Growth and nutrition in children with Ataxia telangiectasia

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Stewart, Emma, Prayle, Andrew P., Tooke, Alison, Pasalodos, Sarah, Suri, Mohnish, Bush, Andy and Bhatt, Jayesh (2016) Growth and nutrition in children with Ataxia telangiectasia. Archives of Disease in Childhood, 101 (12). pp. 1137-1141. ISSN 1468-2044

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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.

Item Type: Article
RIS ID: https://nottingham-repository.worktribe.com/output/804215
Schools/Departments: University of Nottingham, UK > Faculty of Medicine and Health Sciences > School of Medicine > Division of Child Health, Obstetrics and Gynaecology
Identification Number: 10.1136/archdischild-2015-310373
Depositing User: PRAYLE, Andrew
Date Deposited: 24 Jan 2017 14:47
Last Modified: 04 May 2020 18:05
URI: https://eprints.nottingham.ac.uk/id/eprint/40018

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