title: Feeding experiences and growth status in a Rett syndrome population.
abstract: 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.
authors.affiliation: Centre for Child Health Research, University of Western Australia, Telethon Institute for Child Health Research, Perth, Australia. wendyo@ichr.uwa.edu.au
authors.forename: Wendy H
authors.initials: WH
authors.lastname: Oddy
authors.forename: Kathryn G
authors.initials: KG
authors.lastname: Webb
authors.forename: Gordon
authors.initials: G
authors.lastname: Baikie
authors.forename: Susan M
authors.initials: SM
authors.lastname: Thompson
authors.forename: Sheena
authors.initials: S
authors.lastname: Reilly
authors.forename: Susan D
authors.initials: SD
authors.lastname: Fyfe
authors.forename: Deidra
authors.initials: D
authors.lastname: Young
authors.forename: Alison M
authors.initials: AM
authors.lastname: Anderson
authors.forename: Helen
authors.initials: H
authors.lastname: Leonard
mesh_terms: 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