Article Text

other Versions

PDF
Growth and nutrition in children with ataxia telangiectasia
  1. Emma Stewart1,
  2. Andrew P Prayle2,
  3. Alison Tooke1,
  4. Sara Pasalodos3,
  5. Mohnish Suri3,
  6. Andy Bush4,5,6,
  7. Jayesh M Bhatt1
  1. 1Nottingham Children's Hospital, National Paediatric Ataxia Telangiectasia Clinic, QMC, Nottingham, UK
  2. 2University of Nottingham, School of Clinical Science, Queens Medical Centre, Child Health, Nottingham, UK
  3. 3Nottingham Clinical Genetics Service, National Paediatric Ataxia Telangiectasia Clinic, Clinical Genetics Service, City Hospital Campus, Nottingham, UK
  4. 4Imperial College, London, UK
  5. 5National Heart and Lung Institute, London, UK
  6. 6Royal Brompton & Harefield NHS Foundation Trust, London, UK
  1. Correspondence to Dr Jayesh M Bhatt, Consultant Respiratory Paediatrician, Nottingham Children's Hospital, National Paediatric Ataxia Telangiectasia Clinic, QMC, Derby Road, Nottingham NG7 2UH, UK; Jayesh.bhatt{at}nuh.nhs.uk

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.

  • Nutrition
  • Growth
  • Ataxia Telangiectasia
  • Immunodeficiency
  • Respiratory infections

Statistics from Altmetric.com

Request permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.