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J Pediatr Gastroenterol Nutr. 2007 Nov;45(5):582-90.

Feeding experiences and growth status in a Rett syndrome population.

Author information

  • 1Centre for Child Health Research, University of Western Australia, Telethon Institute for Child Health Research, Perth, Australia. wendyo@ichr.uwa.edu.au

Abstract

OBJECTIVES:

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.

MATERIALS AND 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.

PMID:
18030237
[PubMed - indexed for MEDLINE]
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