Neurofibromatosis-Noonan syndrome and growth deficiency in an Iranian girl due to a pathogenic variant in NF1 gene.

BACKGROUND: Mutations in NF1 gene could cause allelic disorders with clinical spectrum of Neurofibromatosis type 1 to Noonan syndrome. Here, a 7-year-old Iranian girl is described with Neurofibromatosis-Noonan syndrome due to a pathogenic variant in NF1 gene. METHODS: Clinical evaluations were performed along with genetic testing using whole exome sequencing (WES). The variant analysis including pathogenicity prediction was also done using bioinformatics tools. RESULTS: The chief compliant of the patient was short stature and lack of proper weight gain. Other symptoms were developmental delay, learning disability, inadequate speech skill, broad forehead, hypertelorism, and epicanthal folds, low set ears and webbed neck. A small deletion, c.4375-4377delGAA, was found in NF1 gene using WES. This variant was classified as pathogenic according to ACMG. CONCLUSIONS: NF1 variants may show variable phenotypes among the patients; identifying such variants is helpful in therapeutic management of the disease. WES is considered as an appropriate test to diagnose Neurofibromatosis-Noonan syndrome.

Predicting childhood overweight status by accelerated weight gain from neonatal period to infancy.

Background: The increased prevalence of obesity in early childhood is a public health problem. Childhood obesity may affect cardiorespiratory fitness and can induce obesity and its comorbidities in adulthood. We aimed to assess childhood overweight status by accelerated weight gain during infancy. Materials and Methods: This is a historical cohort that was conducted on 637 7-year-old students of Guilan province, north of Iran. Data were collected, including demographic characteristics, weight at 4, 6, 12, and 18 months, and clinical examination. The ROC curve was designated based on the standardized z-scores, and the most appropriate cutoff point by sensitivity and specificity was noted for predicting obesity at 7 years. Rapid weight gain (RWG) was also assessed. Results: Among participants, 334 (53.3%) were female. In this study, the mean and standard deviation of RWG in 0-4 months, 0-6 months, 0-12 months, and 0-18 months were 3.50 ± 0.89, 4.64 ± 1.02, 6.54 ± 1.21, and 8.00 ± 1.46 kg, respectively. The highest AUC was dedicated to 0-18 months (0.7 ± 0.05) and the suitable cut-off for RWG in this interval was 8.55 kg with 65.5% and 72.0% sensitivity and specificity, respectively. Conclusion: Although in the previous investigations, the changes in the first 3 years of life had a significant role in further complications, regarding our results, it seems that even earlier consideration of excess weight gain may be necessary.

The Impact of STAT3 rs1053005 Variation on Type 1 Diabetes Mellitus Susceptibility: Association Study and in Silico Analysis.

AIMS: Type 1 diabetes (T1DM) is an autoimmune disorder with multiple genetic and environmental risk factors that are still poorly understood. The signal transducer and activator of transcription (STAT) proteins play a pivotal role in immune-cell genesis and regulation. This study aimed to determine the effect of rs1053005 single nucleotide polymorphism (SNP) in 3'-UTR of STAT3 mRNA on the susceptibility to T1DM in an Iranian population. METHODS: PCR-RFLP was conducted on 250 T1DM patients and 250 control cases to assess STAT3 rs1053005 polymorphism. Moreover, several bioinformatics tools were employed to identify the candidate miRNAs targeting the STAT3 mRNA region under study as well as the effect of rs1053005 on their binding site. RESULTS: Significant variations in the distribution of genotypes and alleles were seen between cases and controls. The comparison results of the frequency of AA, AG, and GG genotypes between T1DM patients and control groups were 49.2% versus 64.8%, 39.2 versus 30%, and 11.6 versus 5.2%, respectively. Individuals who carried GG genotype were at 2.93-fold increased risk of developing T1DM and the G allele was associated with 1.79-fold higher T1DM risk. Bioinformatics analysis demonstrated that due to rs1053005, the interaction of 3 miRNAs were broken, 3 were weakened, 2 were reinforced, and 4 binding sites were created. CONCLUSION: The result of this study indicates an association between STAT3 rs1053005 and T1DM susceptibility which may be due to interference of the SNP with native-binding site of some predicted miRNAs.

Platelet Indices Alterations in Children With Type 1 Diabetes Mellitus.

Platelet (PLT) hyperactivity is a key factor which contributes to cardiovascular complications in patients with type 2 diabetes mellitus even in preclinical stages of disease. To the best of our knowledge, there is limited researches in this regard among patients with type 1 diabetes. The aim of this study was to evaluate hematologic indices indicating PLT activity in children with type 1 diabetes. This was a case-control study which was conducted on 166 inpatients in 17 Shahrivar children hospital, Rasht, Iran during April 2016 to April 2017. Cases and controls were 83 children with type 1 diabetes mellitus and 83 children hospitalized for thorough assessment of short stature, respectively. Groups were matched for age and sex. Demographic characteristics and hematologic variables were assessed. The Shapiro-Wilk test was used to determine the normality of the distribution. Results for continuous and categorical variables were demonstrated as mean±SD and number and percent, respectively. Continuous variables without normal distribution were demonstrated as median (interquartile range). The χ/Fisher's exact test was used to compare categorical variables. The normal and non-normal distributed quantitative variables were respectively assessed by independent T-test or Mann-Whitney U test. P-value <0.05 noted statistical significance. The median (interquartile range) age of all children was 10 (6 to 13) years old. Thirty-five (42.2) of patients with diabetes and 35 (42.2) of control group were male individuals. There were positive correlation between age (r=0.370; P=0.001), hemoglobin (r=0.278; P=0.009), blood sugar (r=0.243; P=0.027), PLT distribution width (r=0.229; P=0.038), plateletcrit (PCT) (r=0.290; P=0.008), PLT to lymphocyte ratio (r=0.230; P=0.037) and glycosylated hemoglobin in children with diabetes. The cut-off point of PCT was 0.19 (sensitivity=87.8%, specificity=66.7%). Only increased PCT (>0.19) was related with poor metabolic control and can put the patients to the risk of future cardiovascular events. The authors recommend considering multiple PLT parameters, and not just one of them, and even designing a scoring system in terms of PLT parameters for type 1 diabetes mellitus management programs.

The Cutoff of Ferritin for Evaluation of Hypothyroidism in Patients With Thalassemia.

BACKGROUND: Thalassemia major is a genetic disease with a recessive autosomal pattern of inheritance that occurs as a result of disorder in hemoglobin synthesis. Researchers aimed to investigate the cutoff of ferritin for the development of hypothyroidism in patients with thalassemia major. MATERIALS AND METHODS: This was a retrospective analytic cross-sectional study that was conducted on the medical records of patients with thalassemia major. Overt hypothyroidism was defined as thyroid stimulating hormone (TSH) level of >6.5 mIU/L and T4 level of < 4.2 ng/dL and subclinical hypothyroidism was defined as TSH level of >6.5 mIU/L and T4 level of >4.2 ng/dL. Data were gathered by a form including age, sex, weight, height, body mass index, thyroid test results, and the mean of last 3 consecutive hemoglobin and ferritin levels. RESULTS: In this study, 67 patients were evaluated. The mean age of the participants was 15.37±3.73 years. The frequency of subclinical hypothyroidism was 10.4%, and no cases of clinical hypothyroidism were noted as well as secondary hypothyroidism. Although there was a significant linear correlation between mean ferritin level and TSH (P=0.008), no significant correlation was noted between levels of ferritin and T4 levels. As ferritin was significantly correlated with TSH, the results showed that the mean serum level of ferritin in ß-thalassemia major patients with hypothyroidism was higher than that in ß-thalassemia major patients with normal thyroid status (P=0.013). The cutoff point for ferritin was 1953 ng/mL (sensitivity=85.7%, specificity=60%). CONCLUSION: It seems that considering the development of hypothyroidism by reaching the ferritin cutoff point, intensification of the iron chelation regimen along with a shorter interval for laboratory endocrine examinations can be recommended.

Assessment of Serum Folic Acid and Homocysteine in Thalassemia Major Patients Before and After Folic Acid Supplement Cessation.

BACKGROUND: Thalassemic patients have ineffective erythropoiesis. In recent treatment protocols, there are little data on folic acid supplementation for patients with thalassemia because it is supposed that regular blood transfusions prevent bone marrow hyperfunctioning. OBJECTIVE: Investigators aimed to assess serum folic acid and homocysteine (Hcy) in thalassemia major patients before and after folic acid supplement cessation. PATIENTS AND METHODS: This study was a before-after controlled clinical trial conducted in 17th Shahrivar Hospital, Rasht, North of Iran, during May to October 2016. The patients enrolled in this study had thalassemia major on regular blood transfusion and older than 2 years of age. They had at least a 6-month history of folic acid supplement consumption before enrollment in the study (1 mg/daily). Complete blood count, serum folic acid, and serum Hcy were measured before discontinuation of folic acid supplement. Then, patients did not receive folic acid for a month and after 1 month of folic acid cessation, the measurements were repeated. All data were entered in SPSS version 20.0 and analyzed. RESULTS: Among the 40 patients in this study, 25 (62.5%) were female. The mean age of the participants was 21.39±11.17 years old. The mean of body mass index was 21.38±3.32 kg/m. Most of the participants had used folic acid supplement >5 years (29, 72.5%). The serum Hcy level was significantly increased (5.24±2.35 vs. 5.93±2.56; P=0.008) and serum folic acid level was decreased significantly (14.74±4.20 vs. 8.80±4.16; P<0.0001) from baseline. CONCLUSIONS: Cessation of folic acid supplementations in beta thalassemia major patients can lead to a significant decrease in serum folic acid and increase in Hcy levels. According to our findings and efficacy of folic acid in patients with beta thalassemia major, it is recommended to use the supplementation in all patients.

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.

The Role of Metabolic Syndrome Components in Sensorineural Hearing Loss in Adolescents: A Case-Control Study.

Introduction Metabolic syndrome (MetS) and its associated components were reported as a possible cause of inner ear dysfunction. However, research about the influence of cardiovascular risk factors on hearing thresholds are conducted mainly in adult patients. Objective The aim of the present study was to investigate auditory function in adolescents with MetS compared with healthy controls. Methods One hundred adolescents with metabolic syndrome and 200 sex- and age-matched controls were recruited from a university pediatric endocrine clinic from May 2018 to July 2020. Hearing loss was defined as hearing level ≥ 15 dB at speech frequency (SFHL) or high frequency (HFHL) in one or both ears. A multivariable conditional logistic regression analysis examined the correlation between MetS components and several important demographic characteristics, and hearing loss. Results A total of 165 (55.0%) boys and 135 (45.0%) girls participated in this study. The rates of SFHL and HFHL in adolescents with MetS were 32.0% and 51.0%, respectively. Those values for controls were 5.0% and 15.5%, respectively. The regression analysis showed high triglycerides as a significant predictor for SFHL (odds ratio 10.87; 95% confidence interval: 1.98, 59.74). Neither predictor of interest was significant for HFHL. Conclusion Hypertriglyceridemia may be an important factor in the pathogenesis of SFHL. However, the strength of the association was not significant with a wide confidence interval. Also, we were unable to find an association between predictors and HFHL with the current sample size. Larger and prospective studies are recommended.

The Consensus on the Diagnosis and Management of Congenital Hypothyroidism in Term Neonates.

Congenital hypothyroidism (CH) is one of the most treatable endocrine disorders in infants and children that can influence the function of many organs in the body. On-time diagnosis and treatment can prevent the adverse effects of thyroid hormone deficiency on the child's neurodevelopment. There are many challenges in screening, post-screening, diagnosis, and managing this disorder. Therefore, this article aimed to mention updated information on this issue. Although there are different approaches for the treatment of hypothyroidism, the authors decided to create a national approach based on the conditions of our country.

Effect of concurrent resistance-aerobic training on inflammatory factors and growth hormones in children with type 1 diabetes: a randomized controlled clinical trial.

BACKGROUND: Exercise training is a major factor in controlling type 1 diabetes mellitus (T1DM) in children. The present study aimed to assess the effect of concurrent resistance-aerobic training on selected inflammatory factors and hormones related to blood glucose homeostasis in children with T1DM. METHODS: In this randomized controlled clinical trial, 40 children (with the mean age of 11.11 ± 2.29 years) were randomly assigned to an experimental (N = 20) or control group (N = 20). They underwent a 16-week training program, composed of concurrent resistance-aerobic training performed intermittently for 60 min three times a week. Before and after training, blood samples were analyzed for glucose homeostasis, selected inflammatory factors, and growth factors. Data were analyzed by paired t-test and analysis of covariance (ANCOVA) in IBM SPSS version 22. RESULTS: The exercise training intervention reduced fasting blood sugar index (P = 0.002) and glycosylated hemoglobin significantly (P = 0.003). The growth hormone levels were increased significantly only in the experimental group (P = 0.037), whereas no significant difference was noted in the insulin-like growth factor-1 (P = 0.712). It was also found that interleukin-1ß and high-sensitivity C-reactive protein did not change in the experimental or control group as compared to the pretest (P > 0.05). CONCLUSION: As it was shown, it seems that concurrent resistance-aerobic training may improve blood glucose homeostasis and growth hormone. Therefore, these findings may suggest the benefit from exercise training of moderate intensity in children with T1DM. Besides, we recommend undertaking further clinical trials to determine if the exercise training was effective. TRIAL REGISTRATION: This study was registered in the Iranian Registry of Clinical Trials under the code IRCT20150531022498N30: https://en.irct.ir/trial/41031 . Registered on July 26, 2019. All experiments on the participants were following the Declaration of Helsinki.

The association between OSA and glycemic control in diabetes.

Background: Obstructive sleep apnea (OSA) is the most common sleep-realted respiratory disorder. It is frequently comorbid with cardiovascular, cerebrovascular, and metabolic diseases and is commonly observed in populations with these comorbidities. Investigators aimed to assess the effect of OSA on glycemic control in patients with diabetes. Methods: In this cross-sectional study, 266 adult patients with diabetes mellitus (DM) attending the outpatient endocrinology clinic at the Guilan University of Medical Sciences were enrolled. Patients completed a checklist that included demographic characteristics, factors, and laboratory results in addition to Berlin and STOP-BANG questionnaires to evaluate the risk of OSA. Data were analyzed by independent t-test, Mann-Whitney U test, and Chi-squared or Fisher's exact tests using the Statistical Package for the Social Sciences (SPSS) version 17. Results: A total of 266 patients with DM were enrolled in this study (34.6% males, mean age 47.00 ± 19.04 years). Based on the Berlin Questionnaire, 38.6% of all participants were at high risk of developing OSA. Based on the STOP-BANG Questionnaire (SBQ), 45.1% were at moderate and high risks. Additionally, this questionnaire showed a significant difference between low and moderate-to-severe groups regarding sex, age, body mass index (BMI), neck size, other chronic diseases, types of DM, use of insulin, Berlin Questionnaire, fasting blood sugar (FBS), and mean HbA1c. Conclusions: Based on the SBQ, our results indicated a significant relationship between OSA and glycemic control according to mean HbA1c and FBS. Therefore, by controlling the OSA, we may find a way to acheieve better glycemic control in diabetic patients.

Preventable Prenatal and Neonatal Risk Factors of Type 1 Diabetes in Childhood.

Background: Childhood type 1 diabetes mellitus (T1DM) is an autoimmune disease which is increasing in incidence, but little is known about the events that trigger the autoimmune process. Most of the time, these processes begin in prenatal and natal periods; therefore, this study aimed to investigate the prenatal and neonatal risk factors of T1DM in childhood. Methods: This case-control study has been performed on children with T1DM who referred to the 17th Shahrivar children's hospital. The control group consisted of healthy siblings of the case group. Data were gathered using a form that included maternal and neonatal characteristics. Data were reported by descriptive statistics in SPSS 19. To investigate the effect of quantitative and qualitative variables on the development of T1DM, logistic regression and Chi-square tests were used, respectively. Results: Birth weight, birth height, and maternal weight gain during pregnancy had a significant relationship with T1DM (odds ratio [OR] = 1.23, 2.57, and 1.14, respectively). In addition, there was a significant relationship between gestational hypertension (OR = 5.27), neonatal jaundice (OR = 3.42), cesarean section (OR = 2.06), and being non-first-born child (OR = 2.32) and T1DM. Also, premature rupture of membrane, maternal urinary tract infection, and nonexclusive breastfeeding had a significant association with T1DM (OR = 4.37, 3.94, and 2.30, respectively). There were no statistically significant differences between maternal age, sex, neonatal respiratory disease, prematurity, and neonatal infections and T1DM (P > 0.05). Conclusions: Prenatal and neonatal risk factors can have a significant role in the occurrence of TIDM. Therefore, considering these risk factors can have a preventive effect on T1DM.

Inborn Errors of Metabolism Associated With Autism Among Children: A Multicenter Study from Iran.

OBJECTIVE: This study aimed to find the common inborn errors of metabolism in Iranian patients with autism spectrum disorder. METHODS: In this cross-sectional multicenter study, 105 children and adolescents with autism spectrum disorder from six centers in different cities of Iran were enrolled between August, 2019 and October, 2020. Metabolic screening, including measuring plasma levels of amino acids, acylcarnitines, creatine, and guani-dinoacetate, and urinary levels of organic acids, purines, and pyrimidines was performed. Other data, including age, parental consanguinity, history of seizure, developmental mile-stones, and physical examination, were also recorded. RESULTS: An inborn error of metabolism was found in 13 (12.4%) patients. Five patients (4.8%) had cerebral creatine deficiency syndrome, 4 (3.8%) had arginine succinate aciduria, 2- methylbutyryl glycinuria, short-chain acyl-CoA dehydrogenase deficiency, and combined methylmalonic aciduria/malonic aciduria. There was a strong association between positive meta-bolic evaluation and parental consanguinity, history of seizures, microcephaly, and delayed development. CONCLUSIONS: Our results suggest that metabolic screening should be performed in the cases of autism associated with parental consanguinity, developmental delay, and a history of seizures. The assays to be considered as a screening panel include plasma or blood amino acids, acylcarnitines, creatine and guanidinoacetate, and urinary levels of organic acids.

Association Between Poor Sleep Quality and Glycemic Control in Adult Patients with Diabetes Referred to Endocrinology Clinic of Guilan: A Cross-sectional Study.

Background: Diabetes is a prevalent chronic medical comorbid condition worldwide. Diabetes mellitus is associated with various sleep disorders. Objectives: We aimed to determine the prevalence of poor sleep and the main factors of sleep interruptions in patients with diabetes mellitus. We further evaluated the association of sleep interruptions with glycemic control in this cohort. Methods: We conducted a cross-sectional study on 266 patients with type 1 and type 2 diabetes recruited from a university outpatient endocrinology clinic. Patients completed a checklist including demographic and disease-related characteristics in addition to the Pittsburgh Sleep Quality Index (PSQI) to evaluate sleep quality. Using the PSQI cutoff score of 5, we created two subgroups of good sleepers (GS) and poor sleepers (PS). Results: Our results showed that good sleeper and poor sleeper patients with diabetes were significantly different regarding sex, employment status, BMI, presence of diabetes-related complications, HbA1c, and 2-hour postprandial blood sugar (2HPPBS) (all significant at P < 0.05). The most prevalent factors of sleep interruptions were "waking up to use a bathroom", "feeling hot", "pain", "having coughs or snores", and "bad dreams". Among the subjective factors of sleep interruption, problems with sleep initiation, maintenance, or early morning awakenings in addition to having pain or respiratory problems such as coughing or snoring had the most significant associations with HbA1c. Conclusions: Our study showed significant subjective sleep disturbances (both quality and quantity) in patients with diabetes mellitus (both type I and II) and its association with diabetes control. We further identified the main factors that led to sleep interruptions in this cohort.

The Cutoff of Gonadotropins for Close Evaluation of Cardiometabolic Risk Factors in Turner Syndrome.

Background: Turner syndrome is a common genetic disorder in females. It is a disorder characterized by variable number of clinical features, so it needs a multidisciplinary approach for care. Therefore, we aimed to define the cutoff of gonadotropins for close evaluation of cardiometabolic risk factors in Turner syndrome. Methods: This is a case-control study on 31 patients with Turner syndrome and 31 healthy individuals. Clinical examination including blood pressure measurement and systems evaluation was performed. Laboratory testing, which included 12-h fasting, assessed lipid profile, glucose, and serum gonadotropin. Results: Turner syndrome had a higher BMI, systolic, and diastolic blood pressure than the normal group (P < 0.001) Patients with Turner syndrome had significantly higher total cholesterol, low-density lipoprotein, triglyceride, and TG-to-high-density lipoprotein ratio compared to the healthy individuals (P < 0.05). With increasing LH and FSH, BMI values, systolic blood pressure, and total cholesterol increased significantly (P < 0.001). Serum TG levels in Turner syndrome were only positively correlated with LH and not correlated with FSH. The cutoff point of LH and FSH for triglyceride in upper 75 percentile were 31 (sensitivity = 38.1%, specificity = 80%) and 48 (sensitivity = 61.9%, specificity = 70%), respectively. Conclusions: Based on dyslipidemia and lower level of ejection fraction, considering cardiometabolic risk factors in lower age groups in Turner syndrome can be recommended.

Dermoscopic Findings of Alopecia in Patients with Hypothyroidism.

Background: Thyroid hormones are essential for the growth and maintenance of hair follicles. Numerous studies have evaluated the relationship between thyroid disorders and hair loss. However, no study has assessed the dermoscopic results in patients with hypothyroidism and hair loss. Objectives: This study aimed to investigate dermoscopic findings of alopecia in patients with hypothyroidism. Methods: This analytic cross-sectional study was performed on patients with hair loss referred to dermatology clinics of Guilan University of Medical Sciences, Iran. Hypothyroid patients and an equal number of euthyroid individuals were compared. After recording the demographic and clinical characteristics, all patients were subjected to hair dermoscopy. Results: A total of 164 patients with and without hypothyroidism with hair loss were studied. The frequency of hair shaft abnormalities (P < 0.001) and vellus hair (P < 0.001) significantly differed between the two groups. Dermoscopic findings related to scale abnormalities (P = 0.002) and their perifollicular type (P < 0.001) significantly differed between the groups. Vascular changes (P < 0.001), perifollicular concentric type (P = 0.012), and interfollicular red loops type (P = 0.005) were significantly higher in patients with hypothyroidism. Also, based on the multiple logistic regression model, the chance of abnormalities of the hair shaft, scales, and vascular changes increased by 3.24, 2.73, and 3.53, respectively, in hypothyroidism compared to euthyroidism. Conclusions: Regarding the promising results of this study, we could detect possible dermoscopic signs of inflammation in hypothyroid patients with hair loss. Further investigations are needed because there is a shortage of evidence on this novel diagnostic method.

Guideline for the Treatment of Hypothyroidism in Prematurity.

Congenital hypothyroidism is one of the most common endocrine disorders in infants and children. Thyroid hormone effects the function of most organs of the body. In premature neonates, thyroid abnormalities are very common but transient. There is a significant difference between the appropriate time for screening in premature and term neonates and there are different viewpoints in treating hypothyroidism in prematurity. According to the probable exceptions in this issue, there is no definite guideline. Therefore, regarding this confusion, this guideline aimed to help clinicians for rapid on-time decision making.

The effect of concurrent resistance-aerobic training on serum cortisol level, anxiety, and quality of life in pediatric type 1 diabetes.

Background Given the importance of anxiety and quality of life for the mental health of children with type 1 diabetes (T1D), exercise prescription can be of crucial significance. The present study aims to explore the effect of concurrent resistance-aerobic training on serum cortisol level, anxiety, and quality of life among pediatric T1D. Methods Forty children (aged 8-14 years) were randomly assigned to experimental (n = 20) and control groups (n = 20) for 16 weeks. The exercise training program was composed of 16 weeks of interval concurrent resistance-aerobic training with a duration of 60 min performed three times a week. The subjects first performed the resistance training (20 min of Pilates exercises and 20 min of body weight-bearing exercises). Then, the aerobic exercises were performed with an intensity of 50-75% of maximum heart rate. Before and after the training, blood tests including cortisol were carried out on the subjects by RIA kit. Anxiety and quality of life were measured by the Revised Children's Manifest Anxiety Scale (RCMAS) and Pediatric Quality of Life (PedsQL), respectively. Body composition was measured by InBody. Data were analyzed by paired and independent t-test at p < 0.05 significance level. Results Sixteen weeks of concurrent resistance-aerobic exercise significantly reduced the anxiety index (p = 0.001) and increased the quality of life (p = 0.003). Although the cortisol index was increased, it did not reveal any significant differences between the experimental and control groups (p = 0.781). No significant differences were observed in the indices of quality of life, anxiety, and cortisol in the control group. Conclusions A 16-week program of concurrent resistance-aerobic training can improve the quality of life and anxiety among children suffering from T1D, but it may not influence the cortisol level (p > 0.05).

The Association Between Hemoglobin HbA1c with Serum Inorganic Phosphate in Children with Type 1 Diabetes.

BACKGROUND: Diabetes mellitus is one of the most common chronic metabolic diseases in children and adolescents, which changes the cellular metabolism. Phosphorus is an essential element for metabolism. Early in the progression of diabetes, a paradoxical metabolic imbalance in inorganic phosphate (Pi) occurs that may lead to reduced high energy phosphate and tissue hypoxia. While low and high uncontrolled blood sugars can be easily recognized by clinical symptoms, low and high plasma inorganic phosphate remain unrecognizable. Therefore, we aimed to assess the association between hemoglobin A1c (HbA1c) with serum inorganic phosphate in children with type 1 diabetes. MATERIALS AND METHODS: This cross-sectional study was conducted on 102 patients selected from a pediatric ward in 17th Shahrivar hospital in Rasht, North of Iran. Clinical data including age, sex, height, weight, BMI, duration of diabetes, the level of HbA1c, and phosphorus were gathered. The level of HbA1c was adjusted by age in the final analysis. RESULTS: The mean age of samples was 9.98±3.91 years old and 46 participants (45.1%) were male. It was found that HbA1c had a reversed and significant relationship with BMI (r=-0.215 and P=0.03), but there was no correlation between phosphate, age, height and weight, duration of diabetes mellitus, or rate of insulin consumption with HbA1c (P>0.05). CONCLUSION: The finding showed that HbA1c had a reversed relationship with BMI but there was no correlation between phosphate and HbA1c.

Developmental Screening of Children with Congenital Hypothyroidism Using Ages and Stages Questionnaires Test.

OBJECTIVES: Congenital hypothyroidism (CH) is one of the most common causes of mental retardation in children. We investigated the developmental status of children with CH screened by Ages & Stages Questionnaires (ASQ) measurement scores. MATERIALS & METHODS: In this retrospective study, neurodevelopmental status of 78 children diagnosed with CH followed up at the Outpatient Pediatric Endocrinology Clinic of Besat Hospital, Hamadan, Iran from May 2006 to Mar 2013, was evaluated by ASQ method. Data on age, sex, birth weight, birth length, head circumference, residency location, parental education level, primary venous TSH and T4 levels, age at diagnosis, treatment start age and initial levothyroxine dosage were extracted from medical records. Data were analyzed using statistical software SPSS. P-value less than 0.05 was considered statistically significant. RESULTS: Of the 78 patients, 34 (43.6%) were female, 32 (41%) had developmental disorder, and 56 (71.8%) were living in urban areas. Types of developmental impairments included: global motor delay in 13 (40.6%) patients, problem-solving in 11 (34.3%), impaired communication skills in 5 (15.6%), impaired fine motor skills in 2 (6.2%), and impairment of personal social skills in 1 (3.1%). The average ages for diagnosis and treatment were 25.65 days in patients with developmental impairment and 17.99 days in those without developmental delay. ASQ results showed significant statistical correlation with initial dose of levothyroxine (P=0.017), age of hypothyroidism diagnosis (P=0.002) and age of treatment initiation (P=0.018). CONCLUSION: Early diagnosis and treatment along with initial levothyroxine dose were most important factors of ASQ scores of children with CH. Higher dose of the levothyroxine is required at onset.