Feeding experiences and growth status in a Rett syndrome population.
OBJECTIVE
Feeding difficulties in Rett syndrome are complex and multifactorial. In this study, we describe the feeding experiences in Rett syndrome and examine the factors affecting growth.


METHODS
Using questionnaire data related to a population-based cohort, ages 2 to 29 years (n = 201), we measured the feeding experiences, growth, and factors affecting growth (enteral nutritional support, mutations, mobility, breath-holding, hyperventilation) in subjects with Rett syndrome.


RESULTS
The mean weight, height, and body mass index z scores in subjects with Rett syndrome were below that of their age group and decreased steadily with age. Twenty percent of subjects had enteral nutrition support, and it was more common in the older age group. Those with truncating mutations had significantly less enteral nutrition support than the other mutation groups. Furthermore, those with low mobility had lower mean body mass index z scores than those with higher mobility, and increased frequency of breath-holding and hyperventilation also was associated with lower body mass index z scores.


CONCLUSIONS
Routine monitoring of growth should continue to determine the severity of nutritional problems in Rett syndrome. Active nutritional management is recommended to ensure females affected with Rett syndrome have the best opportunity to reach their growth potential.
Oddy
Wendy H
WH
Centre for Child Health Research, University of Western Australia, Telethon Institute for Child Health Research, Perth, Australia. wendyo@ichr.uwa.edu.au
Webb
Kathryn G
KG
Baikie
Gordon
G
Thompson
Susan M
SM
Reilly
Sheena
S
Fyfe
Susan D
SD
Young
Deidra
D
Anderson
Alison M
AM
Leonard
Helen
H
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001315:Australia / Q000453:epidemiology; D001827:Body Height; D015992:Body Mass Index; D001835:Body Weight; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D004750:Enteral Nutrition / Q000706:statistics & numerical data; D005247:Feeding Behavior; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006985:Hyperventilation / Q000453:epidemiology; D009752:Nutritional Status; D012119:Respiration; D015518:Rett Syndrome / Q000453:epidemiology / Q000235:genetics / Q000503:physiopathology*; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires