Growth and nutrition in children with ataxia telangiectasia
File(s)
Accepted version
Author(s)
Type
Journal Article
Abstract
BACKGROUND: Ataxia telangiectasia (A-T) is a rare multisystem disease with high early mortality from lung disease and cancer. Nutritional failure adversely impacts outcomes in many respiratory diseases. Several factors influence nutrition in children with A-T. We hypothesised that children with A-T have progressive growth failure and that early gastrostomy tube feeding (percutaneous endoscopic gastrostomy, PEG) is a favourable management option with good nutritional outcomes. METHODS: Data were collected prospectively on weight, height and body mass index (BMI) at the national paediatric A-T clinic. Adequacy and safety of oral intake was assessed. Nutritional advice was given at each multidisciplinary review. RESULTS: 101 children (51 girls) had 222 measurements (32 once, 32 twice, 24 thrice) between 2009 and 2016. Median (IQR) age was 9.3 (6.4 to 13.1) years. Mean (SD) weight, height and BMI Z-scores were respectively -1 (1.6), -1.2 (1.2) and -0.4 (1.4). 35/101 children had weight Z-scores below -2 on at least one occasion. Weight, height and BMI Z-scores declined over time. Decline was most obvious after 8 years of age. 14/101 (14%) children had a PEG, with longitudinal data available for 12. In a nested case control study, there was a trend for improvement in weight in those with a PEG (p=0.10). CONCLUSIONS: Patients with A-T decline in growth over time. There is an urgent need for new strategies, including an understanding of why growth falters. We suggest early proactive consideration of PEG from age 8 years onwards to prevent progressive growth failure.
Date Issued
2016-08-29
Date Acceptance
2016-08-09
Citation
Archives of Disease in Childhood, 2016, 101, pp.1137-1141
ISSN
0003-9888
Publisher
BMJ Publishing Group
Start Page
1137
End Page
1141
Journal / Book Title
Archives of Disease in Childhood
Volume
101
Copyright Statement
© 2016 BMJ Publishing Group. This article has been accepted for publication in Archives of Disease in Childhood following peer review. The definitive copyedited, typeset version is available online at: http://dx.doi.org/10.1136/archdischild-2015-310373
Identifier
PII: archdischild-2015-310373
Subjects
Science & Technology
Life Sciences & Biomedicine
Pediatrics
CYSTIC-FIBROSIS
PULMONARY-FUNCTION
DIABETES-MELLITUS
DEFICIENCY
INTERVENTION
GASTROSTOMY
DISEASES
Ataxia Telangiectasia
Growth
Immunodeficiency
Nutrition
Respiratory infections
1103 Clinical Sciences
1114 Paediatrics And Reproductive Medicine
1117 Public Health And Health Services
Publication Status
Published