Abstract：
OBJECTIVE:Paucity of interlobular bile ducts (PIBD), defined as absence or marked decrease in the number of interlobular bile ducts, is one of the causes of neonatal cholestasis. Treatment includes treating the intractable pruritus caused by persistent cholestasis. PIBD can be part of a familial syndrome of cholestasis named Alagille syndrome (AGS). We report clinical status of a case series of Iranian patients with PIBD. METHODS:In this retrospective study, patients with cholestasis admitted to the pediatric gastroenterology ward in a referral hospital in Shiraz from January 2006 to January 2010 and underwent liver biopsy were evaluated. Clinical and paraclinical status of children with the pathologic diagnosis of PIBD was assessed. FINDINGS:Disease was presented in all jaundiced patients with aged in average 3 days at presentation. Seven patients had the criteria of AGS. Despite medical treatment, cholestasis was not controlled in 6 (28.6%) patients. Liver transplantation led to the survival of 5 patients while the other patient who did not undergo liver transplantation died at 2 months of age. One patient underwent peritoneal dialysis due to renal insufficiency and died at 9 months of age. After 1-5 years of follow-up, the mortality rate was 9.5%. CONCLUSION:In patients with intractable cholestasis, only patients that underwent liver transplantation survived. Thus, the most important criterion for liver transplantation in neonatal PIBD is intractable cholestasis. This is the first report that shows AGS can result in neonatal-onset renal insufficiency.
journal_name
Iran J Pediatrjournal_title
Iranian journal of pediatricsauthors
Mozhgan Z,Bita G,Mahmood H,Hajar Esubject
Has Abstractpub_date
2013-02-01 00:00:00pages
65-70issue
1eissn
2008-2142issn
2008-2150journal_volume
23pub_type
杂志文章abstract：BACKGROUND:Among different categories of sedative agents, benzodiazepines have been prescribed for more than three decades to patients of all ages. The effective and predictable sedative and amnestic effects of benzodiazepines support their use in pediatric patients. Midazolam is one of the most extensively used benzod...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.25(3)2015.494
更新日期：2015-06-01 00:00:00
abstract：OBJECTIVE:Most neonatal encephalopathic disorders appear to be caused by perinatal events. Persistent myocardial ischemia leads to cellular necrosis and release of troponin from cardiac muscles. Fetal distress during labor may be detected by monitoring the fetal heart rate. However little is known about the relationshi...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2013-02-01 00:00:00
abstract：:Congenital adrenal hyperplasia (CAH) is characterized by impaired biosynthesis of cortisol. 21-hydroxylase deficiency is the most common cause of CAH affecting 1 in 10000-15000 live births over the world. The frequency of the disorder is very high in Iran due to frequent consanguineous marriages. Although biochemical ...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2011-06-01 00:00:00
abstract：INTRODUCTION:Truncus arteriosus with intact ventricular septum is a rare and unique variant of persistent truncus arteriosus (PTA) which usually presents with central cyanosis and congestive heart failure in neonate and early infancy. Associated cardiac and non-cardiac anomalies may affect morbidity and mortality of th...
journal_title：Iranian journal of pediatrics
pub_type：
doi：10.5812/ijp.2081
更新日期：2015-10-01 00:00:00
abstract：OBJECTIVE:Due to recent scientific progress in assisted reproductive techniques (ART), infertile couples can now become fertile. Thus, a number of infants in our country are the results of these costly interventions. This study has been undertaken to evaluate the physical growth process of different methods of ART infa...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2011-12-01 00:00:00
abstract：OBJECTIVE:The objective of this randomized controlled trial was to compare the treatment failure of suspected early onset neonatal sepsis with either 3-day or 5-day course of empirical antibiotic therapy. METHODS:Infants with birth weight over 1500 g and/or gestational age over 34 weeks within 7 days postnatal age wit...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2014-12-01 00:00:00
abstract：BACKGROUND:Hepatitis A virus (HAV) infection constitutes an important health problem in developing countries. It is usually a benign self-limiting disease, but may present with atypical clinical findings. CASE PRESENTATION:A twelve-year-old male with ascites, pleural effusion, and acalculous cholecystitis during the c...
journal_title：Iranian journal of pediatrics
pub_type：
doi：
更新日期：2010-12-01 00:00:00
abstract：OBJECTIVE:Late-onset sepsis is responsible for high morbidity and mortality in newborn infants in the world and in particular in developing countries. In this study, we evaluated whether clinical characteristics, laboratory parameters and measurements of serum interleukin-8 (IL-8) are able to discriminate between late ...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2010-03-01 00:00:00
abstract：BACKGROUND:Nijmegen Breakage Syndrome(NBS) is a rare autosomal recessive disorder with specific clinical features, characteristic chromosomal breakage and combined imunodeficiency. Patients with this condition also associate growth retardation with microcephaly, predisposition to malignancy and specific skin manifestat...
journal_title：Iranian journal of pediatrics
pub_type：
doi：
更新日期：2013-02-01 00:00:00
abstract：OBJECTIVE:Cyanotic congenital heart diseases (CCHDs) are a series of cardiac anomalies that have long been recognized as a potential cause of nephropathy. There have been few reports on renal impairment in patients with CCHD before and after corrective cardiac surgery. The aim of this study was to evaluate the prevalen...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2014-02-01 00:00:00
abstract：OBJECTIVE:Growth charts are widely used to assess children's growth status and can provide a trajectory of growth during early important months of life. Racial differences necessitate using local growth charts. This study aimed to provide standardized growth curves of body mass index (BMI) for children living in northe...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2011-03-01 00:00:00
abstract：OBJECTIVES:Henoch-Schonlein Purpura (HSP) is the most widespread systemic vasculitis during childhood. Gastrointestinal tract retention and gastrointestinal bleeding are among its major complications. Neutrophil-Lymphocyte ratio (NLR) and platelet-lymphocyte ratio (PLR) are indicators related to inflammatory diseases. ...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.8191
更新日期：2016-10-10 00:00:00
abstract：BACKGROUND:The Ehlers-Danlos syndrome type VI (EDSVI) is an autosomal recessive connective tissue disease which is characterized by severe hypotonia at birth, progressive kyphoscoliosis, skin hyperelasticity and fragility, joint hypermobility and (sub-)luxations, microcornea, rupture of arteries and the eye globe, and ...
journal_title：Iranian journal of pediatrics
pub_type：
doi：
更新日期：2010-09-01 00:00:00
abstract：OBJECTIVE:The aim of this study was to examine the differences in the health related quality of life and the self care and social function in daily life of children with different disabilities. METHODS:One hundred and two children with physical, emotional and cognitive disabilities (cerebral palsy, mental retardation,...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2013-06-01 00:00:00
abstract：BACKGROUND:Diphallus is a rare anomaly and accompanying anomalies vary from bifid scrotum, bladder exstrophy, imperforate anus and colo-rectal anomaly such as duplication, and other associated anomalies. CASE PRESENTATION:A 2-day old infant is reported with imperforate anus and complete duplication of recto-sigmoid co...
journal_title：Iranian journal of pediatrics
pub_type：
doi：
更新日期：2010-06-01 00:00:00
abstract：BACKGROUND:Burning fat and carbohydrates to provide energy in biological systems causes the formation of free oxygen species. OBJECTIVES:This study aimed to evaluate the oxidative status of serum and breast milk of mothers giving birth prematurely and at full-term. MATERIALS AND METHODS:The study comprised 50 mothers...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.2363
更新日期：2015-08-01 00:00:00
abstract：BACKGROUND:Allgrove syndrome is a rare autosomal recessive condition characterized by adrenal insufficiency, achalasia, alacrima and occasionally autonomic disturbances. Mutations in the AAAS gene, on chromosome 12q13 have been implicated as a cause of this disorder. CASE(S) PRESENTATION:We present various manifestati...
journal_title：Iranian journal of pediatrics
pub_type：
doi：
更新日期：2010-03-01 00:00:00
abstract：OBJECTIVE:Urinary tract infection (UTI) is one of the most common causes of febrile pediatric diseases. Also, vesicoureteral reflux (VUR) is a significant risk factor for UTI. Voiding cystourethrography (VCUG) is the method of choice for evaluation of VUR. This study was done to predict VUR by DMSA scan (technetium 99 ...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2012-03-01 00:00:00
abstract：OBJECTIVE:The aim of this study was to compare the Persian version of the wechsler intelligence scale for children - fourth edition (WISC-IV) and cognitive assessment system (CAS) tests, to determine the correlation between their scales and to evaluate the probable concurrent validity of these tests in patients with le...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2013-04-01 00:00:00
abstract：BACKGROUND:Umbilical arterial blood gas (UABG) analysis is more objective than other methods for predicting neonatal outcome. Acidemic neonates may be at risk for unfavorable outcome after birth, but all neonates with abnormal arterial blood gas (ABG) analysis do not always have poor outcome. OBJECTIVES:This study was...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.25(3)2015.174
更新日期：2015-06-01 00:00:00
abstract：BACKGROUND:Atrial septal defect (ASD) is a common congenital heart disease. OBJECTIVES:The aim of this study was to assess the clinical course of ASD, as well as its frequency of spontaneous closure and regression of diameter. METHODS:In a study conducted from 2000 to 2011, 192 consecutive patients with an isolated A...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.4649
更新日期：2016-08-01 00:00:00
abstract：OBJECTIVE:The aim of this study was to evaluate the bilirubin albumin (B/A) ratio in comparison with total serum bilirubin (TSB) for predicting acute bilirubin-induced neurologic dysfunction (BIND). METHODS:Fifty two term and near term neonates requiring phototherapy and exchange transfusion for severe hyperbilirubine...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2011-03-01 00:00:00
abstract：CONTEXT:Blood pressure (BP) tracks from childhood to adulthood, and has ethnic variations. Therefore, it is important to assess the situation of pediatric BP in different populations. This study aims to systematically review the studies conducted on BP in Iranian children and adolescents. EVIDENCE ACQUISITION:We condu...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章,评审
doi：10.5812/ijp.4496
更新日期：2016-03-05 00:00:00
abstract：OBJECTIVE:To identify and compare risk factors for recurrent respiratory infections in preschool children between resident and floating population in Yiwu, China. METHODS:Investigations was conducted in resident and floating population in Yiwu city, Zhejiang province. A structured questionnaire was used to collect inf...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2014-02-01 00:00:00
abstract：OBJECTIVE:This study aims at providing local reference values for blood pressure by height and determining distribution pattern of systolic and diastolic blood pressure in 6.5-11.5 elementary school children for the first time in Shiraz (Southern Iran). METHODS:Height, systolic blood pressure (SBP) and diastolic blood...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2012-06-01 00:00:00
abstract：OBJECTIVE:Acute bacterial meningitis in pediatrics remains a serious and potentially lethal disease. Its prognosis is critically dependent on rapid diagnosis and treatment. The use of biological markers, like procalcitonin, has been proposed to facilitate the accuracy of the initial diagnosis of bacterial meningitis. T...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2014-12-01 00:00:00
abstract：OBJECTIVE:Shiga toxin-producing E. coli (STEC) is a pathogenic E. coli that may cause hemolytic uremic syndrome (HUS) after diarrheal disease through Shiga toxins. Management of the patients with STEC infection is different from that of other diarrheal diseases due to increase in frequency of HUS after antibiotic admin...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2013-12-01 00:00:00
abstract：OBJECTIVE:The aim of the study was the evaluation of patients treated with a diagnosis of gastroschisis and to establish the factors which affected the morbidity and mortality. METHODS:Twenty-nine patients, managed for gastroschisis during 2000-2010 were reviewed retrospectively. Patients were analysed in respect to g...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2012-09-01 00:00:00
abstract：OBJECTIVE:Injection of botulinum toxin into the anal sphincter is a novel and safe new treatment of chronic idiopathic constipation and anal fissure in children. The purpose of this study was to determine the utility of intra sphincteric injection of botox in the treatment of children with refractory constipation. MET...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2013-10-01 00:00:00
abstract：OBJECTIVE:Comparing the effect of topical human milk application and dry cord care on cord separation time. METHODS:This research was a randomized clinical trial study on 130 singleton and mature newborns. Newborns were placed randomly in groups of topical application of human milk and dry cord care. The umbilical sep...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2012-06-01 00:00:00
