Improving depression screening for adolescents with type 1 diabetes.

OBJECTIVE: Depression is common among adolescents, but rates increase significantly in the presence of chronic health conditions. Outpatient screening for depression is recommended but rarely formally conducted due to barriers of implementation. METHODS: To provide a model for depression screening of youth with chronic health conditions, a standard process using a self-administered electronic version of the Children's Depression Inventory (CDI) was developed. Quality improvement methodology and traditional analytic approaches were used to test the feasibility and outcomes of routine screening in patients 13 to 17 years of age with type 1 diabetes. RESULTS: Of the 528 eligible adolescents, 509 (96%) received at least 1 depression screen during the first year. The process was tested and refined in over 1200 patient encounters, which resulted in an increase in depression screening rates from <5% to a median of 85% over the initial 12 months. Both patients and staff reported acceptance of screening on qualitative surveys. Elevated CDI scores (≥ 16) were found in 8% of the sample; moderate scores (10-15) in 12% of the sample. Low risk scores were found in 80% of the sample. Higher CDI scores correlated with lower blood glucose monitoring frequency and higher hemoglobin A1c, confirming the link between more depression symptoms and poorer diabetes management and control. Suicidal ideation was endorsed in 7% of the population. CONCLUSIONS: Systematic depression screening in adolescents with type 1 diabetes can be reliably implemented with clinically significant results. A systematic approach, such as described in this study, can serve as a model for other chronic health conditions.

Complexities of gender assignment in 17ß-hydroxysteroid dehydrogenase type 3 deficiency: is there a role for early orchiectomy?

BACKGROUND: 17ß-Hydroxysteroid dehydrogenase type-3 (17ßHSD-3) deficiency is a rare cause of 46,XY disorders of sex development. The enzyme converts androstenedione to testosterone, necessary for masculinization of male genitalia in utero. 17ßHSD-3 deficiency is frequently diagnosed late, at puberty, following virilization, with consequent female-to-male gender reassignment in 39-64%. The decision for sex of rearing is difficult, especially if diagnosed in early childhood. Consensus guidelines are equivocal or support male gender assignment. Long-term outcomes data to guide decisions are also lacking; however, in the few cases of early diagnosis and orchiectomy, female gender retention appears more likely.We report two patients with 17ßHSD-3 deficiency, who presented at unusual ages, in whom female gender was chosen. We performed a focused literature review and summary of gender outcomes in 17ßHSD-3 deficiency following early orchiectomy. CASES: Patient A was a phenotypic female who presented at one year of age with bilateral inguinal hernias and external female genitalia. Testes were identified at surgery. The karyotype was 46,XY. She was initially diagnosed with complete androgen insensitivity syndrome; however, androgen receptor mutation analysis was negative. Human chorionic gonadotropin stimulation yielded a low testosterone: androstenedione ratio (0.6, normal >0.8). Genetic testing demonstrated compound heterozygosity for two known mutations of the HSD17B3 gene. She underwent bilateral orchiectomy at two years of age.Patient B was born with female genitalia and virilized at 13 years of age. She did not seek evaluation until 22 years of age. Her karyotype was 46,XY. She had bilateral inguinal testes and low testosterone: androstenedione ratio (0.3). HSD17B3 gene sequencing showed her to be a compound heterozygote for two known mutations. She identified herself as female and underwent bilateral orchiectomy and estrogen replacement therapy. CONCLUSIONS: These two patients highlight the complexities of diagnosis and management in 17ßHSD-3 deficiency. Although existing data are limited, early orchiectomy is likely to result in retention of female gender identity, avoiding the complications related to virilization in adolescence. As such, it is important to pursue a definitive diagnosis to guide clinical decisions, and to have the support and long term follow up with an inter-disciplinary disorders of sex development team.

Who's behind the wheel? Driving with medically intractable epilepsy.

OBJECTIVES: To examine driving in the context of people with medically intractable epilepsy, a population generally discouraged from driving. METHODS: This study tested the relationship between a comprehensive set of factors and driving among 190 patients with a currently valid driver's license who were being evaluated for epilepsy surgery. RESULTS: Almost one third (32.6%) of the sample reported currently driving. Distributional comparisons by current driving status revealed driving was significantly more prevalent among patients who were employed full-time and those who had not experienced a seizure-related MVA. CONCLUSIONS: The decision not to drive in the context of a chronic medical condition is complex and influenced by a myriad of factors such as perceived illness susceptibility and severity.