Cortical bone mineral density in patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency.

BACKGROUND: Prior studies reveal that bone mineral density (BMD) in congenital adrenal hyperplasia (CAH) is mostly in the osteopaenic range and is associated with lifetime glucocorticoid dose. The forearm, a measure of cortical bone density, has not been evaluated. OBJECTIVE: We aimed to evaluate BMD at various sites, including the forearm, and the factors associated with low BMD in CAH patients. METHODS: Eighty CAH adults (47 classic, 33 nonclassic) underwent dual-energy-x-ray absorptiometry and laboratory and clinical evaluation. BMD Z-scores at the AP spine, total hip, femoral neck, forearm and whole body were examined in relation to phenotype, body mass index, current glucocorticoid dose, average 5-year glucocorticoid dose, vitamin D, 17-hydroxyprogesterone, androstenedione, testosterone, dehydroepiandrosterone and dehydroepiandrosterone sulphate (DHEAS). RESULTS: Reduced BMD (T-score <-1 at hip, spine, or forearm) was present in 52% and was more common in classic than nonclassic patients (P = 0·005), with the greatest difference observed at the forearm (P = 0·01). Patients with classic compared to nonclassic CAH, had higher 17-hydroxyprogesterone (P = 0·005), lower DHEAS (P = 0·0002) and higher non-traumatic fracture rate (P = 0·0005). In a multivariate analysis after adjusting for age, gender, height standard deviation, phenotype and cumulative glucocorticoid exposure, higher DHEAS was independently associated with higher BMD at the spine, radius and whole body. CONCLUSION: Classic CAH patients have lower BMD than nonclassic patients, with the most affected area being the forearm. This first study of forearm BMD in CAH patients suggests that low DHEAS may be associated with weak cortical bone independent of glucocorticoid exposure.

Trends in GH use in a Turner syndrome natural history study.

The present observations are derived from 273 girls and women aged 7-40 years participating in the National Institutes of Health natural history study of Turner syndrome (TS) in the interval 2001-2011. There was a higher percentage of GH use among individuals in the pediatric age group (7-17, n = 118, 83%) compared to young adult women with prior GH use (18-40, n = 155, 61%). The major factor in this divergence seems to be a trend toward earlier diagnosis of TS in the younger age group. We find a striking association between history of GH use and lower total body and abdominal fat mass in young adults with TS approximately one decade after discontinuation of GH treatment. The interpretation of this observation is limited by the fact that our study subjects were not randomly assigned to GH treatment. There may be a bias involving poor health care, childhood obesity, delayed diagnosis, absent GH treatment and persistent adult obesity. Further studies on the socioeconomic factors implicated in patterns of GH use and non-use for girls with TS are needed to illuminate this important issue.

A pilot test of the Self-Management and Research Technology project: A text message-based diabetes self-management program for adolescents.

The aims of this study are to: (1) examine the preliminary utility of the Self-Management and Research Technology (SMART) pilot project, (2) identify which adolescents were most likely to benefit from participation, and (3) examine interview feedback to inform future program iterations. Twenty-three adolescents ( Mage = 15.13 years) were enrolled in the six-week text message pilot program consisting of daily interactive blood glucose (BG) prompts and type 1 diabetes-related educational text messages. Medical charts were reviewed for hemoglobin A1c and to corroborate medical record and demographic data. Glucometer data were downloaded to calculate an average monthly BG level and daily BG monitoring frequency. No statistically significant improvements were observed pre-intervention to post-intervention in glycemic outcomes. Participants with a high text message response rate were more likely to demonstrate improvement in average monthly BG levels and daily BG monitoring frequency than those with a low text message response rate. Participants reported satisfaction with the text message program. The text message-based SMART pilot project demonstrated preliminary efficacy for a targeted subset of adolescents who were engaged with the program. Continued research with a larger sample and longer trial duration is warranted to evaluate the potential utility of text message-based interventions.