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 cytidine deaminase (AID) protein is an important molecule involving CSR process. The aim of this study was to investigate the AID gene mRNA production in a group of CVID patients indicating possible role of this molecule in this disorder. METHODS:Peripheral blood mononuclear cells (PBMC) of 29 CVID patients and 21 healthy controls were isolated and stimulated by CD40L and IL-4 to induce AID gene expression. After 5 days AID gene mRNA production was investigated by real time polymerase chain reaction. FINDINGS:AID gene was expressed in all of the studied patients. However the mean density of extracted AID mRNA showed higher level in CVID patients (230.95±103.04 ng/ml) rather than controls (210.00±44.72 ng/ml; P=0.5). CVID cases with lower level of AID had decreased total level of IgE (P=0.04) and stimulated IgE production (P=0.02); while cases with increased level of AID presented higher level of IgA (P=0.04) and numbers of B cells (P=0.02) and autoimmune disease (P=0.02). CONCLUSION:Different levels of AID gene expression may have important roles in dysregulation of immune system and final clinical presentation in CVID patients. Therefore investigating the expression of AID gene can help in classifying CVID patients.
journal_name
Iran J Pediatrjournal_title
Iranian journal of pediatricsauthors
Abolhassani H,Farrokhi AS,Pourhamdi S,Mohammadinejad P,Sadeghi B,Moazzeni SM,Aghamohammadi Asubject
Has Abstractpub_date
2013-08-01 00:00:00pages
451-7issue
4eissn
2008-2142issn
2008-2150journal_volume
23pub_type
杂志文章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...
journal_title:Iranian journal of pediatrics
pub_type: 杂志文章
doi:
更新日期:2013-02-01 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: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:To investigate T-cell immunoglobulin domain and mucin domain-containing molecule-3 (Tim-3) and its ligand galectin-9 mRNA expression in peripheral blood mononuclear cells (PBMCs) from Henoch-Schoenlein Purpura (HSP) patients. METHODS:Quantitative real-time reverse transcription-polymerase chain reaction (PCR...
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: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 bo...
journal_title:Iranian journal of pediatrics
pub_type:
doi:
更新日期:2011-09-01 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:OBJECTIVE:There are some reports in which a condition of zinc deficiency and its associated outcomes with a change in concentration of serum copper among the thalassemic patients has been highlighted. The aim of this prospective study was to determine the serum zinc and copper levels in children with beta-thalassemia m...
journal_title:Iranian journal of pediatrics
pub_type: 杂志文章
doi:
更新日期:2010-09-01 00:00:00
abstract:OBJECTIVE:Epilepsy is a most common serious neurological disorder and is one of the world's most prevalent non-communicable diseases. The aim of this study was to determine the prevalence and risk factors of epilepsy in 0-17 year old children in Trabzon, Turkey. METHODS:A cross-sectional epidemiological investigation ...
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: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: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:OBJECTIVE:The aim of the study was to evaluate clinical characteristics of ureteroceles particularly for diagnostic and treatment challenges. METHODS:Data about patients treated for ureterocele in the two hospital clinics during 1996- 2009 are retrospectively evaluated. FINDINGS:There were 12 girls and 7 boys. Sympto...
journal_title:Iranian journal of pediatrics
pub_type: 杂志文章
doi:
更新日期:2010-12-01 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:BACKGROUND:Congenital midline cervical cleft (CMCC) is a rare developmental anomaly of the anterior neck, mostly found in Caucasian females. Most authors consider it within the spectrum of branchial arch developmental abnormalities. There have been about 100 reported cases in the literature. However, there is no agreem...
journal_title:Iranian journal of pediatrics
pub_type:
doi:
更新日期:2012-12-01 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:OBJECTIVE:Human pandemic influenza H(1)N(1) virus as the cause of febrile respiratory infection ranging from self-limited to severe illness has spread globally during 2009. Signs and symptoms of upper and lower respiratory tract involvement, fever, sore throat, rhinitis, myalgia, malaise, headache, chills and fatigue a...
journal_title:Iranian journal of pediatrics
pub_type: 杂志文章
doi:
更新日期:2011-06-01 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: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: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: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:CONTEXT:Universal immunization against Bordetella pertussis has partially controlled the burden of the disease and its transmission. However, according to recent data, the epidemiology of this vaccine-preventable disease has changed. Now, younger infants, adolescents, and adults are at greater risk of infection. This a...
journal_title:Iranian journal of pediatrics
pub_type: 杂志文章,评审
doi:10.5812/ijp.5514
更新日期:2016-06-12 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
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: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: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: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: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: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:It is believed that fingerprints and palm patterns may represent genetically determined congenital abnormalities in Cystic Fibrosis (CF). The main idea of this paper was to determine differences of fingerprints and palm patterns in CF and normal children. METHODS:Forty-six CF children (27 males, 19 females) ...
journal_title:Iranian journal of pediatrics
pub_type: 杂志文章
doi:
更新日期:2014-10-01 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: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