Pediatric endocrine society survey of diabetes practices in the United States: What is the current state?

BACKGROUND: The Practice Management Committee (PMC) of the Pediatric Endocrine Society (PES) conducted a survey of its membership in February/March, 2016 to assess the current state of pediatric diabetes care delivery across multiple practice types in the United States. METHODS: The PES distributed an anonymous electronic survey (Survey Monkey) via email to its membership and requested that only one survey be completed for each practice. RESULTS: Ninety-three unique entries from the US were entered into analysis. Care is predominantly delivered by multidisciplinary teams, based at academic institutions (65.6%), with >85% of the provider types being physicians. Each 1.0 full time equivalent certified diabetes educators serves on average 367 diabetic youth. Fee-for-service remains the standard method of reimbursement with 57% of practices reporting financial loss. Survey respondents identified under-reimbursement as a major barrier to improving patient outcomes and lack of behavioral health (BH) providers as a key gap in services provided. CONCLUSIONS: Our survey reveals wide variation in all aspects of pediatric diabetes care delivery in the United States. Pediatric Endocrinologists responding to the survey identified a lack of resources and the current fee for service payment model as a major impediment to practice and the lack of integrated BH staff as a key gap in service. The respondents strongly support its organizations' involvement in the dissemination of standards for care delivery and advocacy for a national payment model aligned with chronic diabetes care in the context of our emerging value-based healthcare system.

Accelerated Skeletal Maturation in Disorders of Retinoic Acid Metabolism: A Case Report and Focused Review of the Literature.

Nutritional excess of vitamin A, a precursor for retinoic acid (RA), causes premature epiphyseal fusion, craniosynostosis, and light-dependent retinopathy. Similarly, homozygous loss-of-function mutations in CYP26B1, one of the major RA-metabolizing enzymes, cause advanced bone age, premature epiphyseal fusion, and craniosynostosis. In this paper, a patient with markedly accelerated skeletal and dental development, retinal scarring, and autism-spectrum disease is presented and the role of retinoic acid in longitudinal bone growth and skeletal maturation is reviewed. Genetic studies were carried out using SNP array and exome sequencing. RA isomers were measured in the patient, family members, and in 18 age-matched healthy children using high-performance liquid chromatography coupled to tandem mass spectrometry. A genomic SNP array identified a novel 8.3 megabase microdeletion on chromosome 10q23.2-23.33. The 79 deleted genes included CYP26A1 and C1, both major RA-metabolizing enzymes. Exome sequencing did not detect any variants that were predicted to be deleterious in the remaining alleles of these genes or other known retinoic acid-metabolizing enzymes. The patient exhibited elevated plasma total RA (16.5 vs. 12.6±1.5 nM, mean±SD, subject vs. controls) and 13-cisRA (10.7 nM vs. 6.1±1.1). The findings support the hypothesis that elevated RA concentrations accelerate bone and dental maturation in humans. CYP26A1 and C1 haploinsufficiency may contribute to the elevated retinoic acid concentrations and clinical findings of the patient, although this phenotype has not been reported in other patients with similar deletions, suggesting that other unknown genetic or environmental factors may also contribute.

Metabolic control and quality-of-life self-assessment in adolescents with IDDM.

OBJECTIVE: To examine the relation between metabolic control and self-assessed quality of life in adolescents with IDDM. RESEARCH DESIGN AND METHODS: The Diabetes Quality of Life (DQOL) questionnaire for youths was given to 69 subjects with IDDM aged 10-20 years at the time of their outpatient visit. Subjects with IDDM of < 1 year's duration or with documented psychotic disorder or mental retardation were excluded. Metabolic control was assessed by the mean HbA1c during the preceding year (long-term), by a single HbA1c at the time of the visit (short-term), and by the number of acute events related to IDDM in the preceding year. RESULTS: The DQOL score correlated with mean HbA1c (beta = 6.13, R2 = 0.22, P = 0.0122) and single HbA1c (beta = 3.94, R2 = 0.18, P = 0.05). Self-health assessment was the best predictor of DQOL score (beta = -44.42, R2 = 0.45, P < 0.0001). The Worries subscale score on DQOL correlated with the occurrence of acute events (beta = 6.97, R2 = 0.2, P = 0.006), but did not correlate with either HbA1c level. Correlations of mean HbA1c with the predictors were stronger than the correlations of single HbA1c with the same predictors. CONCLUSIONS: Metabolic control and quality of life are two important outcomes of IDDM care. In our study, adolescents in better metabolic control report better quality of life. Both components need to be addressed in developing successful diabetes treatment strategies for adolescents with IDDM.