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Background This study aimed to evaluate the effect of Neuromuscular Electrical Stimulation (NMES) on serum glucose level in children and adolescents with type-1 diabetes. Methods This before-after, single-group, clinical trial was conducted on 29 patients with type-1 diabetes mellitus with the age range of 7–18 years. The patients underwent NMES in two 20-minute phases on the quadriceps and hamstrings muscles, three sessions per week for a period of 8 weeks. Fasting Blood Sugar (FBS), measured in two ways, by glucometer and laboratory testing, was considered as the primary outcome and the glycated hemoglobin (HbA1c) and the total daily dose (TDD) of insulin were measured as the secondary outcomes. The laboratory FBS and HbA1c were measured 1 day before the intervention (as a baseline value) and then 2 and 6 weeks after the last session of intervention. FBS by glucometer and total daily dose of insulin were recorded daily from 2 weeks before the intervention to the last day of the intervention and consequently, the weekly average of these variables was calculated and used for statistical analysis. Results The serum level of FBS (measured by glucometer) and the total daily dose of insulin reduced significantly 2 weeks after beginning of intervention. The laboratory serum level of FBS decreased significantly in the second week after the end of intervention compared to the baseline values. Although the HbA1c level decreased at follow-up period (2 and 6 weeks after the intervention), it was not significant. Conclusion It seems that 8 weeks of NMES has beneficial effects on the reduction of FBS and TDD of insulin therefore, it could be suggested as the contributory treatment in management of children and adolescents with type-1 diabetes. Trial registration The study was registered at https://fa.irct.ir/user/trial/51739/view (IRCT20100523003998N1) in date of 25/10/2020.

Beta‐ketothiolase (T2, mitochondrial acetoacetyl‐CoA thiolase) deficiency is an autosomal recessive disorder of isoleucine catabolism and ketone body metabolism that is characterized by increased urinary excretion of 2‐methylacetoacetate, 2‐methyl‐3‐hydroxybutyrate, and tiglylglycine. Most patients with T2 deficiency develop their first severe ketoacidotic events between 5 and 24 months of age. We encountered a case of T2 deficiency who developed the first hypoglycemic crisis without ketosis during her neonatal period and repeated such nonketotic hypoglycemic crisis during her infancy and early childhood. This is a very atypical clinical phenotype in T2 deficiency. We finally realized that she also has severe carnitine deficiency which might suppress beta‐oxidation resulting in nonketotic hypoglycemia. After carnitine supplementation, she actually developed episodes with ketonuria. Her carnitine deficiency was probably a secondary deficiency which is rare in T2 deficiency but if present, may modify the clinical manifestation of T2 deficiency from ketoacidotic events to hypoketotic hypoglycemic events.

The growth and development of children affect biochemical variables. This population-based study was designed to evaluate the reference interval for alkaline phosphatase (ALP) routinely measured in the clinical laboratory.For this examination, 873 cases were selected among the healthy children and adolescents aged 1-18 years who referred to the endocrinology clinic of Amirkola Children's Hospital for growth evaluation. After overnight fasting, early morning blood samples were obtained to measure the ALP level and other biochemical parameters using an automatic biochemical analyzer. Subjects were categorized by age, sex, and body mass index (BMI) values. The age groups were categorized as follows: 1-4 years, 5-8 years, 9-13 years, and 14-18 years.There was a significant difference among the age and sex categories; on the contrary, there was no meaningful variation between the two groups categorized by BMI. The reference range for ALP was 474.14-517.71 U/L for children aged 1-4 years, 273.47-871.44 U/L for 5-8 years, 215.04-893.69 U/L for 9-13 years, and 228.9-739.22 U/L for 14-18 years. Also, significant positive correlation was found between ALP with length (P=0.000, r=0.134), weight (=0.04, r=0.073), phosphorus (P) (P=0.001, r=0.122), and alanine aminotransferase (SGPT) (P=0.000, r=0.142) respectively.This project's data established a reference interval for ALP in healthy children and adolescents, which will prepare a basis for diagnosis and monitoring liver- or bone-related disorders.

The New coronavirus (SARS COV-2) can cause acute respiratory disease and also multiorgan dysfunction. There is insufficient data about kidney involvement in children. So, this study was done on children with COVID-19 to evaluate nephrological involvement.All children with confirmed or suspected COVID-19 who were admitted in Children Hospital .were enrolled. They were admitted in hospital from March 2020 to July 2020. Serum Blood Urea Nitrogen (BUN), creatinine, sodium, potassium, calcium and urinalysis were evaluated. Also, glomerular filtration rate (GFR) was calculated by Schertz's formula. All patients were evaluated by chest x-ray and/or computerized tomography scanning (CTS). The data were analyzed by SPSS software and P value less than 0.05 was determined as significant.Forty-seven children with confirmed or suspected COVID-19 were enrolled to this study. At admission, 23.4% and 27.7% of children with COVID-19 infection had abnormal increase in serum BUN and creatinine, respectively. Also 78.8% and 25.5% of children had GFR less than 90 and 60 ml/min /1.732, respectively. Additionally, 13/47 (27.7%) of children had abnormal urine analysis (microscopic hematuria and/or proteinuria). There wasn't a significant relationship between pulmonary lesions and abnormal reduction of GFR (P0/05).In the study, the risk of AKI (acute kidney injury) and decrease of GFR and also abnormal urinalysis is high in children with COVID-19. So, more attention for detection of kidney involvement is necessary and more conservative management for prevention of AKI and decrease of GFR are recommended.

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.

Methylmalonic acidemia (MMA), an inherited metabolic disease, results from genetic defects in methylmalonyl-CoA mutase or any of the proteins involved in adenosylcobalamin synthesis. This enzyme is classified into several complementation groups and genotypic classes. In this work we explain the biochemical, structural and genetic analysis of 25 MMA patients, from Iran. The diagnosis was established by the measurement of propionylcarnitine in blood using tandem mass spectrometry and confirmed using a gas chromatography-flame ionization detector. Using clinical, biochemical, structural and molecular analyses we identified 15 mut MMA, three cblA, one cblB, and four cblC-deficient patients. Among mutations identified in the MUT gene (MUT) only one, the c.1874A>C (p.D625A) variant, is likely a mut- mutation. The remaining mutations are probably mut0. Here, we present the first molecular analysis of MMA in Iranian patients and have identified eight novel mutations. Four novel mutations (p.D625A, p.R326G, p.V157F, p.F379L) were seen exclusively in patients from northern Iran. One novel splice site mutation (c.2125-3C>G) in MUT and two novel mutation (p.N225M and p.A99P) in the MMAA gene were associated with patients from eastern Iran. The rs184829210 SNP was recognized only in patients with the novel c.958G>A (p.A320T) mutation. This study confirms pathogenesis of deficient enzyme activity in MUT, MMAA, MMAB, and MMACHC as previous observations. These results could act as a basis for the performance of pharmacological therapies for increasing the activity of proteins derived from these mutations.

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.

Background: Hyperglycemia is one of the most complications of corticosteroid and asparaginase during induction phase of chemotherapy in children suffering from acute lymphoblastic leukemia (ALL). This study was carried out to evaluate the incidence of hyperglycemia and associated risk factors during chemotherapy induction phase at Amirkola Children's Hospital. Materials and Methods: In this cross-sectional (retrospective) study, 150 children (mean age: 79.16±42.68 months) with ALL were evaluated (2000- 2011). Hyperglycemia was described as random blood glucose level more than 200mg/dl in patients less than 2 years old. In patients older than 2 years, fasting blood glucose level more than 110-125 mg/dl was considered as impaired glucose level and fasting blood glucose level more than 126 mg/dl was defined as diabetes mellitus. The data were analyzed using SPSS (version 18) and running chi square test, pearson Ccorrelation, and logistic regression. P-values less than0.05 was considered statistically significant. Results: Out of 150 children with ALL, 21 (14%) of them had hyperglycemia, but none of them had diabetic ketoacidosis. Hyperglycemia was significantly associated with gender (P=0.014) and age. (P=0.000) which was more likely in patients older than 10 years. The incidence of hyperglycemia was also related to BMI (P=0.000). Relapse rate for ALL was 14.7%, which was not significantly associated with hyperglycemia. Conclusion: Hyperglycemia was common and transient during induction phase of chemotherapy and it was correlated with age, sex, and weight.