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.

Evaluation of the safety and efficacy of biosimilar recombinant growth hormone in children with growth hormone deficiency: non-inferiority, randomized, parallel, multicentric and Phase III trial.

OBJECTIVES: This study is designed in order to compare the efficacy and safety of recombinant human growth hormone (rhGH) with the reference brand. METHODS: According to the inclusion criteria, 85 people in 13 Iranian centers were randomly selected to receive biosimilar Somatropin (Somatin®) (44 people) and reference Somatropin (Norditropin®) (41 people) at a dose of 35 µg/kg/d, seven days/week for 12 months. The primary outcomes included height velocity (HV) was measured during 12 months of treatment. RESULTS: The two intervention groups' Height changes were similar. The mean HV was 10.96 cm/year in the biosimilar group and 10.05 cm/year in the reference groups after 12 months. Estimates of the lower bounds of 95% CI for mean height differences in the biosimilar intervention group compared to the reference intervention group did not exceed the 2 cm margin. Therefore, the non-inferiority of biosimilar intervention compared to the brand product is verified. Common ADRs in both groups were nausea in two patients (2.4%), diarrhea in two patients (2.4%), increased body temperature in one patient (1.2%), and headache in one patient (1.2%). CONCLUSIONS: The finding of this study indicated that Somatin® and Norditropin® have comparable efficacy and safety profiles. CLINICAL TRIAL REGISTRATION: www.IRCT.irIRCT20171122037571N1.

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.

Helicobacter Pylori Infection in Children with Type 1 Diabetes Mellitus: A Case-Control Study.

BACKGROUND: Helicobacter pylori infection is one of the most common chronic bacterial infections. There is challenge on the real rate of prevalence of H. pylori in diabetic patients. This study was done to assess the prevalence of H. pylori infection in children suffering from type 1 insulin-dependent diabetes mellitus. METHODS: In this case-control study, 80 diabetic patients (as the target group) refer to the Endocrinology Clinic of Tabriz Educational and Treatment Center, Tabriz northwestern Iran and 80 non-diabetic patients (as the control group) from the group of children referring to the GI Clinic of the same center  were enrolled in 2012 and 2013. Then H. pylori infection was assessed in two groups using measuring antibody (IgG) and stool antigen (HpSA). RESULTS: H. pylori infection tests were positive in 48 (60%) diabetic patients and in 32 (40%) in non-diabetic patients (P=0.030). There was a meaningful correlation between the frequency of H. pylori and the longer the duration of diabetes (P<0.001). No correlation was seen between H. pylori infection and other factors such as age of the patients (P=0.840), HbA1C level (P=0.312), age at which diabetes was diagnosed (P=0.800), average daily dosage of insulin (P=0.232), and presence of GI symptoms (P=0.430). CONCLUSIONS: Type 1 diabetic children especially cases with the longer duration of diabetes, are at risk acquiring H. pylori infection. Therefore, screening of H. pylori infection is helpful on the follow up of these patients.

Thyroid hormonal status among children with obesity.

BACKGROUND: It is presumed that free T4 and thyroid-stimulating hormone (TSH) levels are related to obesity, but the findings are inconsistent. In this study we evaluated T4 and TSH concentrations between normal children and those with obesity and possible correlations between body mass index (BMI) and these markers. METHODS: In this prospective study, 190 children who were overweight and obese and 133 children without obesity of the same age and sex were evaluated. Thyroid function tests (TSH, total T4, free T4 and free T3) were measured in all subjects in both groups. Thyroid antibodies (thyroid peroxidase and thyroglobulin) were determined among those with elevated TSH levels. RESULTS: Levels of TSH and total T4 were significantly higher in children with obesity compared with the control group. Subclinical hypothyroidism was significantly higher in children with obesity (14.7%) compared with normal subjects (6.8%, p = 0.02). Among children with obesity and increased TSH levels, 10.7% had positive thyroid peroxidase and thyroglobulin antibodies. There was significantly positive correlation between BMI z score and TSH level (r = 0.198, p < 0.001) and T4 level (r = 0.18, p = 0.001). CONCLUSION: TSH and total T4 levels are increased in children who are overweight or obese and are a common finding in these children, but the incidence of thyroid antibodies is low in these patients and so could not be accounted for by thyroid autoimmunity. Due to these findings it is possible that increased TSH and total T4 levels are a consequence of obesity and could be reduced by decreasing BMI.

Insulin resistance and ß cell function in patients with ß-thalassemia major.

Iron overload is a common finding in chronically transfused ß-thalassemia major (ß-TM) patients with possible effect on ß cell function and insulin resistance. In this study we aimed to evaluate glucose metabolism, insulin resistance and ß cell function in ß-TM patients. A total of 78 transfusion-dependent ß-TM patients and 40 age and sex matched normal children were included. Oral glucose tolerance tests (OGTT) were performed in all subjects. Fasting plasma insulin level, insulin resistance index (IRI) and ß cell function index (BFI) were also estimated. ß-Thalassemia major patients had significantly more abnormal OGTT than the control group. ß-Thalassemia major patients had significantly higher levels of aspartate aminotransferase (AST) and alanine aminotransferase (ALT) fasting blood sugar and IRI than the control group. Findings between ß-thalassemia (ß-thal) patients, with and without abnormal OGTT results, were also compared; ß-thal patients with abnormal OGTT had significantly higher duration of chelation therapy, serum ferritin levels, AST, ALT and increased IRI and decreased BFI in comparison to patients with normal OGTT. Abnormal glucose metabolism is common in ß-TM patients with chelation therapy and multiple transfusions which are attributable to impaired ß cells' function and increased insulin resistance.

Tumour necrosis factor-alpha gene polymorphisms in Iranian patients with biliary atresia.

BACKGROUND: Biliary atresia (BA) is a progressive inflammatory destructive process of the bile ducts. This study evaluated the relationship between single-nucleotide polymorphisms in the promoter region of tumour necrosis factor-alpha (TNF-α) gene and bilaiary atresia. MATERIALS AND METHODS: Genomic deoxyribonucleic acid from 16 patients with established diagnosis of BA and 36 patients with INC was obtained. The genotypes of TNF-α-1031 (T/C) and TNF-α-308 (G/A) were determined using the restriction fragment length polymorphism-polymerase chain reaction and the results were analysis with proper statistic software. RESULTS: The frequencies of T/T, T/C in TNF-α-1031 and G/G, G/A in TNF-α-308 were as same as control group. Moreover, we have same deduction for allele frequency and haplotypes analysis (T allele: 84.37%; G allele: 87.5%) in BA patients (T allele: 80.56%; G allele: 86.11%) in controls. In all cases variants of polymorphism did not affect the severity or incidence of BA disease. CONCLUSION: although no significant associations were found between BA and control groups, it seems meaningful that since the nature of BA is multi factorial. Next step will be considering a new target such as downstream modulation of the TNF-α pathway or other cytokines and chemokines which act directly/indirectly.