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.
journal_name
Arch Dis Childjournal_title
Archives of disease in childhoodauthors
Stewart E,Prayle AP,Tooke A,Pasalodos S,Suri M,Bush A,Bhatt JMdoi
10.1136/archdischild-2015-310373subject
Has Abstractpub_date
2016-12-01 00:00:00pages
1137-1141issue
12eissn
0003-9888issn
1468-2044pii
archdischild-2015-310373journal_volume
101pub_type
杂志文章abstract::Aortic arch anomalies in infancy often cause intrathoracic airway obstruction. Airway function was assessed as part of the diagnostic evaluation in six symptomatic infants both by plethysmography and using a chest compression technique to obtain partial flow-volume loops. Two infants had normal intrathoracic airway fu...
journal_title:Archives of disease in childhood
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doi:10.1136/adc.65.2.171
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
pub_type: 杂志文章,多中心研究
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doi:10.1136/adc.66.9.1047
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.164038
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
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doi:10.1136/adc.76.2.159
更新日期:1997-02-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.60.6.581
更新日期:1985-06-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.81.5.395
更新日期:1999-11-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.84.6.504
更新日期:2001-06-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.178228
更新日期:2010-08-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2009.165159
更新日期:2010-05-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.2004.058115
更新日期:2005-05-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.67.10.1286
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
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journal_title:Archives of disease in childhood
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pub_type: 杂志文章
doi:10.1136/adc.2006.104869
更新日期:2007-10-01 00:00:00
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journal_title:Archives of disease in childhood
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doi:10.1136/adc.50.4.269
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
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journal_title:Archives of disease in childhood
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journal_title:Archives of disease in childhood
pub_type: 杂志文章,评审
doi:10.1136/archdischild-2014-306153
更新日期:2016-03-01 00:00:00
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journal_title:Archives of disease in childhood
pub_type: 杂志文章
doi:10.1136/adc.52.6.441
更新日期:1977-06-01 00:00:00