RT Journal Article
SR Electronic
T1 Growth and nutrition in children with ataxia telangiectasia
JF Archives of Disease in Childhood
JO Arch Dis Child
FD BMJ Publishing Group Ltd and Royal College of Paediatrics and Child Health
SP 1137
OP 1141
DO 10.1136/archdischild-2015-310373
VO 101
IS 12
A1 Emma Stewart
A1 Andrew P Prayle
A1 Alison Tooke
A1 Sara Pasalodos
A1 Mohnish Suri
A1 Andy Bush
A1 Jayesh M Bhatt
YR 2016
UL http://adc.bmj.com/content/101/12/1137.abstract
AB 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.