An assessment of obesity and hyperphagia in individuals with Smith-Magenis syndrome

Smith-Magenis syndrome (SMS;OMIM# 182290) is a multiple congenital anomalies and mental retardation syndrome caused by a 3.7- Mb deletion on chromosome 17p11.2 or a mutation in the RAI1 gene. Although the majority of the SMS phenotype has been well described, limited studies are available describing growth patterns in SMS. There is some evidence that individuals with SMS develop obesity. Thus, this study aims to characterize the growth and potential influence of hyperphagia in a cohort of individuals with SMS. A retrospective chart review was conducted of 78 individuals with SMS through Baylor College of Medicine (BCM) at Texas Children¡¯s Hospital (TCH.) All documented height and weight measurements were abstracted and Z-scores (SD units) for height-for-age, length-for-age and BMI-for-age were calculated. Mail-out questionnaires were provided to the corresponding parents of the cohort to assess for the presence of hyperphagia through a validated hyperphagia questionnaire (HQ). Analysis of this data demonstrates that by the age ¡Ý 20 years males with SMS have mean BMI¡¯s in the 85th-90th percentile corresponding to an overweight BMI, and females with SMS had mean BMI¡¯s in the 95th -97th percentile corresponding to an obese BMI. Parents indicated that hyperphagia is present in individuals with SMS as 76% of parent¡¯s report having to lock food away from their child. Females¡¯ age ¡Ý 20 years of age had the highest mean behavior, drive and severity scores as well as the highest BMI. Thus, this study concludes that it appears overweight and obesity, as well as hyperphagia, are present in this cohort of SMS individuals. The results of this study will hopefully enable parents and caregivers of children with SMS to take preventative measures in order to control food related behaviors present in their children as well as to prevent overweight and obesity and the associated negative health consequences.

An analysis of the RightCare policy at Ben Taub General Hospital, Houston, Texas

Introduction. This study is a two-part evaluation of the RightCare policy, a policy implemented to reduce crowding at the Emergency Center (EC) at Ben Taub General Hospital in Houston, Harris County, Texas. This research includes an evaluation of the policy's impact on specific hospital measures, along with a description of the policy's demise from the point of view of hospital staff. Objective. The purpose of this study is two-fold: (1) To determine whether RightCare policy affected the level of crowding in the Emergency Center and (2) to identify the conditions that may have led to the policy's demise. Methods. For the policy impact portion of this research, hospital measures were collected from existing databases. Analysis included a pre-post comparative design in which the 12 months preceding the policy's implementation were compared with the 12 months following the policy's implementation. For the policy perception portion, employees were surveyed using an on-line questionnaire. Results. The results of the study are mixed. Some measures improved, including time spent on ambulance diversion and the proportion of those who left without being seen, while others did not, such as return visits and total length of stay. Employees generally supported the policy, but expressed concerns over insufficient training and funding. Conclusion. The RightCare policy was a good initial attempt to improve crowded conditions in the EC. The study showed that a clearer policy design, improved training, adequate staffing levels, and better communication would improve operational outcomes in the future.^

Descriptive analysis of Ben Taub Pediatric Weight Management Program

The purpose of this study is to descriptively analyze the current program at Ben Taub Pediatric Weight Management Program in Houston, Texas, a program designed to help overweight children ages three to eighteen to lose weight. In Texas, approximately one in every three children is overweight or obese. Obesity is seen at an even greater level within Ben Taub due to the hospital's high rate of service for underserved minority populations (Dehghan et al, 2005; Tyler and Horner, 2008; Hunt, 2009). The weight management program consists of nutritional, behavioral, physical activity, and medical counseling. Analysis will focus on changes in weight, BMI, cholesterol levels, and blood pressure from 2007–2010 for all participants who attended at least two weight management sessions. Recommendations will be given in response to the results of the data analysis.^