technetium Tc 99m dimercaptosuccinic acid renal scintigraphy. Fibroblast growth factor 2 was measured in both groups.

Results: The mean level of FGF2 was 65.0 ± 19.0 pg/mL in the VUR group and 62.5 ± 15.3 pg/mL in the control group (P > .05). There was no correlation between serum levels of FGF2 and sex, age, or the grade of VUR. Of the 28 children with VUR, 19 had renal parenchymal scar on dimercaptosuccinic acid renal scintigraphy. The mean serum level of FGF2 was not significantly different in the children with and without renal parenchymal scar.

Conclusions: This study showed no correlation between serum FGF2 and renal parenchymal scar or grade of VUR.
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January 2015

The Prevalence of Pre-hypertension in Children with Type 1 Diabetes Mellitus.

Int J Prev Med 2014 Mar;5(Suppl 1):S44-9

Department of Nursing, Ali-Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran.

Background: Hypertension is more common in adults with type 1 diabetes mellitus (T1DM) than the general population. The aim of this study was to detect the pre-hypertensive stage in children with T1D and to evaluate its correlation with diabetic nephropathy compared to non-diabetic children.

Methods: This was a prospective cross-sectional study in an out-patient clinic of a university hospital. A total of 62 which consists of 36 males and 26 females patients with stable T1D with a median age of 13 year and 42 age - sex-matched healthy children were entered in the study between September 2008 and February 2011. Three readings of blood pressure were recorded. Fasting blood sample was drawn for hemoglobin A1C (HbA1C), creatinine and a 24 h urine aliquot was collected to measure microalbumin, creatinine and volume to estimate glomerular filtration rate (eGFR).

Results: From 62 children with T1DM, 25.8% were in pre-hypertensive stage, 4.8% Stage 1, and 1.6% Stage 2. In controls, 1 (2.4%) out of 42 children was in pre-hypertensive stage (P < 0.0001). Abnormal blood pressures were correlated with eGFR and the duration of disease (P < 0.05), but there were not associated with microalbominuria or HbA1C level.

Conclusions: There was a higher rate of early stage of high normal blood pressure in children with T1DM compared with the healthy controls and this abnormality was only correlated with puberty stage and glomerular filtration rate.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3990927PMC
March 2014

Validity and reliability of Persian translated version of Pediatric Lower Urinary Tract Scoring System Questionnaire for Iranian children.

Authors:
Nakysa Hooman

Med J Islam Repub Iran 2013 Aug;27(3):147-52

Associate professor, Department of Pediatric Nephrology, Ali-Asghar Children Hospital, Pediatric Transplantation and Dialysis Research Center (PTDRC), Iran University of Medical Sciences, Tehran, Iran. ,

Background: Pediatric lower urinary tract scoring system (PLUTSS) is a questionnaire contains items for assessmentof wetting episodes, voiding frequency and pattern. It is used for screening and evaluation of the responseof children with lower urinary tract symptoms to therapy. We studied the validity and reliability of Persiantranslated version of this questionnaire among Iranian children.

Methods: One hundred and ninety-seven children aged 5-15 years with urinary tract infection, voiding dysfunction,enuresis were enrolled in this study. Thirty-three healthy age-matched children without urinary complaintwere considered as controls. PLUTSS questionnaire was filled out for all children. Sonography was performedto rule out urogenital abnormalities. Internal consistency, test-retest reliability, and validity of the questionnairewere assessed using Cronbach's alpha, intra-class correlation coefficient, and ANOVA test respectively.ROC curve was used to define cut-point and its validity in discrimination between groups. P-value < 0.05was considered significant.

Results: The means for PLUTSS items were 6.8 (±5.4) for voiding dysfunction, 14.4(±5) for enuresis,10.5(±7.8) for recurrent and 8.9(±6) for single urinary tract infection, and 1.9(±1.8) for controls (p<0.001).Cronbach's alpha for the 14 items of questionnaire was 0.74. The intraclass coefficient correlation for assessingtest-retest reliability was 0.82 (p<0.001). ROC curve showed cut point of 5 for differing case from controls withsensitivity of 97.0 and specificity of 74.9(p<0.001).

Conclusions: Persian translated PLUTSS has a proper validity and reliability as an instrument for screeningchildren with urinary tract symptoms but some questions need to be changed in some way to be understandableby our culture.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3917488PMC
August 2013

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

Iran J Kidney Dis 2014 Mar;8(2):105-8

Pediatric Transplantation and Dialysis Research Center, Tehran University of Medical Sciences, Tehran, Iran.

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.

Materials And Methods: Forty-eight children with the first febrile urinary tract infection were included. Twelve patients had low-grade VUR, 9 patients had high-grade VUR, and 27 patients did not have any VUR in their imaging assessment.

Results: There was a significant association between high-grade VUR and higher levels of procalcitonin (P = .04). The sensitivity of a procalcitonin level of 0.31 ng/mL or greater was 90% and the specificity was 32% for diagnosis of high-grade VUR.

Conclusions: We concluded that serum procalcitonin concentration is a sensitive and promising predictor of high-grade VUR.
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March 2014

What is the real risk factor of urinary calculi in Iranian children?

Authors:
Nakysa Hooman

Iran J Kidney Dis 2014 Jan;8(1):2-3

Department of Pediatric Nephrology, Ali-Asghar Children Hospital and Pediatric Transplantation and Dialysis Research Center, Iran University of Medical Sciences, Tehran, Iran.

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January 2014

Carotid artery function in children with idiopathic nephrotic syndrome.

Nefrologia 2013 ;33(5):650-6

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. The inclusion criteria were: idiopathic nephrotic syndrome with normal serum complement, at least one year after initiation of disease, glomerular filtration rate more than 20 mL/min/1.73 m², age over two years old at the time of study. Seventy-five healthy sex-age-matched children considered as a control group. Carotid function parameters and left ventricular mass index were studied in nephrotic children.

Results: Steroid sensitive, resistant, and dependent nephrotic syndrome included one-third each. The mean carotid intima-media thickness (mm) in nephrotic children was 0.42 (±.14) while the mean cIMT in controls was 0.37 (±.08) (p-value <.05). After log transformation, General Linear Multivariate analysis revealed significant difference of carotid intima-media thickness in nephrotic patients (p-value <.001). Subsequently, the factor that influenced on cIMT was duration of disease (P<.05). One-half of nephrotic children who had echocardiography, showed left ventricular hypertrophy. It was correlated with carotid stiffness and systolic hypertension (P<.05).

Conclusions: Carotid intima-media thickness was thicker in nephrotic children. Carotid parameters were influenced by duration of disease and hypertension.
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http://dx.doi.org/10.3265/Nefrologia.pre2013.May.12036DOI Listing
August 2014

Ambulatory blood pressure monitoring for children with β-thalassemia major: a preliminary report.

Iran J Kidney Dis 2013 Jul;7(4):299-303

Department of Pediatric Nephrology, Ali-Asghar Children Hospital, Iran University of Medical Sciences, Tehran, Iran.

Introduction: Heart disease is one of the most common reasons of death in beta-thalassemia major. A few studies have been done in children about blood pressure changes. The aim of this study was to assess hemodynamic changes by ambulatory blood pressure monitoring (ABPM).

Materials And Methods: In this cross-sectional study, 30 patients with beta-thalassemia major aged 5 to 18 years old were evaluated with 24-hour ABPM. The exclusion criteria were an ejection Fraction less than 50% and a glomerular filtration rate less than 90 mL/min/1.73 m2. Hypertension was defined as a mean blood pressure index of 1 and greater with or without load blood pressure greater than 25%. Dipper status was defined as a 10% decrease in nighttime versus daytime mean arterial blood pressure.

Results: High blood pressure was detected in 16.7% of the patients. The whole-day ABPM showed hypertension in 6.7% of the children. During daytime measurements, systolic hypertension was seen in 3.3% (load 3.7%) and diastolic in 6.7% (load 3.3%). These figures for nighttime evaluation were 6.7% (load 3.3%) and 10.3% (load 6.9%), respectively. Nondipper status was detected in 56.7% of the children. There was no significant correlation between abnormal blood pressure and age, sex, body mass index, hemoglobin, number or rates of blood transfusion, or serum ferritin level.

Conclusions: The ABPM may be a useful instrument for early detection of hemodynamic changes in children with beta-thalassemia major.
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July 2013

Toilet training in Iranian children: a cross-sectional study.

Iran J Pediatr 2013 Apr;23(2):154-8

Pediatric Nephrology Division, Ali-Asghar Children's Hospital, Tehran University of Medical Sciences, Iran ; Iranian Society of Pediatric Nephrology, Tehran, Iran.

Objective: There is still controversy about the methods and the age of toilet training that are varied in different cultures. This is a survey of Iranian parents' views about the appropriate age, the true age, the methods used for toilet training, and the association with voiding problems.

Methods: Questionnaires were filled-out containing items on demographic data, the parents' view, the method applied, and the age at which toilet training was accomplished in children aged 2 months to 5 years. In addition, pediatric lower urinary tract scoring system questionnaires were distributed among 217 children aged 5-15 years with lower urinary tract symptoms between 2008 and 2010 in outpatient clinics. P<0.05 was considered significant.

Findings: 566 children (335 girls and 231 boys) were assigned to the study. In asymptomatic group, the majority of parents believed that the appropriate age to start toilet training was 1-2 years. The method used by the parents was intensive in 52% and child-oriented in 44%. There was strong reverse correlation between the level of education of father with applying punishment for training and direct correlation between toilet refusal and the later age of completing toilet training (LR: 6.3, P<0.05). The mean age of completing toilet training was about 23 months in asymptomatic and 23.7 months in symptomatic children (P>0.05). There was no correlation between wetting episodes at day or night and the age of toilet training.

Conclusion: Intensive approach was more popular and the age of toilet training had no influence on the lower urinary tract symptoms.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3663305PMC
April 2013

Bisphosphonates for treatment of severe idiopathic infantile hypercalcemia.

Authors:
Nakysa Hooman

Iran J Kidney Dis 2013 Mar;7(2):79-81

Division of Pediatric Nephrology, Ali-Asghar Children Hospital, Tehran University of Medical Sciences, Tehran, Iran.

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March 2013

The effect of vitamin E or vitamin A on the prevention of renal scarring in children with acute pyelonephritis.

Pediatr Nephrol 2013 Feb 7;28(2):277-83. Epub 2012 Oct 7.

Pediatric Infectious Disease, Ali-Asghar Children Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Background: Numerous factors may contribute to renal tissue injury after urinary tract infection. We have evaluated the effects of vitamins A or E supplementation in combination with antibiotics for the prevention of renal scarring in acute pyelonephritis.

Methods: A simple non-blind randomized clinical trial was conducted on 61 children aged 1 month to 10 years between 2004 and 2006. The inclusion criteria were positive urine culture, clinical findings, and 99mTc-dimercaptosuccinic acid (DMSA) scintigraphy-based evidence in favor of acute pyelonephritis. The children were randomized into three treatment groups: 10-day treatment with only antibiotics (control group; n = 25) and 10-day treatment with supplements of vitamin A (n = 17) or vitamin E (n = 18) in addition to antibiotics during the acute phase of infection. The final analysis was performed after excluding male patients. Each patient was evaluated twice by 99mTc-DMSA scintigraphy performed at least 6 months apart. P < 0.05 was considered to be statistically significant.

Results: The analysis included 108 kidney units. The frequency of inflammation at the beginning of therapy was not significantly different in the three groups (63.3 % in vitamin A, 61 % in vitamin E, and 76.2 % in the control group). A worsening of lesions, based on the second 99mTc-DMSA scan, was observed in 42.5, 0, and 23.3 % of the control, vitamin E, and vitamin A patients, respectively (LR = 26.3, P < 0.001).

Conclusion: Vitamins A or E supplements were effective in reducing renal scarring secondary to acute pyelonephritis.
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http://dx.doi.org/10.1007/s00467-012-2308-4DOI Listing
February 2013

Prognostic value of dialysis effluent leukocyte count in children on peritoneal dialysis with peritonitis.

Iran J Kidney Dis 2012 Mar;6(2):114-8

Department of Pathology, Ali-Asghar Children’s Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Introduction: Early prediction of the efficacy of treatment in peritonitis complicating peritoneal dialysis (PD) is the best way to reduce morbidity. We studied the prognostic value of the third-day dialysis effluent leukocyte count after antibiotic therapy for prediction of treatment outcomes.

Materials And Methods: Medical records of 31 children on PD, younger than 15 years old, admitted in Ali-Asghar Children's Hospital because of PD-related peritonitis, were reviewed retrospectively. Peritonitis was defined by fever, abdominal pain, and cloudy effluent with a leukocyte count greater than 100/mm3 or a positive dialysis effluent culture for microorganisms. For each episode of peritonitis, the leukocyte count of the effluent was measured on the third day after initiation of empiric therapy and culture results were recorded. The receiver operating characteristic curve was used to perform predicting value assessments.

Results: Of 60 episodes of peritonitis, 68.3% were treated successfully. Of the remaining episodes, 15.8% resulted in mortality, 57.9% required catheter removal, and 26.3% led to both. The mean PD effluent leukocyte count on the third day after initiating empiric antibiotics was significantly higher in the group with treatment failure (2258 ± 796/mm3) than in the group with successful treatment (1325 ± 669/mm3; P < .001). The cutoff point of 1240/mm3 was found with optimized sensitivity (100%), specificity (63.4%), positive predictive value (55.9%), and negative predictive value (100%) for prediction of treatment failure (P < .001).

Conclusions: This study showed that the third-day dialysis effluent leukocyte count predicted short outcomes of peritonitis.
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March 2012

The correlation between bladder volume wall index and the pattern of uroflowmetry/external sphincter electromyography in children with lower urinary tract malfunction.

J Pediatr Urol 2012 Aug 5;8(4):367-74. Epub 2011 Aug 5.

Department of Pediatric Radiology, Ali-Asghar Children Hospital, Tehran University of Medical Sciences, Tehran, Iran.

Objective: To evaluate the relationship between bladder volume wall index (BVWI) and the pattern of uroflowmetry in children with lower urinary tract malfunction.

Patients & Methods: 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.

Results: The bladder was thick (<70%) in 39 (28 cases, 11 controls) and thin (>130%) in 35 (18 cases, 17 controls) (P > 0.05). Uroflowmetry was abnormal in 82 (61 cases, 21 controls) (P < 0.05). Severe sphincter dyssynergia was detected in 47% of cases compared with 20% of controls (P < 0.05).There was no relationship between BVWI and uroflowmetry in cases or in controls (P > 0.05). The median post-void residual urine was not statistically different between the groups (20 vs 12.3 ml) (P > 0.05). When both bladder sonography and uroflowmetry were abnormal, they had an association with abnormal urodynamics (P < 0.05).

Conclusion: 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.
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http://dx.doi.org/10.1016/j.jpurol.2011.07.009DOI Listing
August 2012