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Growth and nutrition in children with Ataxia telangiectasia

Stewart, Emma; Prayle, Andrew P.; Tooke, Alison; Pasalodos, Sarah; Suri, Mohnish; Bush, Andy; Bhatt, Jayesh

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Authors

Emma Stewart

Andrew P. Prayle

Alison Tooke

Sarah Pasalodos

Mohnish Suri

Andy Bush

Jayesh Bhatt



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, or 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 (range) age was 9.3 (1.5 to 18.4) years. Mean (sd) weight, height and BMI Z-scores were respectively -1.03(1.57), -1.17 (1.18) and -0.36 (1.43). 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 (13.9%) 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 = 0.06).
Conclusions: A-T patients 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 in order to prevent progressive growth failure.

Citation

Stewart, E., Prayle, A. P., Tooke, A., Pasalodos, S., Suri, M., Bush, A., & Bhatt, J. (in press). Growth and nutrition in children with Ataxia telangiectasia. Archives of Disease in Childhood, 101(12), https://doi.org/10.1136/archdischild-2015-310373

Journal Article Type Article
Acceptance Date Aug 9, 2016
Online Publication Date Aug 29, 2016
Deposit Date Jan 24, 2017
Publicly Available Date Jan 24, 2017
Journal Archives of Disease in Childhood
Print ISSN 0003-9888
Electronic ISSN 1468-2044
Publisher BMJ Publishing Group
Peer Reviewed Peer Reviewed
Volume 101
Issue 12
DOI https://doi.org/10.1136/archdischild-2015-310373
Public URL https://nottingham-repository.worktribe.com/output/804215
Publisher URL http://adc.bmj.com/content/101/12/1137
Contract Date Jan 24, 2017

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