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 manifestations. CASE PRESENTATION:Here we report a 3-year old girl known with NBS associated with cutaneous sarcoid-like lesions. She presented with one year history of squamous lesions on the face and upper and lower limbs. The lesions were biopsied and histopatological examination revealed nonnecrotizing epitheloid granulomas and raised the suspicion of a sarcoid-like entity. CONCLUSION:The interest of this case will serve to better understand clinical manifestations in a rare genetic entity. Close follow-up is advised as cutaneous granulomas may be the first manifestation of systemic granulomas.
journal_name
Iran J Pediatrjournal_title
Iranian journal of pediatricsauthors
Liana RA,Dan G,Nicolae Msubject
Has Abstractpub_date
2013-02-01 00:00:00pages
100-4issue
1eissn
2008-2142issn
2008-2150journal_volume
23pub_type
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：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：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：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：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: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: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:Although there are several echocardiographic criteria, there is not yet a general consensus about the diagnosis of left ventricular noncompaction. The current criteria are mostly based on the areas with maximal noncompaction in the heart. The echocardiographer may miss this maximal point leading to a misdiagn...
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:Metals such as copper (Cu), zinc (Zn), iron (Fe) are essential for human beings. Chronic metabolic disturbances may result from an excess or deficiency of these metals. Ca and Mg are also nutrient elements and play an important role in biological systems. Thus, it is very important to check regularly trace el...
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: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: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：: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: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: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: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：: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: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 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: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: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：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: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：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: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: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：BACKGROUND:Respiratory distress syndrome (RDS) is one of the most common diseases in neonates admitted to NICU. For this important cause of morbidity and mortality in preterm neonates, several treatment methods have been used. To date, non-invasive methods are preferred due to fewer complications. OBJECTIVES:Herein, t...
journal_title：Iranian journal of pediatrics
pub_type： 杂志文章
doi：10.5812/ijp.2352
更新日期：2016-03-05 00:00:00
abstract：BACKGROUND:Acute lymphoblastic leukemia (ALL) is the most common malignancy diagnosed in children. Extramedullary leukemia including hepatosplenomegaly, lymphadenopathy, and mediastinal mass are the clinical presentations of T-cell ALL. Pericardial effusion and atrial mass are rare presentations of pediatric leukemia. ...
journal_title：Iranian journal of pediatrics
pub_type：
doi：
更新日期：2012-03-01 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: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: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
