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Background: There is a need for a non‐invasive, affordable, and reliable method for bone health screening in pediatric patients at risk. Objective: To assess Bone Health Index (BHI) in pediatric patients with type 1 diabetes (T1D) and its relation to bone metabolism, age at onset, duration, control, and insulin dose. Subjects and methods: Left‐hand radiographs were obtained from 65 patients with T1D, mean age 11.23 ± 3.89 years, mean disease duration 5.23 ± 3.76 years and mean glycosylated hemoglobin (HbA1c)‐83 mmol/mol (9.7%). Blood and 24 hours urine samples were collected for bone and mineral metabolism assessment. BoneXpert was used to determine BHI, Bone Health Index standard deviation score (BHI SDS), and bone age. Results: Mean BHI SDS was −1.15 ± 1.19 (n = 54). In 20.37% (n = 11) BHI SDS was < −2SD with mean value −2.82 ± 0. 69, P <.001. These patients had lower levels of beta cross laps (0.77 ± 0.33 ng/mL vs 1.17 ± 0.47 ng/mL), osteocalcin (47.20 ± 14.07 ng/mL vs 75.91 ± 32.08 ng/mL), serum magnesium (0.79 ± 0.05 mmol/L vs 0.83 ± 0.06 mmol/L) and phosphorus (1.48 ± 0.29 mmol/L vs 1.71 ± 0.28 mmol/L) but higher ionized calcium (1.29 ± 0.04 mmol/L vs 1.26 ± 0.05 mmol/L), P <.05, compared to patients with BHI SDS in the normal range. We found a positive correlation between BHI SDS and age at manifestation (r = 0.307, P = 0.024) and a negative one with disease duration (r = −0.284, P =.038). No correlations were found with HbA1c, insulin dose, height, weight, BMI. Conclusions: To the best of our knowledge, this is the first study to assess bone health in pediatric patients with T1D using BHI. We found significantly decreased cortical bone density and bone turnover in 20.37%. Earlier age at onset and diabetes duration may have a negative impact on cortical bone density in patients with poor control. Longitudinal studies are needed to follow changes or to assess future interventions. [ABSTRACT FROM AUTHOR]

Objective: To assess the prevalence of underweight (UW), overweight (OW), and obesity in children and adolescents with type 1 diabetes (T1D). Methods: An international cross‐sectional study including 23 026 T1D children (2‐18 years, duration of diabetes ≥1 year) participating in the SWEET prospective, multicenter diabetes registry. Body mass index SD score (BMI‐SDS) was calculated using the World Health Organization BMI charts. Children were categorized as UW (BMI‐SDS < −2SD), OW (+1SD < BMI‐SDS ≤ +2SD), and obese (OB) (BMI‐SDS > +2SD). Hierarchic regression models were applied with adjustment for sex, age, and duration of diabetes. Results: The prevalence of UW, OW, and obesity was: 1.4%, 22.3%, and 7.3% in males and 0.6%, 27.2%, and 6.8% in females. Adjusted BMI‐SDS was significantly higher in females than in males (mean ± SEM: 0.54 ± 0.05 vs 0.40 ± 0.05, P < 0.0001). In males, BMI‐SDS significantly decreased by age (P < 0.0001) in the first three age categories 0.61 ± 0.06 (2 to <10 years), 0.47 ± 0.06 (10 to <13 years), 0.34 ± 0.05 (13 to <16 years). In females, BMI‐SDS showed a U‐shaped distribution by age (P < 0.0001): 0.54 ± 0.04 (2 to <10 years), 0.39 ± 0.04 (10 to <13 years), 0.55 ± 0.04 (13 to <16 years). BMI‐SDS increased by diabetes duration (<2 years: 0.38 ± 0.05, 2 to <5 years: 0.44 ± 0.05, and ≥5 years: 0.50 ± 0.05, P < 0.0001). Treatment modality did not affect BMI‐SDS. Adjusted HbA1c was significantly higher in females than in males (8.20% ± 0.10% vs 8.06% ± 0.10%, P < 0.0001). In both genders, the association between HbA1c and BMI‐SDS was U‐shaped with the highest HbA1c in the UW and obesity groups. Conclusions: The high rate of OW and obesity (31.8%) emphasize the need for developing further strategies to prevent and treat excess fat accumulation in T1D. [ABSTRACT FROM AUTHOR]

The objective of this study was to describe the clinical characteristics of syndromic neonatal diabetes in a family with aGATA6mutation. A girl, currently aged 12 years 3 months, was born with intrauterine growth retardation: weight 1600 g (–4.3 SDS) at term. After birth, foramen ovale and patent ductus arteriosus (PDA) were diagnosed by echocardiography. Diabetes was diagnosed on the 9th day after birth. Exocrine pancreatic insufficiency was clinically diagnosed at about 2 years of age and pancreatic agenesis was revealed later by magnetic resonance imaging. Her father had undergone surgery during infancy for PDA and had developed insulin dependent diabetes at 12 years of age. Ultrasound revealed a thin pancreas with normal length and anatomical structure. He has subclinical exocrine pancreatic insufficiency, low insulin needs and no late complications of diabetes up to the age of 40 years. Sequencing ofGATA6identified a heterozygous splicing mutation, 1136-2A>G, in the girl and her father. Testing of the paternal grandparents showed that the mutation was likely to have arisende novoin the father. Identification of aGATA6mutation explains the cardiac anomalies and diabetes in this family. This case highlights the marked intra-familial variability of both exocrine and endocrine pancreatic phenotypes in patients withGATA6mutations. [ABSTRACT FROM PUBLISHER]

Background Intensified insulin delivery using multiple daily injections ( MDI) or continuous subcutaneous insulin infusion ( CSII) is recommended in children with type 1 diabetes ( T1D) to achieve good metabolic control. Objective To examine the frequency of pump usage in T1D children treated in SWEET (Better control in Paediatric and Adolescent diabete S: Working to cr Eate C En Ters of Reference) centers and to compare metabolic control between patients treated with CSII vs MDI. Methods This study included 16 570 T1D children participating in the SWEET prospective, multicenter, standardized diabetes patient registry. Datasets were aggregated over the most recent year of treatment for each patient. Data were collected until March 2016. To assess the organization of pump therapy a survey was carried out. Results Overall, 44.4% of T1D children were treated with CSII. The proportion of patients with pump usage varied between centers and decreased with increasing age compared with children treated with MDI. In a logistic regression analysis adjusting for age, gender and diabetes duration, the use of pump was associated with both: center size [odd ratio 1.51 (1.47-1.55), P < .0001) and the diabetes-related expenditure per capita [odd ratio 1.55 (1.49-1.61), P < .0001]. Linear regression analysis, adjusted for age, gender, and diabetes duration showed that both HbA1c and daily insulin dose (U/kg/d) remained decreased in children treated with CSII compared to MDI ( P < .0001). Conclusions Insulin pump therapy is offered by most Sweet centers. The differences between centers affect the frequency of use of modern technology. Despite the heterogeneity of centers, T1D children achieve relatively good metabolic control, especially those treated with insulin pumps and those of younger age. [ABSTRACT FROM AUTHOR]