Cardiac characterization of 16 patients with large NF1 gene deletions.

The aim of this study was to characterize cardiac features of patients with neurofibromatosis 1 (NF1) and large deletions of the NF1 gene region. The study participants were 16 patients with large NF1 deletions and 16 age- and sex-matched NF1 patients without such deletions. All the patients were comprehensively characterized clinically and by echocardiography. Six of 16 NF1 deletion patients but none of 16 non-deletion NF1 patients have major cardiac abnormalities (p = 0.041). Congenital heart defects (CHDs) include mitral insufficiency in two patients and ventricular septal defect, aortic stenosis, and aortic insufficiency in one patient each. Three deletion patients have hypertrophic cardiomyopathy. Two patients have intracardiac tumors. NF1 patients without large deletions have increased left ventricular (LV) diastolic posterior wall thickness (p < 0.001) and increased intraventricular diastolic septal thickness (p = 0.001) compared with a healthy reference population without NF1, suggestive of eccentric LV hypertrophy. CHDs and other cardiovascular anomalies are more frequent among patients with large NF1 deletion and may cause serious clinical complications. Eccentric LV hypertrophy may occur in NF1 patients without whole gene deletions, but the clinical significance of this finding is uncertain. All patients with clinical suspicion for NF1 should be referred to a cardiologist for evaluation and surveillance.

Proposition 36: issues and challenges.

A discussion of California's Substance Abuse and Crime Prevention Act of 2000 (Proposition 36) and how it represents a paradigm shift from incarceration to treatment and collaboration.

Home-based management of urinary incontinence: a pilot study with both frail and independent elders.

OBJECTIVE: The participation of older rural women and their caregivers in a pilot research study on behavioral management interventions for urinary incontinence is described. DESIGN: A quasiexperimental design was used. SETTING AND SUBJECTS: Women 55 years old and older and living in a rural county in North Florida who had episodes of urinary incontinence twice or more per week were included. Outreach was directed at two groups of elders with incontinence, those who were functioning independently and those who were frail and dependent on caregivers for assistance with activities of daily living. METHODS: Behavioral management of continence comprised three techniques for the management of urinary incontinence: self-monitoring, scheduling regimens and pelvic muscle exercise with biofeedback. MAIN OUTCOME MEASURES: The main outcome measures were episodes of urine loss and amount (In grams) of urine loss with time, determined with a weighed pad test. RESULTS: Behavioral management of continence resulted in significant decrease in urinary incontinence; decreases in frequency and volume of urine loss were found among all study participants when data were analyzed. CONCLUSIONS: Although behavioral management of continence was effective in reducing incontinence among independent, community-dwelling elderly women, there was a marked lack of response to this project by frail elders and their caregivers. The same barriers to implementing time-intensive behavioral management interventions among frail elders in long-term care facilities may operate in the home setting.

Spasm of the gastroepiploic artery used for coronary artery bypass grafting.

The GEA, though similar to the internal mammary artery in size and flow characteristics, is different histologically as well as physiologically. When used as a free graft, the GEA is very vasospastic, which may influence its long-term patency. When it is necessary to use the GEA as a conduit, it may be preferable to use it in situ rather than as a pedicle graft.