Abstract：
BACKGROUND:Classic Pelizaeus-Merzbacher disease is a rare x-linked disorder of proteolipid protein expression first described clinically in 1885. This disease is characterized by abnormal eye movements, very slow motor development and involuntary movements. The causative gene is PLP1. CASE PRESENTATION:A 1-year-old boy was referred to our clinic due to abnormal eye movements. He had horizontal and flickering eye oscillation, psychomotor retardation, hypotonia and head nodding. We found hypomyelination in brain MRI. CONCLUSION:The possibility of Pelizaeus-Merzbacher disease should be considered in boys with abnormal eye movements, psychomotor retardation and hypotonia.
journal_name
Iran J Pediatrjournal_title
Iranian journal of pediatricsauthors
Ashrafi MR,Mohammadi M,Alizadeh H,Nikkhah Asubject
Has Abstractpub_date
2011-09-01 00:00:00pages
395-8issue
3eissn
2008-2142issn
2008-2150journal_volume
21pub_type
abstract：BACKGROUND:B-type natriuretic peptide (BNP) levels are elevated in children with congenital heart disease involving a left-to-right shunt (LRS) and are also raised in dilated cardiomyopathy (DCM). As far as we know, there are few reports in the literature comparing the change of the NT-proBNP in LRS and DCM especially ...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.4485
更新日期：2016-05-15 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：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：OBJECTIVE:Bruxism is defined as the habitual nonfunctional forceful contact between occlusal tooth surfaces. The aim of this study was to determine the prevalence of bruxism and correlated factors in children referred to dental schools of Tehran, based on Parent's report. METHODS:This cross-sectional descriptive study...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2010-06-01 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：BACKGROUND:Intracranial arteriovenous malformation rarely causes pulmonary hypertension and congestive heart failure in the newborn. Its diagnosis is challenging because cardiomegaly may suggest an intra-cardiac structural lesion. CASE PRESENTATION:We present two newborns, one 2-day-old male and the other 11-day-old f...
journal_title：Iranian journal of pediatrics
pub_type：
doi：
更新日期：2013-04-01 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：:Low back pain (LBP) is common among children and adolescents. In younger children particularly those under 3, LBP should be considered as an alarming sign for more serious underlying pathologies. However, similar to adults, non specific low back pain is the most common type of LBP among children and adolescents. In th...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2011-09-01 00:00:00
abstract：BACKGROUND:Scrotal exploration is considered the procedure of choice for acute scrotum. OBJECTIVES:We evaluated the importance of early diagnosis and testicular salvage on the therapeutic outcomes of patients with pediatric testicular torsion (TT) and testicular appendage torsion (TAT) in our geographic area. PATIENT...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.248
更新日期：2015-02-01 00:00:00
abstract：OBJECTIVE:The objective of this study was to determine the normative data and psychometric properties of the parent and teacher rating form of the child behavior checklist (CBCL) in an Iranian community sample. METHODS:A sample of 6-12 year old students was randomly selected from ten elementary schools in Tehran, Iran...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2011-09-01 00:00:00
abstract：BACKGROUND:Healthcare-associated bloodstream infections (HCA-BSI) are a major cause of morbidity and mortality in neonatal intensive care units (NICUs). OBJECTIVES:We aimed to determine the causative organisms and risk factors of HCA-BSIs in NICUs. METHODS:This study was performed between January 2011 and December 20...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.5213
更新日期：2016-07-27 00:00:00
abstract：CONTEXT:To assess the efficacy of preoperative chemotherapy in Wilms' tumor patients and explore its true value for specific subgroups. OBJECTIVES:In the presence of these controversies, a meta-analysis that examines the efficacy of preoperative chemotherapy in Wilms' tumor patients and specific subgroups is needed to...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.366
更新日期：2015-04-01 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: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：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: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：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:Beta thalassemia major is a prevalent hereditary disease in Mediterranean region especially Iran. Early blood transfusion is necessary for most of the patients and frequent transfusion can cause various medical problems for the patients. The aim of this study was to find major causes of hospital admission in ...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2011-12-01 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：: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：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
abstract：OBJECTIVE:To determine the risk factors associated with lower respiratory tract infections (LRTI) related hospitalizations in preterm infants receiving palivizumab throughout the high season for respiratory syncytial virus (RSV) infection. METHODS:Premature infants who were commenced on palivizumab prophylaxis during ...
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: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:Common variable immunodeficiency (CVID) is a heterogeneous disorder characterized by reduced serum level of IgG, IgA or IgM and recurrent bacterial infections. Class switch recombination (CSR) as a critical process in immunoglobulin production is defective in a group of CVID patients. Activation-induced cytid...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2013-08-01 00:00:00
abstract：OBJECTIVE:The goal of this study was to evaluate the effect of protein energy malnutrition on protein glycosylation by investigating transferrin isoform pattern and its relationship to the degree of malnutrition and the biochemical markers of nutritional status in children. METHODS:Forty one children with mild (n=23) ...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2014-06-01 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:Providing a safe and efficient dental treatment for a young patient is a challenge for the dentist and the child. The purpose of this study was to investigate the effectiveness, safety and acceptability of buccal midazolam in dental pediatric patients and to compare it with oral Midazolam. METHODS:Eighteen u...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2014-04-01 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：OBJECTIVE:To analyze the clinical and radiological features of paragonimiasis in children and raise the awareness of this disease. METHODS:A total of 58 paragonimiasis patients were reviewed. They were 42 boys and 16 girls aged 2.0 to 15.3 years. FINDINGS:Among these patients, 20 were diagnosed in the recent 5 years,...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：
更新日期：2012-12-01 00:00:00
abstract：BACKGROUND:Infantile Onset Pompe Disease (IOPD) is a rare autosomal recessive neuromuscular disorder. It is associated with cardiomegaly, hypotonia, paresis, and death in the first year of life. Since 2006, following the use of Alglucosidase alfa as Enzyme Replacement Therapy (ERT), the patients' survival is improved t...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.4473
更新日期：2016-02-01 00:00:00
