Kathleen Motil examines a patient with Rett Syndrome.

Helping Girls with Rett Syndrome Improve Growth

By Jill Lee
February 23, 1999

A long-running energy deficit may play a role in the hampered nutrition and growth caused by Rett Syndrome, according to studies by a pediatrician with the Children’s Nutrition Research Center in Houston, Texas. Rett Syndrome, a debilitating disorder that strikes only girls, causes mental retardation and growth failure. There is no cure.

The CNRC is a cooperative research facility run jointly by the USDA’s Agricultural Research Service and Baylor College of Medicine. The Rett studies were done by CNRC pediatric gastroenterologist Kathleen J. Motil, M.D, Ph.D.,and pediatric neurologist Daniel Glaze, M.D., of The Rett Center at Baylor College of Medicine.

Motil, Glaze and colleagues compared the energy balance--calories consumed minus calories used--of healthy girls to those of children with Rett. The girls with Rett Syndrome had a positive energy balance, but it was lower than average for girls their age. It's possible this subtle, long-running energy deficit plays a role in slowing growth.

Motil and her colleagues have been testing new therapies to help girls with Rett Syndrome. She has had success with a gastrostomy button. This surgically implanted device allows nutrients to be delivered to the body while the girls sleep. One patient, aged seven, increased her weight from 31 pounds to 48 in a year, allowing her to sit up for the first time.

Currently, Motil is trying to find out why girls with Rett Syndrome who have a gastrostomy button increase body fat more than lean muscle. Understanding why this happens might lead to even better treatment options someday.

The prevalence of Rett Syndrome in various countries ranges from 1 in 10,000 to 1 in 23,000 live female births, according to the International Rett Syndrome Association.

A story about the research appears in the February issue of ARS' Agricultural Research magazine and on the World Wide Web at:

http://www.ars.usda.gov/is/AR/archive/feb99/rett0299.htm

The Agricultural Research Service is USDA’s chief scientific agency.

Scientific contact: Kathleen J. Motil, M.D., ARS Children’s Nutrition Research Center at Baylor College of Medicine, Houston, Texas, phone (713) 798-7178, fax (713) 798-7187, [email protected].

U.S. Department of Agriculture
 


This article comes from Science Blog. Copyright © 2004
http://www.scienceblog.com/community