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Background: Brain damage and mental retardation in children can be caused by either excess or depletion of certain amino acids. Up to now there is no reliable reference interval for free plasma amino acid in Iranian children. Objectives: The aim of this study was to provide the reference intervals of free plasma amino acid in healthy Iranian children using high performance liquid chromatography (HPLC) as an accurate method. Methods: 133 children referred to Children's Medical Center, Tehran, Iran for the annual checkup entered the study after filling out the informed consent form. Demographic data and blood samples after overnight fasting were collected. Plasma amino acid concentrations were determined by reverse phase HPLC (RP-HPLC) equipped with a fluorescence detector after precolumn derivatization of amino acids with o-phthalaldehyde (OPA). Results: Female children exhibited significantly higher plasma concentrations of taurine and lysine than male children. Other 20 amino acid values were not significantly different between two gender groups. Positive correlation was observed between age and taurine, leucine and lysine. Conclusions: Our data provided reference intervals of free plasma amino acids in healthy Iranian children. We believe our data can be used as a guideline for pediatricians to identify the significantly deviated amino acid values in children with medical issues such as genetic disorders and some acquired illnesses. Our data is also applicable for further investigations in the field of family medicine. [ABSTRACT FROM AUTHOR]

Background: Shigellosis is a diarrheal disease caused by Shigella spp. The majority of cases and deaths occur among children less than 5 years old. In severe cases, antibiotic therapy is recommended to lessen the risk of serious complications and death. Prevalence of different Shigella species and their antibiotic resistance patterns are changing over the time. Objectives: The current study aimed at assessing the changes in the prevalence of Shigella species and their antibiotic susceptibility among 0- to 14-year-old children referred to Children’s Medical Center, Tehran, Iran, from 2009 to 2014. Methods: The results of stool cultures were retrospectively analyzed to determine the prevalence of different Shigella species, their antibiotic susceptibility patterns and their changes in the largest university affiliated pediatrics center in Tehran. Results: Among 40 700 stool cultures, 507 cases were positive for Shigella spp. Most cases occurred among children under 5 years old. Shigellosis was more prevalent among males. The most common species were Shigella sonnei and Shigella flexneri. In general, they were most sensitive to cefotaxime and most resistant to cotrimoxazole. Resistance to cefotaxime and nalidixic acid increased annually. Resistance to ampicillin had a descending trend. Resistance to cotrimoxazole remained almost constant. Conclusions: The most common species of Shigella changed from Shigella flexneri to Shigella sonnei over the years. In comparison with the previous studies, resistance to nalidixic acid increased and resistance to ampicillin decreased. The prevalence and antibiotic resistance patterns of Shigella species, at different times and regions are changing. To make appropriate decisions on treatment, it is necessary to monitor the changes. [ABSTRACT FROM AUTHOR]

Background: Chronic abdominal pain in children is a common disorder. For an accurate diagnosis of its cause, sometimes invasive diagnostic procedures such as endoscopy should be performed. Objectives: The purpose of the study was to evaluate the fecal calprotectin in children with upper gastrointestinal signs and symptoms and to compare it with endoscopic findings. Methods: A total of 131 children aged 1-14 years with upper gastrointestinal symptoms were enrolled during 2012 - 2013 at two centers. One hundred and twenty patients underwent endoscopy and biopsy. Before endoscopy, the level of calprotectin was measured by the enzyme-linked immunosorbent assay test in stool samples and the results were compared with the endoscopic and pathology findings. Results: Of the 120 children included in this study, 71 (59.2%) were males and 49 (40.8%) females with a mean age of 93.6 months. Of the 112 patients in whom biopsies were taken, 16 had esophagitis, 89 chronic gastritis (79.5%) and 57 colonization with Helicobacter pylori. Conclusions: There was a statistically significant correlation between fecal calprotectin and gastritis and severity of H. pylori infection. Fecal calprotectin level measurement can avoid unnecessary endoscopies and is also useful for evaluation of therapy response. [ABSTRACT FROM AUTHOR]

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 administration. Few studies were conducted in Iran and epidemiology of STEC remains obscure; this necessitates examination of stools especially in young children for this bacterium. Methods: We determined the frequency of STEC in 947 E. coli strains isolated from diarrheal stools of children less than 14 years in Tehran with conventional culture methods and multiplex-PCR via determining the STX1 and STX2 genes, between October 2008 and September 2009. We also evaluated the association between stool exam findings and presence of STEC. Findings: Twenty seven (2.8%) of E. coli isolates were positive for STX1 or STX2 genes, most of which occurred in spring (P<0.05). There was no significant association between STEC positivity and stool exam findings. Eighteen out of 27 (66.7%) Shiga toxin positive samples were isolated from males and the rest from females. The most common STX-positive diarrheal samples showed loose consistency (P<0.017). Conclusion: Although the low frequency of STEC in our population indicates that it is not a major problem in our population, STEC should be regarded as an important infection because of its severe consequences. Further studies with greater sample size are needed to confirm our results. [ABSTRACT FROM AUTHOR]

Objective: Alpha1-antitrypsin deficiency (A1ATD) is the most important indication for liver transplantation in children. The gene frequencies vary in different ethnic groups. In the present study, we attempt to determine the frequencies of the most common defective alleles, Z and S, in Iranian children suffering from idiopathic neonatal cholestasis. Eighty-seven infants were typed for Z and S alleles. Methods: In a single center study, 87 consecutive liver biopsies from infants with cholestasis were reviewed and patients with neonatal cholestasis enrolled in the study and cases with confirmed biliary tract atresia excluded. Formalin fixed paraffin embedded blocks were used for DNA extraction. AAT genotype was determined by polymerase chain reaction (PCR) assay and amplification of the two most common deficiency variants, S and Z alleles, and then sequencing of PCR products. Findings: There were 48 (55.2%) males and 39 (44.8%) females, with a median age of 60 days. Out of 87 of the study subject, 2 (2.2%) were heterozygous for the S allele, and no ZZ, SS or MZ individual was found in the patients. No other polymorphism was found in the sequencing results. Conclusion: In comparison to other populations, AAT deficiency seems not to be an important etiologic factor for neonatal cholestatic liver disease in Iran; however, further studies are recommended to estimate the true mutant gene frequencies. [ABSTRACT FROM AUTHOR]

Objective: Exposure to environmental tobacco smoke (ETS) is one of the major factors of predisposing children to develop several hazardous health problems. We decided to investigate the association between nicotinine, one of the nicotine metabolites and esophagitis in children with gastroesophageal reflux disease (GERD). Methods: In a case control study 46 children suffering from esophagitis referred to endoscopy ward were recruited. The control group consisted of 45 healthy children. Urine samples were collected and urinary cotinine level (UCL) measured. Findings: The mean age of esophagitis and control groups were 5.11±2.93 and 6.72±2.8 respectively. Sixty children were passive smokers; 31 of them had non-smoker parents. In control group, 32 (71.1%) children and in esophagitis group 29 (63%) children had non-smoker parents. The mean value of UCL in patients suffering from esophagitis was significantly higher than those in normal group (P=0.04, 24.98±6.4 ng/ml vs. 15.16 ± 3.9 ng/ml). Considering 50ng/ml as a cutoff point for UCL, it was significantly higher in passive smoker group than in non smoker group (P=0.02). The mean cotinine level differed significantly in esophagitis and control group. Conclusion: Our results indicate the increased risk of developing esophagitis in children with ETS exposure. [ABSTRACT FROM AUTHOR]

Objective: Wilson's disease (WD) is an autosomal recessive disease with genetic abnormality on chromosome 13 causing defect in copper metabolism and increased copper concentration in liver, central nervous system and other organs, which causes different clinical manifestations. The aim of this study was to determine the sensitivity of different clinical and paraclinical tests for diagnosis of Wilson's disease. Methods: Paraffin blocks of liver biopsy from 41 children suspicious of WD were collected. Hepatic copper concentrations were examined with atomic absorption spectrophotometry (Australian GBC, model: PAL 3000). Fifteen specimens had hepatic copper concentration (dry weight) more than 250µg/g. Clinical and laboratory data and histologic slides of liver biopsies of these 15 children were reviewed retrospectively Liver tissue was examined for staging and grading of hepatic involvement and also stained with rubeonic acid method for copper. Findings: patients were 5-15 years old (mean age=9.3 years, standard deviation=2.6) with slight male predominance (9/15=60%). Five (33%) patients were 10 years old. Three (20%) of them were referred for icterus, 8 (54%) because of positive family history, 2 (13%) due to abdominal pain and 2 (13%) because of hepatosplenomegaly and ascites. Serum AST and ALT levels were elevated at the time of presentation in all patients. In liver biopsy, histological grade and stage was 0-8 and 0-6 respectively, 2 (13%) had cirrhosis, 1 (7%) had normal biopsy and 12 (80%) showed chronic hepatitis. Hepatic copper concentrations were between 250 and 1595 µ/g dry weight. The sensitivity of various tests were 85% for serum copper, 83% for serum ceruloplasmin, 53% for urinary copper excretion, 44% for presence of KF ring and 40% for rubeonic acid staining on liver biopsies. Conclusion: None of the tests stated in the article were highly sensitive for diagnosis of WD, so we suggest that diagnosis should be based on combination of family history, physical examination and different tests. [ABSTRACT FROM AUTHOR]

Objective: Acute appendicitis is the most common cause of abdominal surgery in children. Similarity between signs and symptoms of appendicitis and other common pediatric illnesses, atypical manifestations of appendicitis in young children, and children's inability to give precise explanation for their symptoms contribute to considerable delay in proper diagnosis and increased rate of perforation. Current study reports the surgical and pathological findings of appendectomies in the largest Children's Hospital in Iran. It also evaluates whether common protocol for pathologic evaluation following appendectomy is beneficial. Methods: Pathologic reports of 947 appendectomies, performed with the presumptive diagnosis of acute appendicitis, were gathered. Correlation between surgical and pathologic findings was assessed. Demographic characteristics of patients between surgical and pathological subgroups were also compared. Findings: The mean age of participants was 6.9+3.5 years. Eighty seven (25.5%) children had abnormal pathological findings and normal surgical report. None of miscellaneous findings including appendicular carcinoid tumor 3 (0.3%), oxyuriasis 2 (0.2%), and mycobacterial infection 4 (0.5%) were recognizable during the surgery. Of all pathologically confirmed cases with perforated appendicitis, 9.7% were not detected during the surgery. Conclusion: In current study, acute appendicitis was the most common pathological diagnosis, however, high normal appendectomy rate along with noticeable proportion of surgically missed perforated appendicitis and unusual histopathologies strongly supported routine histological examination. [ABSTRACT FROM AUTHOR]

Objective: Hirschsprung's disease (HD) is a complex disorder resulting from absence of ganglion cells in the bowel wall leading to functional obstruction and bowel dilatation proximal to the affected segment. The aim of our study was to evaluate rectal biopsies from constipated children in different age groups to see in which age it is more likely to encounter HD to avoid unnecessary rectal biopsy. Methods: Records of all children with chronic constipation undergoing a rectal biopsy to exclude HD were obtained from the files of Children's Medical Center in Tehran, Iran. A detailed retrospective demographic review, including age of beginning of signs and symptoms was made of all cases. Findings: Totally, 172 biopsies were taken from 168 children in a five year period, of which 127 cases (75%) had HD. The mean age of constipated patients at biopsy was 39 months and the mean age of patients with proven HD was 18 months. Males were affected more than females. Congenital anomalies associated with HD were found in 9.6%. In 85 (91%) cases constipation had begun in neonatal period. Conclusion: Our data supports previous studies that if constipation begins after the neonatal period, the child is unlikely to have HD. In neonates delay in meconium passage is the most important clinical sign of HD. [ABSTRACT FROM AUTHOR]

Objective: Beta-thalassemia major is an autosomal recessive disease causing severe and hemolytic anemia, which begins about 2-6 months after birth. Iron overload, which arises from recurrent transfusion and ineffective erythropoiesis, can enhance oxidative stress in thalassemic patients. The aim of this study was to evaluate the serum total antioxidant capacity of patients with Ã- Thalassemia major. Methods: Sixty six Iranian patients with Ã-thalassemia major and 66 age-gender matched controls were evaluated for serum total antioxidant status (TAS), uric acid (UA), bilirubin and albumin. In addition, serum ferritin and transaminases were recorded in these subjects. Findings: Significant increases of TAS, UA, and bilirubin were observed in the patient group, compared with the control group (P<0.01). Mean TAS and bilirubin in male patients was higher than in females (P=0.005 and P=0.008, respectively). There was also direct correlation between TAS and albumin (P<0.001), bilirubin (P<0.001) and UA (P=0.002). Conclusion: Endogenous antioxidants such as ferritin, UA and bilirubin can result in increased level of TAS in the patients with Beta-thalassemia major. Compensatory excess of TAS to oxidative stress could also be the reason for difference between our findings and previous studies. [ABSTRACT FROM AUTHOR]

Objective: This study aims to evaluate the role of breastfeeding in the acquisition of Helicobacter pylori (H. pylori) infection in Iran and to compare the histopathologic changes occurring in children feeding on breast milk with those in infants feeding on formula. Methods: In a case-control study parents of children with and without H. pylori infection who had undergone endoscopic survey and gastric biopsy in the Children's Medical Center, Tehran, were asked about their feeding practices during the first 6 months after birth, the duration of breastfeeding period, the symptoms, and the duration of symptoms and concomitant diseases. Findings: A total of 154 children were included in this study. From this sample, 77 children formed the case group and 77 children formed the control group. A significant difference was found between H. pylori infection and feeding with formula (P=0.045). In case group, a significant difference was found between breastfeeding and age of the infected child (P=0.034), shorter duration of symptoms (P=0.016), and finally degree of H. pylori colonization (P=0.021). Conclusion: It appears that breastfeeding in the first 6 months after birth can decrease the degree of H. pylori colonization, postpone infection until older age, shorten the duration of symptoms, and be concomitant with milder gastritis. [ABSTRACT FROM AUTHOR]