Helping Girls with Rett Syndrome Improve
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
Research Center in Houston, Texas. Rett Syndrome, a debilitating disorder
that strikes only girls, causes mental retardation and growth failure. There is
The CNRC is a cooperative research facility run jointly by the
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
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
A story about the research appears in the February issue of ARS' Agricultural Research magazine
and on the World Wide Web at:
The Agricultural Research Service is USDAs chief scientific agency.
Scientific contact: Kathleen J. Motil,
M.D., ARS Childrens Nutrition Research Center at Baylor College of
Medicine, Houston, Texas, phone (713) 798-7178, fax (713) 798-7187,