Publications by authors named "Mahmood Haghighat"

56 Publications

Clinical and genetic spectrum of glycogen storage disease in Iranian population using targeted gene sequencing.

Sci Rep 2021 Mar 29;11(1):7040. Epub 2021 Mar 29.

Student Research Committee, Shiraz University of Medical Sciences, Shiraz, Iran.

Glycogen storage diseases (GSDs) are known as complex disorders with overlapping manifestations. These features also preclude a specific clinical diagnosis, requiring more accurate paraclinical tests. To evaluate the patients with particular diagnosis features characterizing GSD, an observational retrospective case study was designed by performing a targeted gene sequencing (TGS) for accurate subtyping. A total of the 15 pediatric patients were admitted to our hospital and referred for molecular genetic testing using TGS. Eight genes namely SLC37A4, AGL, GBE1, PYGL, PHKB, PGAM2, and PRKAG2 were detected to be responsible for the onset of the clinical symptoms. A total number of 15 variants were identified i.e. mostly loss-of-function (LoF) variants, of which 10 variants were novel. Finally, diagnosis of GSD types Ib, III, IV, VI, IXb, IXc, X, and GSD of the heart, lethal congenital was made in 13 out of the 14 patients. Notably, GSD-IX and GSD of the heart-lethal congenital (i.e. PRKAG2 deficiency) patients have been reported in Iran for the first time which shown the development of liver cirrhosis with novel variants. These results showed that TGS, in combination with clinical, biochemical, and pathological hallmarks, could provide accurate and high-throughput results for diagnosing and sub-typing GSD and related diseases.
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http://dx.doi.org/10.1038/s41598-021-86338-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC8007705PMC
March 2021

Percutaneous endoscopic gastrostomy in children: a single center study at Tertiary hospital Iran.

Rev Gastroenterol Peru 2018 Apr-Jun;38(2):125-127

Gastroenterohepatology Research Center, Shiraz University of Medical Sciences. Shiraz, Iran.

Introduction: The aim of this study was to evaluate complications after percutaneous endoscopic gastrostomy among children who underwent percutaneous endoscopic gastrostomy in Nemazee hospital.

Materials And Methods: All children who underwent percutaneous endoscopic gastrostomy were included in the current study. Place of the study was department of pediatric gastroenterology of Nemazee children hospital of Shiraz university of medical sciences. Duration of the study was 5 year starting from 2008. All drugs such as aspirin, NSAIDS, and heparin were discontinued 1-7 days before procedures. All patients were kept NPO 6-8 hours before procedure according to the age. Single dose antibiotic was prescribed for all cases before procedure. During procedure, all patients were sedated using propofol and or midazolam. Some patients required intubation.

Results: Of 39 cases who underwent PEG, 4 (10.2%) patients showed complication. The most common indication for PEG insertion were neurologic problem (84.6%) and metabolic disease (10.2%). Of our patients, 84.6% of the cases had the weight below third percentile.

Conclusion: The most common indication for percutaneous endoscopic gastrostomy was cerebral palsy. The complication rate in our study was 10.2%. Celulitis was the most common complication.
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December 2018

Caustic ingestion in children in south of Iran. Retrospective study from Shiraz - Iran.

Rev Gastroenterol Peru 2017 Jan-Mar;37(1):22-25

Gastroenterohepatology Research Center, Nemazee Teaching Hospital,, Shiraz University of Medical Sciences. Shiraz, Iran.

Caustic ingestion is a major health concern in both developed and developing countries, that may lead to serious esophageal injury. The clinical presentation of caustic ingestion in children vary from asymptomatic to serious and fatal sequelae, such as perforation and stricture formation.

Objective: Due to the lack of a comprehensive study in our area, this study has evaluated clinical and endoscopic manifestations and complications of caustic ingestion in children in south of Iran.

Materials And Methods: In this retrospective study, we reviewed 75 children with caustic ingestion who admitted in Nemazee Hospital of Shiraz University of Medical Science during 6 years (2006-2011). Sign and symptoms were recorded for each case.

Results: The most common symptoms were dysphagia, oral lesions, vomiting, and drooling. Esophageal injuries were detected in both acid and alkali ingestion, but gastric injuries was significantly more in acid ingestion. During follow up period, 20% of all cases developed esophageal stricture.

Conclusion: Dysphagia, oral lesions, vomiting, and drooling were the most common findings.Esophageal stricture was found in 20% of cases during 3 months of follow up.
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April 2018

Perforated Meckel's Diverticulum in a 3-day-old Neonate; A Case Report.

Middle East J Dig Dis 2016 Oct;8(4):323-326

Assistant Professor of Pediatric Gastroenterology, Abuzar Children's Hospital, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran.

Perforation of Meckel's diverticulum is a rare complication in neonatal period. A 3-dayold term male neonate was transferred to our emergency room due to bowel perforation. Surgical exploration was done and perforated Meckel's diverticulum was detected. Pathological report of the tissue showed inflamed diverticulum with heterotopic gastric mucosa. This is the first report of Meckel's diverticulum perforation in a neonate in our country.
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http://dx.doi.org/10.15171/mejdd.2016.43DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5145301PMC
October 2016

Solitary rectal ulcer syndrome: Is it really a rare condition in children?

World J Clin Pediatr 2016 Aug 8;5(3):343-8. Epub 2016 Aug 8.

Seyed Mohsen Dehghani, Maryam Bahmanyar, Bita Geramizadeh, Anahita Alizadeh, Mahmood Haghighat, Department of Pediatric Gastroenterology, Gastroenterohepatology Research Center, Shiraz Transplant Research Center, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz 71937-11351, Iran.

Aim: To evaluate the clinicopathologic characteristics of the children with solitary rectal ulcer.

Methods: Fifty-five children with a confirmed diagnosis of solitary rectal ulcer were studied in a period of 11 years from March 2003 to March 2014. All data were collected from the patients, their parents and medical records in the hospital.

Results: From 55 studied patients, 41 were male (74.5%) and 14 female (25.5%). The mean age of the patients was 10.4 ± 3.7 years and the average time period from the beginning of symptoms to diagnosis of solitary rectal ulcer was 15.5 ± 11.2 mo. The most common clinical symptoms in our patients were rectal bleeding (n = 54, 98.2%) and straining during defecation or forceful defecation (n = 50, 90.9%). Other symptoms were as follows respectively: Sense of incomplete evacuation (n = 34, 61.8%), mucorrhea (n = 29, 52.7%), constipation (n = 14, 25.4%), tenesmus and cramping (n = 10, 18.2%), diarrhea (n = 9, 16.4%), and rectal pain (n = 5, 9.1%). The colonoscopic examination revealed 67.3% ulcer, 12.7% polypoid lesions, 10.9% erythema, 7.3% both polypoid lesions and ulcer, and 1.8% normal. Most of the lesions were in the rectosigmoid area at a distance of 4-6 cm from the anal margin. Finally, 69.8% of the patients recovered successfully with conservative, medical and surgical management.

Conclusion: The study revealed that solitary rectal ulcer is not so uncommon despite what was seen in previous studies. As the most common symptom was rectal bleeding, clinicians and pathologists should be familiar with this disorder and common symptoms in order to prevent its complications with early diagnosis.
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http://dx.doi.org/10.5409/wjcp.v5.i3.343DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4978629PMC
August 2016

DIAGNOSTIC ACCURACY OF UREA BREATH TEST FOR HELICOBACTER PYLORI INFECTION IN CHILDREN WITH DYSPEPSIA IN COMPARISON TO HISTOPATHOLOGY.

Arq Gastroenterol 2016 Apr-Jun;53(2):108-12

Chronic Disease Care Research Center and Department of Pediatric Gastroenterology, Abuzar Children's Hospital, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, Iran., Ahvaz Jundishapur University of Medical Sciences, Chronic Disease Care Research Center, Abuzar Children's Hospital, Ahvaz Jundishapur University of Medical Sciences, Ahvaz , Iran.

Background: Helicobacter pylori infection is the gram negative bacillus with the close association with chronic antral gastritis.

Objective: In this study, we evaluate the accuracy of urea breath test (UBT) with carbon isotope 13 in comparison with histopathology of gastric antrum for detection of H. pylori infection in children with dyspepsia.

Methods: This cross-sectional study was performed at specialized laboratory of Shiraz Gastroenterohepatology Research Center and Nemazee Hospital, Iran, during a 12-months period. This study investigated the sensitivity, specificity, and positive and negative predictive values of UBT in comparison with biopsy-based tests. We included a consecutive selection of 60 children who fulfilled Rome III criteria for dyspepsia. All children were referred for performing UBT with carbon isotope 13 (C13) as well as endoscopy. Biopsies were taken from antrum of stomach and duodenum. The pathologic diagnosis was considered as the standard test.

Results: The mean age of the participants was 10.1±2.6 (range 7-17 years). From our total 60 patients, 28 (46.7%) had positive UBT results and 32 (53.3%) had negative UBT results. Pathologic report of 16 (57.1%) out of 28 patients who had positive UBT were positive for H. pylori and 12 (42.9%) ones were negative. Sensitivity and specificity of C13-UBT for detection of H. pylori infection were 76.2% and 69.2% respectively.

Conclusion: Sensitivity and specificity of C13-UBT for detection of H. pylori infection were 76.2% and 69.2% respectively. Another multicenter study from our country is recommended.
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http://dx.doi.org/10.1590/S0004-28032016000200011DOI Listing
February 2017

Evaluation of the Correlation Between tTG-IgA Titer and Duodenal Biopsy Findings in Children With Suspected Celiac Disease.

Iran J Pediatr 2016 Feb 30;26(1):e3615. Epub 2016 Jan 30.

Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, IR Iran.

Background: Celiac disease is an immune-mediated inflammation of the small intestine caused by sensitivity to dietary gluten in genetically sensitive individuals.

Objectives: In this study, we aimed to evaluate the predictive value of tissue transglutaminase (tTG) antibodies for the diagnosis of celiac disease in a pediatric population in order to determine if duodenal biopsy can be avoided.

Patients And Methods: The subjects were selected among individuals with probable celiac disease, referring to a gastrointestinal clinic. After physical examinations and performing tissue transglutaminase-immunoglobulin A (tTG-IgA) tests, upper endoscopy was performed if serological titer was higher than 18 IU/mL. Therapy started according to pathologic results.

Results: The sample size was calculated to be 121 subjects (69 female and 52 male subjects); the average age of subjects was 8.4 years. A significant association was found between serological titer and pathologic results; in other words, subjects with high serological titer had more positive pathologic results for celiac disease, compared to others (P < 0.001). Maximum sensitivity (65%) and specificity (65.4%) were achieved at a serological titer of 81.95 IU/ml; the calculated accuracy was lower in comparison with other studies. As the results indicated, lower antibody titer was observed in patients with failure to gain weight and higher antibody titer was reported in diabetic patients.

Conclusions: As the results indicated, a single serological test (tTg-IgA test) was not sufficient for avoiding intestinal biopsy.
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http://dx.doi.org/10.5812/ijp.3615DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4733291PMC
February 2016

BOWEL PREPARATION BEFORE COLONOSCOPY FOR CHILDREN: comparison of efficacy of three different methods.

Arq Gastroenterol 2015 Dec;52(4):311-4

Medical School, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Colonoscopy is an important diagnostic and therapeutic procedure. Adequate bowel preparation is mandatory. Several regimens were discussed in the literature. Among the drugs which has recently used, polyethylene glycol is one of the most popular agents.

Objectives: The aim of this study was to compare efficacy of three different methods for 1 day preparation before colonoscopy.

Methods: This study included children with the range of ages (2-21) who had an indication of colonoscopy. Exclusion criteria were based on the history of previous surgery, parental disagreement, and patients who did not use preparation protocol. Three methods for bowel preparation were studied: 1- Polyethylene glycol only; 2- Polyethylene glycol and bisacodyl suppositories; 3- Polyethylene glycol plus normal saline enema. Boston Bowel Preparation Score was used for evaluation of preparation. SPSS version 16.0 (Chicago, IL, USA) were used for data analysis.

Results: In this study 83 cases completed the bowel preparation completely. Acceptable bowel preparation was seen in 24 (85.71%), 36 (94.73%), and 14 (82.35%) of cases in PEG, PEG + bisacodyl, and PEG + normal saline enema groups respectively. PEG + bisacodyl suppositories was more effective than PEG + normal saline for the preparation of the first segment ( P=0.05). For second and third segment of colon, BPPS score was higher in PEG + bisacodyl suppositories compared to other regimens, but this difference was not statistically significant.

Conclusion: There was no significant difference between 1 day colonoscopy regimens in terms of bowel preparation score. Lowest score was seen in PEG + enema group compared to other group.
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http://dx.doi.org/10.1590/S0004-28032015000400011DOI Listing
December 2015

Complications of blind versus ultrasound-guided percutaneous liver biopsy in children.

Arab J Gastroenterol 2015 Sep-Dec;16(3-4):90-3. Epub 2015 Oct 30.

Gastroenterohepatology Research Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran. Electronic address:

Background And Study Aims: Liver biopsy is a well-established procedure in the diagnosis and follow-up of liver diseases. Complications of liver biopsy are rare but potentially lethal. The aim of this study was to evaluate the complications of percutaneous liver biopsy and to compare the complications of blind and ultrasound-guided percutaneous liver biopsy in paediatric wards of Nemazee Hospital of Shiraz in the south of Iran.

Patient And Method: To complete the questionnaire, registered information of liver biopsies due to different causes in paediatric patients between 2008 and 2012 was retrospectively reviewed. All children aged between 0 and 18years, who underwent liver biopsy (due to any indication), participated in this study.

Results: Liver biopsies were obtained from 210 patients. Seven of 210 cases were excluded due to unreliable data. A total of 209 liver biopsies were done in the rest of the cases (n=203). Of all cases of liver biopsies, 22 (10.5%) experienced complications after biopsy. Pain (n=7) was the most frequent complication in 22 cases of liver biopsy. Mortality rate was one (0.5%) due to rupture of subcapsular haematoma. In terms of complication (p=0.592), there was no significant difference statistically between patients with blind liver biopsy (n=16) and patients with ultrasound-guided liver biopsy (n=6).

Conclusion: In terms of complications, there was no significant difference when the patients were evaluated with and without ultrasound-guided biopsy.
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http://dx.doi.org/10.1016/j.ajg.2015.09.009DOI Listing
September 2016

Combination of erythromycin and propranolol for treatment of childhood cyclic vomiting syndrome: a novel regimen.

Gastroenterol Hepatol Bed Bench 2015 ;8(4):270-7

Children and Adolescents Health Research Center, Zahedan University of Medical Sciences, Zahedan, Iran.

Aim: This study aimed to evaluate the erythromycin efficacy in childhood cyclic vomiting syndrome.

Background: Cyclic vomiting syndrome (CVS) is an unusual cause of episodic emesis in children and erythromycin is an effective treatment.

Patients And Methods: In this prospective study, 301 patients with a final diagnosis of CVS enrolled in two separated groups. The first group received erythromycin for 7 days and propranolol for at least 9 months (n=155). The second group was treated with propranolol alone for at least 9 months (n=146). These two groups were compared for response to the treatment and the recurrence of symptoms after treatment completion. Relationship of response, recurrence, and characteristics of the disease was assessed.

Results: Both groups showed a significant difference in terms of response to treatment (P=0.002), however the recurrence after treatment completion had no considerable difference (P=0.563). There was no relationship between CVS characteristics and these two items (response and recurrence).

Conclusion: In our point of view, the addition of erythromycin to standard propranolol treatment can improve the response to treatment, although it has no significant effect on recurrence of CVS symptoms. We suggest the use of erythromycin for 7 days in addition to CVS standard therapy.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4600517PMC
October 2015

Serological Evaluation of Celiac Disease in Children with Congenital Heart Defect; A Case Control Study.

Middle East J Dig Dis 2015 Apr;7(2):98-103

Fellow of Pediatric Gastroenterology, Department of Pediatric Gastroenterology, Shiraz University of Medical Sciences, Shiraz, Iran.

BACKGROUND Due to the increased prevalence of celiac disease in chromosomal anomalies and other congenital anomalies, this study was conducted to evaluate the seroprevalence of celiac disease (CD) in patients with congenital heart defects (CHD). METHODS This case-control study was done on 1002 children in two groups of CHD patients (n=402) and controls (n=600). The serum tissue transglutamianse (TTG) levels were investigated. The two groups were compared in terms of TTG IgA levels and p<0.05 was considered as the significant level. RESULTS The means of serum TTG IgA levels in children with CHD and the control groups were 19.17±46.67 and 7.77±10.02 u/mL respectively (p=0.001). After ANOVA analysis a significant difference between two cyanotic and acyanotic subgroups of cases and control groups was observed (p=0.000). The follow up tukey test showed only non-significant difference between the cyanotic and acyanotic cases. The frequency of TTG IgA with the consideration of 20 u/mL as cut-off point showed a significant association with groups (X2=28.31 and p=0.000). CONCLUSION According to the results the serum TTG IgA levels were significantly higher in patients with CHD than normal children and screening for CD in children with CHD is recommended.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4430799PMC
April 2015

Serologic evaluation of celiac disease in patients with beta thalassemia major and control.

Gastroenterol Hepatol Bed Bench 2015 ;8(2):153-9

Children and Adolescents Health Research Center, Zahedan Medical University, Zahedan, Iran.

Aim: This study reports evaluated prevalence of CD in patients with Beta-thalassemia major.

Background: Celiac Disease (CD) is an autoimmune disorder triggered by ingestion of gluten in genetically predisposed individuals.

Patients And Methods: In this case-control study in a period of 3 years, which was performed on 620 children in two groups of Beta-thalassemia major patients (n=200) and control (n=420), serum tissue transglutamianse (tTG) IgA levels were measured. The two groups were compared together in terms of tTG IgA levels, and p<0.05 was considered significant.

Results: The means of serum tTG IgA levels in patients with Beta-thalassemia major and control groups were 28.81±68.44 and 6.94±6.68 U/mL, respectively. There was a significant difference in favor of the case group (p=0.000). Body mass index in the two case and control groups had a significant difference (t=3.859, p=0.001). Belonging to each group will change the probability of having less than 20 in tTG IgA (odds=0.285) and it means that belonging to the control group has a protective role. There is only a significant association in the case of all population (r=0.102, p=0.011). Body mass index in the two case and control groups had a significant difference (t=3.859, p=0.001).

Conclusion: Probability of CD should be considered since the prevalence of CD is high in patients with and Beta-thalassemia major. Patients with thalassemia major are recommended for screening for CD.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4403028PMC
April 2015

Evaluation of immunity status to routine vaccination in pediatric liver transplant candidates.

Turk J Gastroenterol 2014 Dec;25 Suppl 1:26-31

Shiraz Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, Islamic Republic of Iran; Gastroenterohepatology Research Center, Shiraz University of Medical Sciences, Shiraz, Islamic Republic of Iran.

Background/aims: Generally, prevention of infections by vaccination is the least invasive and most cost-effective approach to reduce the incidence of infections and the morbidity and mortality in transplant recipients. Genetic diversity and different liver disease among patients contributes to variability in immune responses to vaccines and pathogens. The aim of this study was to evaluate immunity status to different vaccinated organisms in pediatric liver-transplant candidates.

Materials And Methods: The vaccination charts of 90 patients who were referred to Organ Transplant Center of Shiraz University of Medical Sciences were reviewed and compare with National Immunization Program recommendation, after that 10 mL blood was drawn from these patients for serologic studies by ELISA.

Results: Eighty percent of the patients had protective antibody titers for poliomyelitis, 65.6% for rubella, 62.3% for diphtheria, 60% for tetanus, 57.7% for pertussis, 55.5% for measles, 42.2% for hepatitis B and 36.7% for mumps.

Conclusion: Overall seroconversion rates were not satisfactory for many infections that may be due to lower rate of vaccination or even the underlying liver disease that interfere with optimal immunogenecity of vaccination. Therefore, vaccination charts should be periodically reviewed and updated, also repeated measurements of serum antibodies and appropriate revaccination if titers decline is recommended to prevent the vaccine-preventable disease in liver transplant candidates after transplant.
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http://dx.doi.org/10.5152/tjg.2014.5139DOI Listing
December 2014

Clinical Manifestations among Children with Chronic Functional Constipation.

Middle East J Dig Dis 2015 Jan;7(1):31-5

4. Fellow of Pediatric Gastroenterology, Gastroenterohepatology Reseach Center, Nemazee Teaching Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

BACKGROUND Constipation is one of the most frequent cause of patient visits to pediatric gastroenterology clinics. Early diagnosis and treatment is important. There are few studies about clinical manifestations of constipation in children. We aimed to find the relative frequency of gastrointestinal manifestations of constipation among constipated children. METHODS This cross-sectional study was carried out on children aged < 18 years old with chronic functional constipation referred to Imam Reza Clinic of Shiraz University of Medical Sciences. Children with organic causes of chronic constipation were excluded from study. Rome III criteria were used for defining constipation. The duration of study was 1 year starting from September 2010. Abdominal pain, fecal mass, rectal bleeding, anorexia, fecal soiling, retentive posture, withholding behavior, anal fissure, and peri-anal erythema were recorded for each case based on history and physical examination. Data were analyzed using SPSS software, version 13.0 (Chicago, IL, USA). RESULTS Of 222 children with functional constipation, 124(55.9%) were girls and 98 (44.1%) were boys with a mean ± SD age of 5±3.12 years. The mean ± SD duration of constipation was 2.2±1.9 years. Large and hard stool was present in 93.7% of the patients. Painful defecation and withholding behavior were seen in 92.3% and 91.9% of the patients, respectively. Fecal impaction was more frequent among boys compared with girls (p<0.01). Fecal soiling was present in 40.8% of the boys and 28.2% of the girls (p=0.04). CONCLUSION Large and hard stool, painful defecation and withholding behavior were the most frequent signs or symptoms among children with chronic functional constipation. Fresh rectal bleeding and anal fissure were the least frequent signs and symptoms in this group.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4293798PMC
January 2015

Evaluation of cholestasis in Iranian infants less than three months of age.

Gastroenterol Hepatol Bed Bench 2015 ;8(1):42-8

Gastroenterohepatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Aim: The aim of this study is to find-out the possible etiologies in Iranian infants less than three months in Shiraz, South of Iran.

Background: Cholestatic jaundice most probably occurs due to a pathological condition and the most frequent causes in early infancy are neonatal hepatitis and biliary atresia. Early diagnosis and treatment of infantile cholestasis can improve prognosis of liver diseases by prevention of the complications of these disorders.

Patients And Methods: In this retrospective study, 122 infants under 3 months of age with cholestasis were studied in Nemazee Hospital (affiliated to Shiraz University of Medical Sciences) during the years 2001-2011. Demographic data, duration of jaundice, liver biopsy and the causes of cholestasis were recorded.

Results: There were 76 males (62.3%) and 46 females (37.7%) with a mean age of 54.4 ± 23.7 days. The most common clinical finding was jaundice that was seen in all patients (100%).The onset of jaundice was the first day to the fifty two days of age, with an average age of 15.6 ± 16.1 days. Other findings included hepatomegaly in 92 patients (76.4%), clay-color stool in 54 (44.3%), and splenomegaly in 29 patients (23.8%). In this study, the most common causes of cholestasis were biliary atresia (30=24.6%), idiopathic neonatal hepatitis (30= 24.6%) and bile ducts paucity (16=10.3).

Conclusion: The results of this study showed that biliary atresia and neonatal hepatitis are the most common causes of infantile cholestasis in this area. It is recommended that biliary atresia should be discriminated from other forms of neonatal cholestasis.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4285931PMC
January 2015

Evaluation of problem-based learning in medical students' education.

J Adv Med Educ Prof 2014 Jan;2(1):1-5

Gastroenterohepatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Introduction: In traditional medical education systems much interest is placed on the cramming of basic and clinical facts without considering their applicability in the future professional career. The aim of this study is to evaluate a novice medical training method (problem-based learning) as compared to the contemporary teacher-based medical education or traditional methods.

Methods: Selection of the study subjects was done through simple sampling and according to the division of medical students introduced from Medical Faculty to the Pediatrics Department with no personal involvement. 120 medical students were assigned to 8 groups of 15 students each. For four months, 4 groups were trained with traditional method and 4 other groups underwent problem-based learning method on selected subject materials. In each method, a pre-course test at the beginning and a post-course test at the end of each course were given to each group. The questionnaire used in this study as the instrument was composed of 39 questions, 37 multiple choice questions and two short answer questions. Three professors of pediatric gastroenterologist took part in the training. Two of these professors were responsible for solving task training method. The third professor used traditional teacher-centered methodology to eliminate any possible bias. Scores obtained from these tests were analyzed using paired t-test and independent t-test. P-values of less than 0.05 were considered as significant.

Results: The scores of the students undergoing the traditional method were 14.70±3.03 and 21.20±4.07 in the first and second test, respectively. In problem-based learning, the scores were 15.82±3.29 in the first and 27.52±4.72 in the second test. There was a significant difference between the mean scores of post-course exams of the two groups (p=0.001), while no significant difference was observed between the mean scores of pre-course exams of the groups (p=0.550).

Conclusion: It may be concluded that problem-based learning method leads to a significant increase in learning and recalling output compared to the traditional method. Given the evolving medical education in the country's medical schools toward problem-based learning, it is suggested that the grounds be laid so that this change will take place based on thought, principles and problem solving.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4235539PMC
January 2014

Management of Bleeding in Post-liver Disease, Surgery and Biopsy in Patients With High Uncorrected International Normalized Ratio With Prothrombin Complex Concentrate: An Iranian Experience.

Iran Red Crescent Med J 2013 Dec 5;15(12):e12260. Epub 2013 Dec 5.

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, IR Iran.

Background: To evaluate the efficacy of prothrombin complex concentrate (PCC) in the management of bleeding in patients with liver disease and patients undergoing surgery or biopsy who had a high uncorrected international normalized ratio (INR).

Objectives: In this study, we examined an Iranian sample and investigated the efficacy of PCC to manage bleeding in patients with liver disease and also patients with high uncorrected INR who were scheduled for surgery or biopsy.

Materials And Methods: A total of 25 patients including 16 patients with post-liver disease bleeding (group 1) and 9 patients with high uncorrected INR who were scheduled for surgery or biopsy (group 2) were enrolled. All patients were treated with 25 IU/kg PCC, and efficacy was defined as any reduction in or cessation of bleeding episodes and correction of INR before surgery or biopsy. The patients were also evaluated for any adverse effects.

Results: INR decreased significantly in both groups of patients, with no bleeding episodes during or after the study in group 1 and during or after surgery/biopsy in group 2. All patients tolerated the therapy well without any significant adverse effects.

Conclusions: The efficacy of PCC therapy was satisfactory in this study. PCC therapy in patients with liver disease and patients undergoing surgery or biopsy seems to be effective and safe, and may be a good treatment strategy for these patients, if fresh frozen plasma or vitamin K are not effective.
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http://dx.doi.org/10.5812/ircmj.12260DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3955503PMC
December 2013

Clinical and para clinical findings in the children with tyrosinemia referring for liver transplantation.

Int J Prev Med 2013 Dec;4(12):1380-5

Gastroenterohepatology Research Center, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Hereditary tyrosinemia type 1 (HT1) is a rare autosomal recessive inborn error of metabolism caused by deficiency of fumarylacetoacetate hydrolase enzyme. This disease manifests with severe liver and kidney impairment and is associated with an increased risk of liver cancer. The aim of this study was to evaluate clinical, laboratory, imaging, and histopathologic characteristics in the children with HT1 who had referred for liver transplantation.

Methods: The present retrospective study was conducted on 45 children with HT1 who had referred to Organ Transplantation Center affiliated to Shiraz University of Medical Sciences between March 2005 and March 2010.

Results: There were 64.4% boys and 35.6% girls with mean age of 3.75±1.28 year (ranges from 2 months to 13 years). The most first clinical presentation was hepatic (80%) and the most prevalent physical findings were hepatomegaly (57.8%), splenomegaly (51.1%), ascites (42.2%), and jaundice (37.9%). The most relevant laboratory parameters were the high serum succinylacetone, alpha-fetoprotein, and tyrosine levels. The most common findings in the patient's abdominal ultrasonography were multiple hepatic nodules (75.6%) and inhomogeneous parenchymal echogenicity of liver (48.9%), while hyper and hypo attenuated nodules (60%) and non-homogeneous pattern of liver parenchyma (53.3%) were the most prevalent findings in abdominal computed tomography scan. In the histopathology of the liver, the most important finding was cirrhosis in all the patients. In this study, 14 patients (31.1%) received Nitisinone (2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyklohexanedione; NTBC).

Conclusions: This study described clinical and laboratory findings in the children with HT1 who had referred for liver transplantation because of end-stage liver disease from all over country, which indicates delay in diagnosis and treatment of this disease. Considering the results of this study, newborn screening for this disease is highly suggested.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3898443PMC
December 2013

Intestinal pseudo-obstruction as an unusual gastrointestinal presentation in pediatric human immunodeficiency virus infection.

Iran J Med Sci 2014 Jan;39(1):68-70

Department of Pediatric Gastroenterology, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Intestinal pseudo-obstruction is a condition in which the intestine's ability to push food through is reduced. It often leads to the dilation of the various parts of the bowel. It can be idiopathic or inherited from a parent, or caused by another disease. We report a rare case of human immunodeficiency virus (HIV) infection in a 3-year-old boy who referred with acute abdominal pain, and was later diagnosed as having intestinal pseudo-obstruction caused by HIV. The underlying causes of intestinal pseudo-obstruction should be taken into account. HIV induced pseudo-obstruction may be considered in the differential diagnosis of pediatric intestinal pseudo-obstruction in order to provide a timely diagnosis and optimal care of children with HIV.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3895898PMC
January 2014

Urinary tract infection and enuresis in children with chronic functional constipation.

Iran J Kidney Dis 2013 Sep;7(5):363-6

Nephrology Urology Research Center, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Introduction: There is still controversy regarding the possible role of chronic functional constipation in disorders of the urinary tract, specifically urinary tract infection (UTI) and enuresis. The aim of this study was to investigate the frequency of (UTI) and enuresis in children with chronic functional constipation.

Materials And Methods: We included 120 children (60.8% girls) with chronic functional constipation based on the Rome III criteria. Detailed history of UTI and enuresis or symptoms pointing to these diagnoses was obtained. Urinalysis, urine culture, and abdominal ultrasonography were performed for all of the participants.

Results: The mean age of the patients was 7.4 ± 3.2 years. Seventy-five percent of the patients had constipation for more than 1 year. The most common urinary symptoms were dysuria (16.7%), urinary frequency (12.5%), and dribbling (4.2%). The frequencies of nocturnal and daytime enuresis were 22.5% and 3.3%, respectively. Pyuria was seen in 10 girls (8.3%). Overall, 7 patients (5.8 %) had a positive urine culture, of whom all were girls and 6 had pyuria. Urinary tract ultrasonography was normal in these patients.

Conclusions: Urinary symptoms, especially nocturnal enuresis, were found in a significant number of children who had chronic functional constipation, but UTI was not so common in the present study. Therefore, we suggest that nocturnal enuresis be considered in children with chronic functional constipation, but screening for UTI is not recommended in these patients.
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September 2013

Clinical characteristics of pediatric esophagitis in southern iran; a single-center experience.

Iran J Med Sci 2013 Jun;38(2 Suppl):169-73

Department of Pediatric Gastroenterology, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz, Iran;

Background: We sought to determine the clinical characteristics of pediatric esophagitis in southern Iran.

Methods: This cross-sectional study was conducted over a 4-year period, from 2005 to 2009, in Nemazee Hospital, a tertiary healthcare center in Shiraz, southern Iran. We consecutively included all pediatric patients (<18 years) who underwent endoscopy in our center and had pathology-confirmed diagnosis of esophagitis. Data regarding the patients' demographic characteristics, comorbidities, and clinical findings were recorded using a questionnaire. All the patients underwent upper gastrointestinal endoscopy and biopsy of the esophagus, and the findings were recorded in the questionnaire.

Results: We studied 125 children, comprising 61 (48.8%) girls and 64 (51.2%) boys at a mean age of 6.6±5.5 years. Repeated vomiting was the prominent symptom in our series, with it being reported by 75 (60%) patients, followed by fever in 35 (28%). Erythema (33.6%), esophageal ulcer (11.2%), and whitish patch (8.0%) were the most common endoscopic findings, while reflux esophagitis (32.8%), chronic (6.4%) and acute esophagitis (5.6%), and candida esophagitis (5.6%) were the most common histological diagnoses. Only one (0.8%) patient was diagnosed as having eosinophilic esophagitis, aspergillosis, and graft-versus-host disease.

Conclusion: Reflux was the most common cause of esophagitis in the pediatric population of southern Iran. Contrary to previous reports, the prevalence of eosinophilic esophagitis was far less than that estimated, while the prevalence of opportunistic infections was higher secondary to post-liver transplantation immunosuppression.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3771219PMC
June 2013

Multiplex ARMS PCR to Detect 8 Common Mutations of ATP7B Gene in Patients With Wilson Disease.

Hepat Mon 2013 16;13(5):e8375. Epub 2013 May 16.

Department of Medical Genetics, Shiraz University of Medical Sciences, Shiraz, IR Iran ; Department of Molecular Medicine, Shiraz University of Medical Sciences, Shiraz, IR Iran.

Background: Wilson disease is a rare disorder of copper metabolism due to mutation in ATP7B gene. Proper counseling of patients with Wilson disease, and their families necessitates finding mutation in ATP7B gene. Finding mutations in ATP7B gene with 21 exons, and more than 500 mutations is expensive and time-consuming.

Objectives: The aim of this study was to provide a simple multiplex amplification refractory mutation system PCR (M-ARMS-PCR) for screening eight common mutations in ATP7B gene.

Patients And Methods: Two sets of ARMS mutant and normal specific primer pairs were designed for genotyping of p.R778L, p.R969Q, p.H1069Q, and p.3400delC mutations as Set 1 and p.W779G, c.3061-1G > A, p.I1102T, and p.N1270S mutations as Set 2. The Multiplex ARMS assay was then subsequently tested in 65 patients with Wilson disease with known and unknown ATP7B mutations.

Results: Using these two sets, we identified H1069Q mutation in four patients, c.2335T > G mutation in three, c.3061-1G > A splice site mutation in five, c.3305T > C mutation in one, and c.3809A > G mutation in two patients.

Conclusions: The Multiplex ARMS assay used in this study can be an efficient, reliable, and cost effective method as a primary screen for patients with Wilson disease.
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http://dx.doi.org/10.5812/hepatmon.8375DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3753551PMC
September 2013

Autoimmune hepatitis in children: experiences in a tertiary center.

Iran J Pediatr 2013 Jun;23(3):302-8

Gastroenterohepatology Research Center, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran ; Shiraz Transplant Research Center, Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Objective: Autoimmune hepatitis (AIH) is a necroinflammatory liver disease of unknown etiology that occurs in the children of all ages. The present study aimed to evaluate the clinical and paraclinical presentations, including pattern of autoantibodies, response to treatment, mortality, and liver transplantation outcome in the Iranian children with AIH.

Methods: The medical records of 87 children (56 girls and 31 boy) diagnosed with AIH between 2001 and 2010 were retrospectively analyzed for clinical and paraclinical profiles and also treatment outcome.

Findings: The mean age of the patients was 10.1±4.5 years (64.4% females). The most common clinical findings were jaundice (70.1%), splenomegaly (67.8%), and hepatomegaly (51.7%). Antinuclear, anti-smooth muscle, and anti LKM antibodies were positive in 14/62, 22/53 and 6/40 patients, respectively (36 patients had type 1 AIH, 6 patients had type 2 AIH, 26 patients were seronegative, and autoantibodies were not available in 19 cases). The most common histological finding in the liver biopsies was chronic hepatitis with interface activity that was seen in 65 (74.7%) patients. The complete response was seen in 52 (59.8%) patients and 24 (27.6%) patients underwent liver transplantation. One-year and five-year survival rates were 87.5% and 80% in the transplanted patients.

Conclusion: AIH should be kept in mind in the differential diagnosis of both acute and chronic liver diseases in the children and treatment with combination of corticosteroids and azathioprine is a good treatment option. In the patients with end stage liver cirrhosis that did not respond to medical therapy, liver transplantation is the treatment of choice.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3684475PMC
June 2013

Erratum to "the comparative study of the effectiveness of cimetidine, ranitidine, famotidine, and omeprazole in treatment of children with dyspepsia".

ISRN Pediatr 2013 28;2013:206546. Epub 2013 Mar 28.

Gastroenterohepatology Research Center, Pediatric Gastroenterology Department, Nemazee Hospital, Shiraz University of Medical Sciences, Shiraz 71937-11351, Iran.

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http://dx.doi.org/10.1155/2013/206546DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3654482PMC
October 2015

Prevalence of celiac disease in healthy Iranian school children.

Ann Saudi Med 2013 Mar-Apr;33(2):159-61

Shiraz University of Medical Sciences, Gastroenterohepatology Research Center, Shiraz Transplant Research Center, Shiraz 7193711351, Iran.

Background And Objectives: Other than its classic presentation, celiac disease can be completely asymptomatic in a proportion of the general population. Subjects with silent celiac disease are at risk of potential complications of the disease, which indicates the importance of early diagnosis. In this study we investigated the prevalence of silent celiac disease in healthy children in our area.

Design And Setting: Cross-sectional screening of healthy children in Shiraz city.

Subjects And Methods: Fifteen hundred school children, 6 to 12 years of age in Shiraz (Southern Iran) were screened for celiac disease through serological testing of their serum anti-tissue transglutaminase immunoglobulin A antibodies. A small intestinal biopsy was performed for children with positive serology tests and pathologic reports were given according to the modified Marsh criteria.

Results: Of the total students included, with a mean (SD) age of 9.5 (1.3) years, 30 subjects had positive anti-tissue transglutaminase immunoglobulin A antibodies, resulting in a total seropositivity of 2%. The prevalence of biopsy proven celiac disease (silent celiac) was 0.6%.

Conclusion: As in many other regions worldwide, this study estimated a relatively high prevalence of silent celiac disease in children in our area, citing the disease as an important health problem in our region.
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http://dx.doi.org/10.5144/0256-4947.2013.159DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6078606PMC
November 2013

Paucity of intrahepatic bile ducts in neonates: the first case series from iran.

Iran J Pediatr 2013 Feb;23(1):65-70

Shiraz University of Medical Sciences, Shiraz, Iran.

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.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3574994PMC
February 2013

Thyroid hormone levels in children with liver cirrhosis awaiting a liver transplant.

Exp Clin Transplant 2013 Apr 21;11(2):150-3. Epub 2013 Feb 21.

Gastroenterohepatology Research Center, Namazi Hospital, Shiraz University of Medical Sciences, Shiraz, Iran.

Objectives: Evidence exists that decreased in triiodothyronine (T3) and thyroxine (T4) levels are associated with the severity of liver disease, and these hormones could be used as disease prognostic factors, but there are paradoxes in this regard in the literature. This study aimed at evaluating the correlation between thyroid hormone levels and severity of liver disease.

Materials And Methods: We measured thyroid hormone levels in 83 children with liver cirrhosis using radioimmunoassay techniques.

Results: Four patients (4.8%) showed a decrease in the amount of T3 and 9 patients (10.8%) revealed increased levels of T3. Also, decreases were seen in the T4 levels of 7 patients (8.4%), and 4 patients (4.8%) showed increases in levels of T4. The serum albumin levels were lower and international normalized ratio was higher in patients with low T3 and low T4. This study reveals that the Model for End-Stage Liver Disease and Pediatric End-Stage Liver Disease scores are statistically related to the decreased amounts of T4 (P = .036). The Model for End-Stage Liver Disease and Pediatric End-Stage Liver Disease scores and the Child scores were higher in low T3 patients, but this was not significant (P > .05).

Conclusions: Decreased levels of thyroid hormones are correlated with the severity of disease and can be seen in more advanced cirrhosis. Patients with decreased T4 levels need a liver transplant more immediately than those patients that do not have decreased T4 levels.
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http://dx.doi.org/10.6002/ect.2012.0182DOI Listing
April 2013

The Role of Cow's Milk Allergy in Pediatric Chronic Constipation: A Randomized Clinical Trial.

Iran J Pediatr 2012 Dec;22(4):468-74

Transplant Research Center, Shiraz University of Medical Sciences, Shiraz, Iran ; Gastroenterohepatology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

Objective: Cow's milk allergy has different presentations in children and can cause functional bowel symptoms such as chronic constipation. The aims of this study were to investigate the role of cow's milk allergy as a cause of chronic constipation and effect of cow's milk free diet (CMFD) on its treatment in children.

Methods: We performed a randomized clinical study comparing CMFD with cow's milk diet (CMD) in two groups each consisting of 70 patients (age range, 1-13 years) with chronic functional constipation (defined as Rome III criteria). All subjects had been referred to a pediatric gastroenterology clinic and had previously been treated with laxatives for at least 3 months without success; also all 140 patients performed skin prick test. The case group received CMFD for 4 weeks. After that they received CMD for 2 extra weeks. The control group received CMD for whole 6 weeks. A response was defined as decreased in signs and symptoms that not fulfilled Rome III criteria after 4 weeks of CMFD and came back to Rome III criteria after 2 weeks of CMD challenge.

Findings: After 4 weeks 56 (80%) patients of the case group responded in comparison to 33 (47.1%) patients in the control group (P=0.0001). In the case group after 2 weeks challenge 24 out of 56 (42.8%) responders developed constipation according to Rome III criteria. With other words, the frequency of cow's milk allergy among constipated patients was 80%. Only one patient had positive skin prick test.

Conclusion: In children, chronic constipation can be a manifestation of cow's milk allergy. At present, although several aspects must be further investigated, a therapeutic attempt with elimination diet is advisable in all children with constipation unresponsive to correct laxative treatment.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3533146PMC
December 2012

Epidemiology of Pediatric Acute Poisoning in Southern Iran: A Hospital-Based Study.

Bull Emerg Trauma 2013 Jan;1(1):28-33

Department of Pediatrics, Shiraz University of Medical Sciences, Shiraz, Iran.

Objective: To determine the epidemiology of acute childhood poisoning in Shiraz, southern Iran.

Methods: This was a prospective cross-sectional descriptive study, being performed in Nemazee and Dastgheib Hospitals affiliated with Shiraz University of Medical Sciences. The study included pediatric patients (<18 years) referred to our centers due to acute poisoning. Demographic and etiological factors were prospectively recorded and analyzed.

Results: A total of 773 patients with mean age of 3.86 ± 1.5 years were recruited in the study. The most common group which included 352 (45.5%) patients, aged between 8 months and 5 years followed by 330 (42.6%) cases aged from 12-18 years. In majority of cases, poisoning was due to opium in 222 (23.5%) followed by analgesics in 181 (19.1%), which mostly included acetaminophen in 75(7.9%), anti-depressants in 170 (17.9%), anti-hypertensive drugs in 65 (6.8%) and hydrocarbons in 60 (6.3%). There were 260 (33.7%) boys and 513 (66.3%) girls. The poisoning occurred inadvertently in 387 (50.1%) cases while 298 (38.5%) patients committed suicide. Most cases (255 patients; 32.9%) were asymptomatic at presentation.

Conclusion: Our study substantiated the following findings: A) Alarmingly, opium is the most common cause of acute childhood poisoning in our area. B) Easy access to toxic material is the most common risk factor for acute childhood poisoning. C) Female predominance of acute childhood poisoning accompanied by high rate of suicidal attempts shows that psychiatric problems, especially depression is most common among young girls.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4771239PMC
January 2013

Etiology and complications of liver cirrhosis in children:report of a single center from southern iran.

Middle East J Dig Dis 2013 Jan;5(1):41-6

Gastroenterohepatology Research Center, , Nemazee Teaching Hospital, School of Medicine, Shiraz University of Medical Sciences, Shiraz, Iran.

Background: Liver cirrhosis is one of the major causes of hospitalization and mortality in children. A wide spectrum of disorders including developmental abnormalities, infections, metabolic and genetic disorders can lead to liver cirrhosis in pediatric patients. Determination of its etiology is important for treatment, prevention of progressive liver damage, family counseling and prioritizing liver transplantation. The aim of this study is to evaluate causes of liver cirrhosis in children in Southern Iran.

Methods: We included all cirrhotic children aged less than 18 years who referred to an outpatient pediatric gastroenterology clinic affiliated with Shiraz University of Medical Sciences between March 2009 and September 2010 in this cross-sectional study. The etiology of cirrhosis was determined according to clinical findings, laboratory tests, imaging studies such as ultrasonography or computed tomography scan, hepatobiliary scintigraphy and histopathologic examination of the liver biopsy. Cirrhosis with unknown etiology was considered as cryptogenic.

Results: A total of 106 cirrhotic children aged between 5 months to 18 years with a mean age of 8.24 ± 6.12 years that included 60 boys (56.6%) and 46 girls (43.4%) were enrolled in the study. The most common causes of liver cirrhosis were Wilson disease (n=22; 20.7%), biliary atresia (n=19; 17.9%), and cryptogenic cirrhosis (n=14; 13.2%). Other causes were autoimmune hepatitis (n=12; 11.3%), idiopathic neonatal hepatitis (n=10; 9.4%), hepatorenal tyrosinemia (n=9; 8.5%), glycogen storage disease (n=6; 5.7%), and progressive familial intrahepatic cholestasis (n=4; 3.8%).

Conclusion: Considering the most common etiology of liver cirrhosis in children in this part of Iran we suggest testing for Wilson disease in all cirrhotic children.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3990133PMC
January 2013