Growth and nutrition in children with Ataxia telangiectasia

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...

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Main Authors:	Stewart, Emma, Prayle, Andrew P., Tooke, Alison, Pasalodos, Sarah, Suri, Mohnish, Bush, Andy, Bhatt, Jayesh
Format:	Article
Language:	English
Published:	BMJ 2016
Online Access:	http://eprints.nottingham.ac.uk/40018/ http://eprints.nottingham.ac.uk/40018/ http://eprints.nottingham.ac.uk/40018/ http://eprints.nottingham.ac.uk/40018/3/2016%2007%2014%20Growth%20and%20nutrition%20in%20A-T.pdf

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spelling	nottingham-400182017-10-15T14:45:04Z http://eprints.nottingham.ac.uk/40018/ Growth and nutrition in children with Ataxia telangiectasia Stewart, Emma Prayle, Andrew P. Tooke, Alison Pasalodos, Sarah Suri, Mohnish Bush, Andy Bhatt, Jayesh 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. BMJ 2016-08-29 Article PeerReviewed application/pdf en http://eprints.nottingham.ac.uk/40018/3/2016%2007%2014%20Growth%20and%20nutrition%20in%20A-T.pdf Stewart, Emma and Prayle, Andrew P. and Tooke, Alison and Pasalodos, Sarah and Suri, Mohnish and 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 http://adc.bmj.com/content/101/12/1137 doi:10.1136/archdischild-2015-310373 doi:10.1136/archdischild-2015-310373
repository_type	Digital Repository
institution_category	Local University
institution	University of Nottingham Malaysia Campus
building	Nottingham Research Data Repository
collection	Online Access
language	English
description	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.
format	Article
author	Stewart, Emma Prayle, Andrew P. Tooke, Alison Pasalodos, Sarah Suri, Mohnish Bush, Andy Bhatt, Jayesh
spellingShingle	Stewart, Emma Prayle, Andrew P. Tooke, Alison Pasalodos, Sarah Suri, Mohnish Bush, Andy Bhatt, Jayesh Growth and nutrition in children with Ataxia telangiectasia
author_facet	Stewart, Emma Prayle, Andrew P. Tooke, Alison Pasalodos, Sarah Suri, Mohnish Bush, Andy Bhatt, Jayesh
author_sort	Stewart, Emma
title	Growth and nutrition in children with Ataxia telangiectasia
title_short	Growth and nutrition in children with Ataxia telangiectasia
title_full	Growth and nutrition in children with Ataxia telangiectasia
title_fullStr	Growth and nutrition in children with Ataxia telangiectasia
title_full_unstemmed	Growth and nutrition in children with Ataxia telangiectasia
title_sort	growth and nutrition in children with ataxia telangiectasia
publisher	BMJ
publishDate	2016
url	http://eprints.nottingham.ac.uk/40018/ http://eprints.nottingham.ac.uk/40018/ http://eprints.nottingham.ac.uk/40018/ http://eprints.nottingham.ac.uk/40018/3/2016%2007%2014%20Growth%20and%20nutrition%20in%20A-T.pdf
first_indexed	2018-09-06T13:03:28Z
last_indexed	2018-09-06T13:03:28Z
_version_	1610863355162001408

Growth and nutrition in children with Ataxia telangiectasia

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