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Background Considering the increasing prevalence of type 1 diabetes mellitus (T1DM) as an autoimmune disease in recent years and the positive effects of vitamin D (VD) on this disease, especially the preventive effect of VD on progressive reduction of pancreatic β-cells, we aimed to investigate the effect of VD on pancreatic β-cell function in T1DM patients. Materials and Methods From Sep 2016 to Nov 2017, this single-blind clinical trial study was performed on patients who have affected with T1DM in the last five years, referred to Endocrinology Clinic of Amirkola Children's Hospital in Babol city of Iran. The patients with VD level less than 30 ng/mL were treated with 50, 000IU Pearl VD for 9 months. The patients' C-peptide, 25 (OH) D, HbA1C and total daily dose(TDD) insulin were compared at the beginning and end of the study. Data were analyzed using SPSS software version 22.0. Results By examining all 30 randomly selected children meeting the inclusion criteria, it was found that VD increased the C-peptide level (0.06 nmol/L), slightly improved the pancreatic β-cell function as well as decreased their HbA1c (0.64%), and TDD insulin levels (0.05 unit) although none of the relationships was significant. However, a significant decrease in HbA1c level was found in the female group (p=0.04) as well as in the age group less than 10 years (p=0.007). Conclusion VD intake had no significant effect on serum C-peptide levels and reduction of HbA1c and TDD insulin though a significant decrease in HbA1c level was observed in the female group and in the patients less than ten years.

Background: Methylmalonate-semialdehyde Dehydrogenase Deficiency (MMSDHD) is an uncommon autosomal recessive disorder. MMSDH is an enzyme encoded by the protein coding gene ALDH6A1 in humans. Case Presentation: We present a 4-year-old boy with elevated liver enzymes, 3-hydroxyisobutyric aciduria (MMSDHD) and cardiac symptoms. He had a mutated ALDH6A1 gene, c.184c>G (p.Pro62Ala). Conclusions: This is one of the rare case reports in the world and the first one in Iran that reports MMSDHD with cardiac disease.

Background: Thalassemia major (TM) is one of the most common hereditary anemia with multiple endocrinopathies (especially hypogonadism). So, we evaluated the rate of delayed puberty (DP) and its relation with serum ferritin level in patients. Materials and Methods: This cross-sectional (descriptive-analytical) study was conducted on 100 patients with TM between 14-64 years old, admitted to Amirkola Thalassemia Center, Babol, Iran, in 2016. The pubertal status, (Marshall-Tanner scale), existance of DP, and its different types were evaluated. Mean serum ferritin level was measured and the data were classified to three groups of 2500 ng/ml. Data were analyzed using SPSS (version20). Results: Out of 100 patients, 64 (64%) and 36 (36%) were female and male, respectively. Considering age, 23, 77 patients (%) were under and over 20 years old, respectively. Totally, 69 (69%) of them had DP, of whom 64 (92.8%) ones had secondary (central) hypogonadotropic hypogonadism. Mean serum ferritin level (±SD) was 2707.94±1683.42 ng/ml. In addition, 26, 29, and 45 patients had ferritin level 2500 ng/ml, respectively. Thirty two patients with DP (46.4%) had ferritin level above 2500 ng/ml (p-value= 0.623). Conclusion: The results showed a high frequency of DP in TM patients, requiring careful examination and follow-up in terms of puberty for early diagnosis and proper treatment to improve their quality of life, and prevention of the complications like osteoporosis. We couldn't find any significant relationship between serum ferritin level and hypogonadism, even for cases who received enough iron chelators. Keywords: Delayed Puberty, Ferritin, Hypergonadotropic Hypogonadism, Hypogonadotropic Hypogonadism, Thalassemia Major

OBJECTIVES: This study aimed to investigate the association between Helicobacter pylori infection with diabetes mellitus type one and the effect of infected Helicobacter pylori on glycemic control. METHODS: This case control study was conducted on children with and without type 1 diabetes mellitus (T1DM). Demographic data and gastrointestinal symptoms in both groups and glycemic control status and duration of diabetes were recorded in patients with T1DM. Stool test was done on all children to detect Helicobacter pylori antigen. RESULTS: Sixty three children with T1DM with a mean of 10.88 ± 2.84 years and 105 control children with an average age 10.17 ± 2.55 years (P = 0/09) were involved in this study. The frequency of Helicobacter pylori infection in patients with T1DM was 17/63 (27%) and 25/105 (23.8%) in control group, (P = 0/64). The frequency of bloating, epigastric pain and nausea was not significantly different between the two groups. The frequency of epigastric pain in children with diabetes with helicobacter infection was significantly higher than non-infected children with diabetes (29.4% vs. 2.2%) (P = 0.004).The mean duration of diabetes (P = 0.53), age diagnosis of diabetes (P = 0.09), fasting blood glucose (P = 0.18), glycosylated hemoglobin (P = 0.08) and the daily insulin dose (P = 0.18) in patients with T1DM with and without helicobacter pylori infection had not significantly different. CONCLUSIONS: There was no significant association between Helicobacter pylori infection and diabetes in children 5–15 years old, and glycemic control status was not difference in patients with T1DM with and without Helicobacter pylori infection.

To evaluate the efficacy of stoss therapy using fortified biscuit for vitamin D-deficient children. A total of 108 children aged 30–72 months with vitamin D deficiency were studied in a randomized single-blind clinical trial. The deficient children were assigned to three groups, namely, vitamin D-fortified biscuit (BG), capsule vitamin D (CG), and ampoule vitamin D (AG). Capsules and biscuits containing 50,000 IU of cholecalciferol were consumed twice per week for 3 consecutive weeks. Ampoules with 300,000 IU of cholecalciferol were injected intramuscularly in a single dose. Three weeks after treatment, serum 25(OH)D concentrations were measured, and the three groups were compared. Each method of treatment could increase the mean serum 25(OH)D concentration to optimal level. Serum 25(OH)D concentrations ≥100 ng/mL were observed in six children, including four from AG and two from CG (P = 0.09). The comparison of the mean serum 25(OH)D concentrations after treatment showed between ampoule and capsule (P = 0.3) and capsule and biscuit (P = 0.62) were insignificant; however, the ampoule and biscuit groups differed significantly (P = 0.012). Stoss therapy using fortified biscuit may be an effective way to improve compliance in children who cannot take capsules without adverse effects and may also be recommended for prevention purposes.

Rickets is failure in mineralization of growing bone and cartilage due to extreme vitamin D deficiency (VDD). The study aimed to identify rickets among vitamin D deficient children and determine any relationship between clinical findings and paraclinical evidence.This study was conducted in two stages. In the first stage, blood was drawn from 406 children aged 30-72 months for measurement of 25(OH)D level. Of these children, 108 had 25(OH)D levels of20 ng/dL and were evaluated physically for signs and symptoms scores (0-1) of VDD and rickets. Biochemical analysis and radiography of the child's left wrist and hand was performed.Of the 119 children (29.67%) with 25(OH)D levels of20 ng/dL, 42 (10.3%) had vitamin D levels of ≤15 ng/dL. There was no correlation between serum 25(OH)D level and levels of calcium (Ca) (r = -0.16), alkaline phosphatase (ALP) (r = -0.12), P (r = 0.13), and parathyroid hormone (PTH) (r = -0.15,) in children with VDD. The mean of signs and symptoms scores had no significant difference between children with (1.59 ± 0.8) and without (1.73 ± 1.01) VDD (P = 0.3). None of the children with VDD had radiographic evidence of rickets. Radiographic data showed that 69.2% (72), 10.6% (11), and 20.2% (21) of the children had delayed, normal, and advanced bone age, respectively.Abnormal radiological findings of rickets were not found on wrist X-rays. Thus, this investigation is not necessary within the range of vitamin D levels described in the current study.