Compliance and Lifestyle of Children with Celiac Disease in the Iranian Children.

Background: Celiac disease is popular and needs a proper and constant gluten-free diet. However, data on the experience of the disease by children are insufficient. A few children have difficulty adjusting their lifestyles, and gluten-free foods are difficult for them. The present study aimed to find influential factors in the growth disorders and nonresponse to the treatment diet in celiac patients. Materials and Methods: We gave a list of all children with celiac disease to the project manager and according to the criteria extracted additional information from their files. Duodenal biopsies on 382 patients with suspected celiac disease and 93 patients with positive pathology were included in the study, regardless of antibody and genetic titer, then analyzed their information using appropriate statistical tests. Results: The mean age of individuals was 9.48 ± 3.88, and 35 were male and 58 female. At the age of <5, there was more growth disorder than other age groups. The recovery percentage in short stature was significantly better in children with higher marches, and they responded better to the treatment regimen. Individuals with comorbidities had higher anti-tTG and lower Hb levels, higher incidence of growth disorder, did not respond to the treatment regimen. Those with a first-degree relative with celiac disease had a lower growth disorder than others. Conclusion: Identifying and correcting nutritional disorders in patients with celiac disease need to evaluate persistent symptoms and identify their causes to plan appropriate treatment and follow-up of patients with celiac disease step by step and continuously.

Clinical Phenotype and Disease Course of Inflammatory Bowel Disease in Iran: Results of the Iranian Registry of Crohn's and Colitis (IRCC).

BACKGROUND: Data on the epidemiology of inflammatory bowel disease (IBD) in the Middle East are scarce. We aimed to describe the clinical phenotype, disease course, and medication usage of IBD cases from Iran in the Middle East. METHODS: We conducted a cross-sectional study of registered IBD patients in the Iranian Registry of Crohn's and Colitis (IRCC) from 2017 until 2022. We collected information on demographic characteristics, past medical history, family history, disease extent and location, extra-intestinal manifestations, IBD medications, and activity using the IBD-control-8 questionnaire and the Manitoba IBD index, admissions history, history of colon cancer, and IBD-related surgeries. RESULTS: In total, 9746 patients with ulcerative colitis (UC) (n=7793), and Crohn's disease (CD) (n=1953) were reported. The UC to CD ratio was 3.99. The median age at diagnosis was 29.2 (IQR: 22.6,37.6) and 27.6 (IQR: 20.6,37.6) for patients with UC and CD, respectively. The male-to-female ratio was 1.28 in CD patients. A positive family history was observed in 17.9% of UC patients. The majority of UC patients had pancolitis (47%). Ileocolonic involvement was the most common type of involvement in CD patients (43.7%), and the prevalence of stricturing behavior was 4.6%. A prevalence of 0.3% was observed for colorectal cancer among patients with UC. Moreover,15.2% of UC patients and 38.4% of CD patients had been treated with anti-tumor necrosis factor (anti-TNF). CONCLUSION: In this national registry-based study, there are significant differences in some clinical phenotypes such as the prevalence of extra-intestinal manifestations and treatment strategies such as biological use in different geographical locations.

Six-minute walk test and factors affecting exercise capacity in children with cystic fibrosis.

PURPOSE: This study aimed to compare the result of the six-minute walk test (6MWT) in patients with cystic fibrosis (CF) aged < 20 years old and individuals without CF. METHODS: In this cross-sectional study, 50 children and adolescents with CF and 20 children and adolescents without CF underwent the 6MWT. Vital signs before and immediately after the 6MWT and six-minute walk distance (6MWD) were evaluated. RESULTS: The mean change in heart rate, percentage of peripheral oxygen saturation (SpO2%), systolic blood pressure, respiratory rate, and dyspnea severity during the 6MWT was significantly higher in patients with CF. In the case group, 6MWD was associated with regular chest physical therapy (CPT) and forced expiratory volume (FEV)> 80%. Patients with CF receiving regular CPT or mechanical vibration and with FEV in the first second > 80% showed better physical capacity during the 6MWT (smaller Sp02% decline and lower dyspnea perception). CONCLUSION: Children and adolescents with CF have lower physical capacity compared to individuals without CF. CPT and mechanical vibration could be used to increase physical capacity in this population.

The status of antimicrobial resistance of Helicobacter pylori in Eastern Azerbaijan, Iran: comparative study according to demographics.

Helicobacter pylori-associated infections are extremely common in Iran, but few data about antibiotic sensitivity of H. pylori are available for this region. The purpose of this study was to investigate the prevalence of resistance in isolates against commonly used antibiotics in Eastern Azerbaijan, Iran, and the dependence of prevalence on the sex and age of patients. H. pylori isolates were collected by culture from gastric biopsies. Antibiotic susceptibility of isolates was determined by use of the disk agar diffusion test, and the minimum inhibitory concentration of clarithromycin was established by use of the Etest. A total 395 of biopsy specimens were studied; 112 samples of H. pylori were isolated (28.3 %), 55 (49 %) from males and 57 (51 %) from females. The prevalence of resistance to clarithromycin, metronidazole, erythromycin, amoxicillin, ciprofloxacin, rifampin, nitrofurantoin, and tetracycline were 16 (14.3 %), 86 (76.8 %), 29 (26.0 %), 32 (28.6 %), 37 (33.0 %), 32 (28.6 %), 13 (11.6 %), and 21 (18.7 %), respectively. Antimicrobial resistance was not statistically significantly associated with sex or age. Furthermore, the prevalence of resistance to metronidazole was high and that to clarithromycin was reasonable, consistent with reported low success in H. pylori treatment in this area. Therefore, continuous surveillance of antibiotic resistance of H. pylori is essential.

Diagnostic Value of Fecal Calprotectin in Children with Gastritis, Duodenitis and Helicobacter Pylori.

Background: Fecal calprotectin (FC) is suggested as a novel biomarker for the diagnosis of gastrointestinal (GI) diseases; however, few studies have investigated its diagnostic value for Helicobacter pylori (H. pylori). Therefore, the current study evaluated the level of FC and its diagnostic value in patients with H. Pylori and its related conditions including gastritis and duodenitis. Methods: In this case-control study, 120 children with upper GI symptoms, who were indicated to undergo upper GI endoscopic examination, were consecutively included. Patients were categorized into different groups based on their endoscopic findings including H. pylori, gastritis, duodenitis or normal. Results: Patients with gastritis (P = 0.014) and those with duodenitis (P < 001) had significantly higher FC. The level of FC was higher in patients with H. pylori but this difference was marginally significant (P = 0.054). The level of FC had poor ability to diagnose the presence of H. pylori (P = 0.054) and gastritis (area under the curve, AUC = 0.639, P = 0.014). However, it had acceptable power to diagnose patients with or duodenitis (AUC = 0.718, P < 0.001). The sensitivity and specificity of FC for diagnosis of gastritis were 64 and 65 percent (cut-off = 45.2 µg/g), and for duodenitis were 77 and 61 percent (cut-off = 46.2 µg/g), respectively. Conclusions: FC can be considered as an objective and diagnostic tool for duodenitis. However, due to the low sensitivity and specificity, it is suggested to consider it as an objective supplementary test beside other established diagnostic modalities.

The effect of Helicobacter pylori treatment on remission of idiopathic central serous chorioretinopathy.

PURPOSE: The aim of this study was to evaluate the effect of Helicobacter pylori (H. pylori) treatment on remission of idiopathic central serous chorioretinopathy. METHODS: Twenty-five patients with idiopathic central serous chorioretinopathy (ICSCR) who were infected with H. pylori were treated with an anti-H. pylori treatment; another twenty-five patients with the same clinical presentations served as the control. Baseline examination and follow up visits at 2, 4, 6, 8, 12, and 16 weeks after the onset of treatment included visual acuity testing and subretinal fluid measurement. The difference between mean visual acuity at the end of 16 weeks and the time of subretinal fluid reabsorption was compared between the two groups. RESULTS: Subretinal fluid reabsorption time was 9.28±3.20 weeks in the treatment group and 11.63±3.18 weeks in the control group, which was statistically significant (p=0.015). After 16 weeks, mean visual acuity improved to 0.003±0.01 (logMAR) in the treatment group and 0.004±0.02 (logMAR) in the control group. This improvement did not represent a statistically significant difference (p=0.97). CONCLUSIONS: An anti-H. pylori treatment regimen is effective in the treatment of idiopathic central serous chorioretinopathy patients and anti-H. pylori treatment can provoke the faster reabsorption of subretinal fluid.

Use of oral midazolam in pediatric upper gastrointestinal endoscopy.

BACKGROUND: The purpose of this prospective, randomized study was to compare the safety and efficacy of oral versus i.v. midazolam in providing sedation for pediatric upper gastrointestinal (GI) endoscopy. METHODS: Sixty-one children (age <16 years) scheduled for upper GI endoscopy were studied. Patients were randomly assigned to receive oral or i.v. midazolam. Measurements were made and compared for vital signs, level of sedation, pre- and post-procedure comfort, anxiety during endoscopy, ease of separation from parents, ease and duration of procedure, and recovery time. RESULTS: Patients were aged 1-16 years (mean 7.5 + or - 3.42 years); 30 patients received oral medication, and 31 received i.v. medication. There were no statistically significant differences in age or gender between groups. There were no significant differences in level of sedation, ease of separation from parents, ease of ability to monitor the patient during the procedure, heart rate, systolic arterial pressure, or respiratory rate. Oxygen saturation was significantly lower in the i.v. group than the oral group 10 and 30 min after removal of the endoscope, and recovery time was longer in the oral than the i.v. group. CONCLUSIONS: Oral administration of midazolam is a safe and effective method of sedation that significantly reduces anxiety and improves overall tolerance for children undergoing esophagogastroduodenoscopy.

Bowel perforation by ventriculoperitoneal shunt catheter mimicking gastroenteritis.

Colon perforation is a rare and serious complication of ventriculoperitoneal shunt. The authors report a 7-month-old male infant with vomiting and watery diarrhea after colon perforation by a ventriculoperitoneal shunt. A minimal laparotomy incision was performed accurately where preoperative colonoscopy had detected the penetration site of shunting tube in the left colon.

Comparative evaluation of Cryptosporidium infection in malnourished and well-nourished children: Parasitic infections are affected by the interaction of nutritional status and socio-demographic characteristics.

Cryptosporidium, as a small protozoan parasite, is a leading cause of persistent diarrhea in children in developing countries and has both a short and long-term impact on the growth of children. In the present study, Cryptosporidium infection was compared in malnourished and well-nourished children by modified acid-fast staining, nested-polymerase chain reaction (nested-PCR) and loop-mediated isothermal amplification (LAMP) methods. As a case-control study, Cryptosporidium infection in 94 malnourished children was evaluated and compared with those of 188 age and gender-matched well-nourished children. Oocysts of Cryptosporidium were detected by modified acid-fast staining method. The extracted DNA was amplified by nested-PCR and LAMP techniques. In addition, positive amplicons were directly sequenced for phylogenetic analysis. Cryptosporidium oocysts were found in the stools of two (2.12 %) children who were hospitalized and had diarrhea by nested-PCR while three isolates (3.2 %) were found by LAMP. Cryptosporidium-positive children were more malnourished compared to those who were negative for Cryptosporidium infection but this important finding was not statistically significant. C. parvum was the main species of Cryptosporidium detected in malnourished children in northwest Iran. LAMP can be considered as a sensitive field monitoring assay in patients with low parasite burden. Nutritional status and socio-demographic factors may have interactive effects on the incidence and severity of parasitic diseases.

A multicentre development and evaluation of a dietetic referral score for nutritional risk in sick infants.

BACKGROUND & AIMS: Unrecognized nutritional issues may delay recovery in hospitalized infants. It has been proposed that nutritional risk screening should be performed at hospital admission, but few tools include infants. The aim of this study was to develop and test a tool to identify sick infants in need of dietetic input. METHODS: Hospitalised infants were recruited from hospitals in the United Kingdom (UK), Greece and Iran. Weight, skinfold thickness and mid upper arm circumference (MUAC) were measured, with detailed dietetic assessment in the UK and Greece. Simple screening questions were used in the UK cohort to formulate a score (infant early nutrition warning score-iNEWS) which was then validated in the Greek and Iranian groups. RESULTS: After dietetic assessment, 20 (9.6%) UK and 22 (22%) Greek infants were rated as needing dietetic input. Underweight, poor weight gain/loss and reduced intake were all independent predictors of perceived need for dietetic input in stepwise multivariate regression analysis. The score based on these items (iNEWS), had 84% sensitivity, 91% specificity and 49% positive predictive value to predict need for dietetic input in the UK cohort. In the Greek cohort this was 86%, 78% and 53% respectively. In all three countries, infants with high iNEWS had significantly lower average skinfold thickness (between -1 and -1.8 SD, p < 0.0001) and MUAC (between -1.8 and -2 SD, p < 0.0001) than those at low risk. CONCLUSIONS: iNEWS, a simple nutritional risk tool, identifies most hospitalised infants who need dietetic input. CLINICAL TRIAL REGISTRATION: https://clinicaltrials.gov/ct2/show/NCT03323957.

Lipoprotein(a), homocysteine, and retinal arteriosclerosis.

PURPOSE: Elevated levels of lipoprotein(a) [Lp(a)] and homocysteine (Hcy) have been implicated as risk factors for vascular diseases. The study was performed to explore the possible relationship between retinal arteriosclerosis and serum Lp(a) and Hcy levels. METHODS: Study subjects consisted of 80 nonsmoking male patients with retinal arteriosclerosis and 54 healthy nonsmoker males as controls. Retinal arteriosclerosis was graded according to the Scheie classification. Serum levels of lipids, lipoproteins, Lp(a), and Hcy were measured by standard methods. RESULTS: The serum level of Hcy was higher in patients (24.2+/-8.1 micromol/l) than controls (10.5+/-4.1 micromol/l); p<0.01. Serum levels of Lp(a) in patients (47.9+/-33.1 mg/dl) was also higher than controls (11.7+/-7.6 mg/dl); p<0.01. There was a significant direct linear correlation between the degree of retinal arteriosclerosis and Lp(a) level (r=0.61, p<0.01), the degree of retinal arteriosclerosis and Hcy level (r=0.72, p<0.01), and also between Lp(a) and Hcy levels (r=0.67, p<0.01). CONCLUSIONS: The association between retinal arteriosclerosis and serum Lp(a) and Hcy levels suggests that Lp(a) as well as Hcy could play a role in the development of retinal arteriosclerosis.

Common MEFV mutation analysis in Iranian Azeri Turkish patients with familial Mediterranean fever.

OBJECTIVES: To identify the frequency and distribution of familial Mediterranean fever (FMF) gene (MEFV) mutations among Azeri Turkish patients from northwestern Iran. METHODS: One hundred ninety unrelated patients were referred by specialists to the Molecular-Medical Genetic Center of Tabriz. A clinical diagnosis of FMF was made according to published criteria. Mutation screening of the MEFV gene was performed for the 5 most commonly known mutations, namely M694V, V726A, M680I, M694I, and E148Q, by using amplification refractory mutation system for the first 4 and by polymerase chain reaction restriction-digestion testing for E148Q. These methods may also be used as a screening tool within affected families. RESULTS: Of the unrelated patients investigated, 120 (63%) had 1 or 2 mutations. Of those with mutations, 41 were homozygous, 37 were compound heterozygous, and 42 had only 1 identifiable mutation. Of the studied alleles, the most frequent mutation was M694V (28%), followed by V726A (9%), E148Q (7%), M680I (7%), and M694I (1%) mutations. CONCLUSIONS: Our results indicate that the common Mediterranean mutations are frequent in the Azeri Turkish FMF patients but with some differences in the frequency of individual mutations. The high frequency of E148Q in Azeri Turks compared with Mediterranean ethnic groups is rather interesting. The results open the way for further investigations on patients diagnosed as having FMF and in whom no mutations or only 1 mutated allele were found.

Defining a Core Data Set for Registry of Esophageal Atresia in the Northwest of Iran.

Defining the core data set is the main step for establishing a registry system. The aim of this study was to define the core data set for the registry of esophageal atresia in the northwest of Iran. METHODS: First, the preliminary list of data elements was extracted from the related registries of other countries, as well as from the literature. Then, a group of multidisciplinary experts was asked to score the tabulated list of data elements in terms of their importance using a 5-point Likert scale through a dual-round Delphi technique. Availability of data was assessed through a medical record review of 410 patients with esophageal atresia who had been hospitalized between March 2006 and March 2016 in Tabriz Children's Hospital. RESULTS: The main classes of data were defined, including maternal information, patient demographics, clinical information, complications, and follow-up data. Thirty-two of 51 data elements (the core data elements) had 100% availability. Demographic data were completely available for 60% of the data elements. For clinical data, the availability rate was above 75%, while for complications and follow-up, it was 100% (except for the weight and height). In the category of maternal data, no data was available on the genetic screening and amniocentesis. CONCLUSION: This study presents the core data set required for establishing an esophageal atresia registry in the northwest of Iran. A considerable number of identified cases and high availability of patient data indicated the feasibility of establishing the first esophageal atresia registry in the area.

Autoimmune hepatitis in Iranian children.

OBJECTIVE: To evaluate the clinical and para-clinical presentation, including autoantibody pattern and response to treatment in Iranian children with autoimmune hepatitis (AIH). METHODS: Data of 60 children presenting with AIH (56 girls) were analyzed retrospectively for clinical, serological and histological profile. RESULTS: Clinical findings included jaundice (67%),hepatomegaly (50%) and ascites (30%). Forty children (38 girls) had type I AIH and 14 (12 girls) had type II AIH. Liver histology was comparable in the two groups and cirrhosis on initial biopsy was equally frequent in Types I and II AIH (63% vs 34%; p=ns). CONCLUSION: Our study describes the presentation and clinical course of Iranian children with AIH. Treatment with corticosteroids and/or azathioprine induces remission of autoimmune hepatitis clinically, biochemically as well as histologically.

Portal venous thrombosis after umbilical vein catheterization.

PURPOSE: Portal vein thrombosis has been associated with umbilical venous catheterization. This prospective study was done to determine the incidence of neonatal portal venous thrombosis associated with catheterization of the umbilical vein . METHODS: Neonates who had undergone umbilical vein catheterization for exchange transfusion between March 2003 and March 2004 in Children's Hospital of Tabriz, Iran, were included. Doppler ultrasonography was performed within 1-2 weeks after the removal of the catheter. In the cases with portal venous thrombosis, subsequent serial ultrasonography was performed at intervals of every 1-2 months until clot resolution. Risk factors, if any were identified and correlated with catheter-related thrombi. RESULTS: Ultrasonography detected clinically silent portal venous thrombosis in 17 (34%) of 50 neonates. Follow-up ultrasonography was available in 13 of 17 babies, and revealed complete or partial resolution in all the cases. Sepsis was identified as a significant risk factor (p < 0.001). CONCLUSION: Umbilical venous catheter-associated thrombosis is common, and spontaneous resolution occurs in most cases.

Association of ACE, VEGF and CCL2 gene polymorphisms with Henoch-Schönlein purpura and an evaluation of the possible interaction effects of these loci in HSP patients.

BACKGROUND: Henoch-Schönlein purpura (HSP) is a multisystem, small vessel, leucocytoclastic vasculitis. It is predominantly a childhood vasculitis, rarely reported in adults. Studies have shown that several different genetic factors such as genes involved in inflammatory system and renin-angiotensin system (RAS) are important in the pathogenesis of Henoch-Schönlein purpura. OBJECTIVES: The purpose of this study was to evaluate the independent effect of 3 gene polymorphisms including CCL2-2518 C/T, VEGF-634G/C and ACE(I/D) with HSP disease and their possible joint interactions in developing the disease. MATERIAL AND METHODS: In this case-control study 47 HSP cases and 74 unrelated healthy controls were enrolled for evaluation. All individuals were genotyped for CCL2-2518C/T, VEGF-634G/C and ACE(I/D) gene polymorphisms. The possible association of these polymorphisms with susceptibility to develop HSP disease independently and in different joint combinations was evaluated. RESULTS: The frequencies of TT genotype and T allele of CCL2-2518C/T gene polymorphism and CC genotype and C allele of VEGF-634G/C gene polymorphism were significantly high in HSP children (p-values = 0.005 and = 0.007 respectively). Interestingly, studying the joint interaction of these 2 genotypes (CC genotype of VEGF G-634C and TT genotype of CCL2 C-2518T) in this cohort showed a more significant effect in the development of the disease (p < 0.000, OR = 6.009). The frequency of TT genotype of CCL2 gene when combined with II genotype of ACE gene in HSP children was significantly higher (p < 0.000, OR = 4.213). CONCLUSIONS: The results of this pilot study provide evidence of the possible gene-gene interaction effects of CCL2, VEGF and ACE genes in developing HSP disease.

Molecular analysis of glycogen storage disease type Ia in Iranian Azeri Turks: identification of a novel mutation.

Glycogen storage diseases (GSDs) are caused by abnormalities in enzymes that are involved in the regulation of gluconeogenesis and glycogenolysis. GSD I, an autosomal recessive metabolic disorder, is the most common GSD and has four subtypes. Here, we examined GSD Ia caused by the defective glucose-6-phosphatase catalytic (G6PC) gene. We investigated the frequency of GSD Ia and clarified its molecular aspect in patients with the main clinical and biochemical characteristics of GSD, including 37 unrelated patients with a mean age of three years at the time of diagnosis. All patients belonged to the Azeri Turkish population. Hypoglycaemia and hypertriglyceridaemia were the most frequent laboratory findings. Mutations were detected by performing direct sequencing. Mutation analysis of the G6PC gene revealed that GSD Ia accounted for 11% in GSD patients with involvement of liver. Three patients were homozygous for R83C mutation. In addition, a novel stop mutation, Y85X, was identified in a patient with the typical features of GSD Ia.

Efficacy of proton pump inhibitors and H2 blocker in the treatment of symptomatic gastroesophageal reflux disease in infants.

PURPOSE: Gastroesophageal reflux disease (GERD) occurs in pediatric patients when reflux of gastric contents presents with troublesome symptoms. The present study compared the effects of omeprazole and ranitidine for the treatment of symptomatic GERD in infants of 2-12 months. METHODS: This study was a clinical randomized double-blind trial and parallel-group comparison of omeprazole and ranitidine performed at Children Training Hospital in Tabriz, Iran. Patients received a standard treatment for 2 weeks. After 2 weeks, the patients with persistent symptoms were enrolled in this randomized study. RESULTS: We enrolled 76 patients in the present study and excluded 16 patients. Thirty patients each were included in group A (ranitidine) and in group B (omeprazole). GERD symptom score for groups A and B was 47.17±5.62 and 51.93±5.42, respectively, with a P value of 0.54, before the treatment and 2.47±0.58 and 2.43±1.15, respectively, after the treatment (P=0.98). No statistically significant differences were found between ranitidine and omeprazole in their efficacy for the treatment of GERD. CONCLUSION: The safety and efficacy of ranitidine and omeprazole have been demonstrated in infants. Both groups of infants showed a statistically significant decrease in the score of clinical variables after the treatment.

Topical tranexamic acid as a novel treatment for bleeding peptic ulcer: A randomised controlled trial.

BACKGROUND: Peptic ulcers are among the most common causes of upper gastrointestinal (GI) bleeding in children. The standard care for GI bleeding is endoscopy for diagnostic and therapeutic purposes. We aimed to assess the effect of topical tranexamic acid (TXA) via endoscopic procedures in children with GI bleeding caused by bleeding ulcers. PROCEDURE: In this randomised controlled trial, 120 children were evaluated by diagnostic procedures for GI bleeding, of which 63 (30 girls, 33 boys) aged 1-month to 15 years were recruited. The patients were randomly divided into case and control groups. In the case group, TXA was administered directly under endoscopic therapy. In the control group, epinephrine (1/10,000) was submucosally injected to the four quadrants of ulcer margins as the routine endoscopic therapy. In both groups, the patients received supportive medical therapy with intravenous fluids and proton pump inhibitor drugs. RESULTS: The mean ± standard deviation age of the children was 5 ± 2.03 years. Rebleeding occurred in 15 (11.4%) and 21 (9.8%) patients in the case and control groups, respectively (P = 0.50). The frequency of blood transfusion episodes (P = 0.06) and duration of hospital stay (P = 0.07) were not statistically different between the groups. CONCLUSION: Using topical TXA via endoscopic procedures may be effective in cases of GI bleedings caused by active bleeding ulcers. In order to establish this therapeutic effect, a large number of clinical studies are needed.

Diagnostic value of anti-smooth muscle antibodies and liver enzymes in differentiation of extrahepatic biliary atresia and idiopathic neonatal hepatitis.

BACKGROUND: We aimed to evaluate the diagnostic value of anti-smooth muscle antibodies (ASMA) and two liver markers (gamma-glutamyl transpeptidase [GGT] and alkaline phosphatase [ALP]) for differentiating between patients with extrahepatic biliary atresia (EHBA) and idiopathic neonatal hepatitis (INH). MATERIALS AND METHODS: During April 2010-2011, all infants at 2 weeks of age who were diagnosed with cholestasis and admitted to Children's Hospital of Tabriz were enrolled. Based on the results of physical examination, laboratory, imaging and pathological studies, neonates were divided into two groups (EHBA and INH). Receiver operating characteristics analysis was used to define sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy for ASMA, GGT and ALP. RESULTS: Thirty neonates with cholestasis (18 with EHBA and 12 with INH) and mean age of 54.66 ΁ 25.86 days were enrolled. Total and direct bilirubin, serum glutamic oxaloacetic transaminase, serum glutamic pyruvic transaminase and ASMA titres were highly not significant (P > 0.05) in patients with INH. GGT (P = 0.008) and ALP (P = 0.01) had statistically significant differences that were higher in patients with EHBA. The sensitivity, specificity, PPV and NPV, accuracy, LR+ and LR- of SMA in differentiating cases with BA were 66.7%, 75%, 80% 60%, 70%, 2.68 and 0.44, respectively. For GGT, the values were 88.9%, 66.7%, 80%, 80%, 79.1%, 3.08 and 0.31, respectively. Finally, for ALP, the values were 77.8%, 75%, 82.4%, 69.2%, 80%, 2.66 and 0.24, respectively. CONCLUSION: Our study showed that ASMA may be a useful biomarker for differentiation of EHBA from INH. Further studies with larger samples are recommended for confirming the results of this study.