FAMILY HISTORY OF DIABETES IS ASSOCIATED WITH INCREASED RISK OF RECURRENT DIABETIC KETOACIDOSIS IN PEDIATRIC PATIENTS.

Objective: To determine the relationship between family history of diabetes mellitus (DM) and diabetic ketoacidosis (DKA) recurrence in youth with established type 1 diabetes mellitus (T1DM). Methods: We performed a retrospective chart review of patients with DKA admitted to a pediatric hospital between January, 2009, and December, 2014. We compared patients with recurrent (≥2 admissions) and nonrecurrent DKA (1 admission) and investigated patient level factors, including family history, that may be associated with DKA recurrence in pediatric patients with established T1DM. Results: Of the 131 subjects in the study, 51 (39%) subjects were in the recurrence group. Age ≥15 years old, public health insurance, and family history of T1DM or type 2 diabetes mellitus were associated with recurrent DKA admissions in both univariable and multivariable analyses. Family history was associated with DKA recurrence, with an incidence rate ratio of 1.5 (95% confidence interval = 1.0 to 2.3; P = .03). The association was not explained by type of familial diabetes, first degree relative status, or whether the family member lived in the household. Conclusion: Recognition that a positive family history of DM may be associated with a higher risk for DKA recurrence in patients with established T1DM may allow for targeted education and focus on a previously unidentified population at increased risk for DKA. Understanding the mechanism underlying the effect of family history of diabetes on the rates of DKA in patients with established T1DM may allow for improved identification and education of patients who may be at risk for DKA recurrence. Abbreviations: CI = confidence interval; DKA = diabetic ketoacidosis; EHR = electronic health record; IBD = inflammatory bowel disease; IRR = incidence rate ratio; T1DM = type 1 diabetes mellitus; T2DM = type 2 diabetes mellitus.

Facilitating the transition from paediatric to adult care in endocrinology: a focus on growth disorders.

PURPOSE OF REVIEW: Many childhood-onset growth disorders (COGDs) require continued care into adulthood, and the time of transition between paediatric and adult providers carries a high risk for interruptions in medical care and consequent worsening of disease management. RECENT FINDINGS: Research into best practices for healthcare transition (HCT) describes three distinct stages. Stage 1, transition planning and preparation, begins in the paediatric setting during early adolescence and ensures that the patient has adequate medical knowledge, self-management skills, and readiness for transition. Stage 2, transfer to adult care, occurs with variable timing depending on transition readiness and is best facilitated by warm hand-offs and, when possible, joint visits with the paediatric and adult provider(s) and/or involvement of a care coordinator. Stage 3, intake and integration into adult care, entails retaining the patient in the adult setting, ideally through the involvement of a multidisciplinary approach. SUMMARY: This review covers general principles for ensuring smooth transition of adolescents and young adults (AYA) with COGD, disease-specific medical considerations for paediatric and adult endocrinologists during the transition process, and general and disease-specific resources to assess transition readiness and facilitate transition.

Psychological and Behavioral Predictors of Weight Loss in the Reach Ahead for Lifestyle and Health-Diabetes Lifestyle Intervention Cohort.

BACKGROUND: Understanding the effect lifestyle intervention (LI) has on important psychological and behavioral variables that are associated with weight loss can help inform LI design, content, and delivery. OBJECTIVE: The aim was to determine the modifiable psychological and behavioral factors that are associated with percent weight loss (%WL) and their relative importance in predicting %WL at 12, 24, and 36 months in the REAL HEALTH-Diabetes randomized controlled trial LI. DESIGN: This is a secondary analysis of LI arms of the REAL HEALTH-Diabetes randomized controlled trial LI cohort over a 24-month intervention period and 12-month follow-up period. Patient-reported outcomes were measured using validated questionnaires that were either self-administered or administered by a research coordinator. PARTICIPANTS/SETTING: Adults with type 2 diabetes and overweight/obesity (N = 142) from community health centers, primary care, and local endocrinology practices affiliated with Massachusetts General Hospital in Boston, MA, between 2015 and 2020, were randomized to LI and were included in the analysis. INTERVENTION: The LI was a lower intensity adaptation of Look Action for Health in Diabetes's (HEALTH) evidence-based LI delivered either in-person or via telephone. Registered dietitians delivered 19 group sessions during the first 6 months followed by 18 monthly sessions. MAIN OUTCOME MEASURES: The association of psychological (diabetes-related distress, depression, autonomous motivation, diet and exercise self-efficacy, and social support for healthy behaviors) and behavioral (fat-related diet and dietary self-regulation) variables with %WL. STATISTICAL ANALYSIS: Baseline and 6-month change scores in psychological and behavioral variables were modeled as predictors of %WL at 12, 24, and 36 months using linear regression. Random forests were used to compare the relative importance of changes in the variables in predicting %WL. RESULTS: Six-month improvement in autonomous motivation, exercise self-efficacy, diet self-efficacy, and dietary self-regulation were associated with %WL at 12 and 24 months, but not at 36 months. Improvement in fat-related diet behavior and depressive symptoms were the only variables associated with %WL at all three timepoints. Autonomous motivation, dietary self-regulation, and low-fat diet behaviors were the three most important predictors of %WL during the 2-year LI. CONCLUSIONS: The REAL HEALTH-Diabetes randomized controlled trial LI resulted in 6-month improvements in modifiable psychological and behavioral factors that were associated with %WL. LI programs for weight loss should focus on skills and strategies to promote autonomous motivation, flexible dietary self-regulation, and habituation of low-fat eating habits during the intervention period.

A novel approach to hypophysitis: outcomes using non-glucocorticoid immunosuppressive therapy.

OBJECTIVE: To determine pituitary function before and after nonglucocorticoid immunosuppressive therapy (NGIT) in subjects with hypophysitis and evaluate their clinical and radiologic outcomes. DESIGN: Retrospective, longitudinal study. METHODS: We reviewed a large database, selected subjects with hypophysitis treated with NGIT, and collected information on the duration of therapy, and clinical, hormonal, and radiologic outcomes. RESULTS: Twelve subjects met the inclusion criteria. Five subjects had primary hypophysitis (PH), while seven had secondary hypophysitis (SH) due to an underlying systemic inflammatory disease. Mean age ± SD was 48.0 ± 15.7 years and 40.9 ± 13.0 years, for PH and SH, respectively. The majority were female (PH 60% and SH 86%). BMI ± SD at presentation was 25.2 ± 2.5 kg/m2 and 26.8 ± 6.7 kg/m2 for PH and SH, respectively. The most common symptom at presentation was fatigue (75%). All PH subjects (100%) and 2 (28.6%) SH subjects had polyuria/polydipsia. There was a significant decrease in mean pituitary stalk thickness after NGIT (P = .0051) (mean duration 16.5 ± 4.8 months). New hormone loss or recovery occurred rarely. Mycophenolate mofetil was the most used NGIT: adverse effects prompted discontinuation in 2 out of 7 subjects. CONCLUSIONS: Subjects with hypophysitis receiving NGIT had stable or improved brain/pituitary magnetic resonance imaging findings with a significant decrease in pituitary stalk thickness. NGITs did not improve anterior pituitary function. Our findings suggest that NGIT may be considered as an alternative therapy for patients with hypophysitis who require immunosuppression.

Craniosynostosis as the Presenting Feature of X-linked Hypophosphatemic Rickets.

Craniosynostosis is the premature closure of cranial sutures. Primary, or congenital, craniosynostosis is often sporadic but may be associated with genetic or chromosomal abnormalities. Secondary craniosynostosis presents after gestation, and can occur in metabolic bone diseases, including rickets. We describe the first reported cases of primary craniosynostosis in 2 unrelated, term infants with X-linked hypophosphatemic rickets (XLH). The diagnosis of XLH in both patients was confirmed by genetic testing. At the time craniosynostosis was detected, the patient in the first case did not have any other clinical features of XLH. The second patient developed clinical findings of craniosynostosis, followed by rickets. These are the earliest reported cases of craniosynostosis in XLH and demonstrate that craniosynostosis may be a presenting feature of this disease.