PT  - JOURNAL ARTICLE
AU  - Ugur, Suha
AU  - Bailey, Hannah
AU  - Bush, Andrew
AU  - Cliffe, Lucy
AU  - Mcdermott, Elizabeth
AU  - Payle, Andrew
AU  - Suri, Mohnish
AU  - Bhatt, Jayesh
TI  - Does declining nutritional status in ataxia telangiectasia cause decline in immunological function
AID  - 10.1183/13993003.congress-2020.4651
DP  - 2020 Sep 07
TA  - European Respiratory Journal
PG  - 4651
VI  - 56
IP  - suppl 64
4099  - http://erj.ersjournals.com/content/56/suppl_64/4651.short
4100  - http://erj.ersjournals.com/content/56/suppl_64/4651.full
SO  - Eur Respir J2020 Sep 07; 56
AB  - Introduction: Ataxia-Telangiectasia (A-T) is a rare autosomal-recessive disease, characterised by neurodegeneration, malignancy, immunodeficiency and respiratory disease. Median life expectancy is 25 years, with respiratory disease and cancer as the leading cause of early death. Individuals with A-T demonstrate progressive under-nutrition from the first decade (Archives of disease in childhood, 2016; 101(12): pp.1137-1141). In Cystic Fibrosis, respiratory health and nutritional status are closely linked (Journal of paediatrics, 2003; 141(6): pp.624-630). It is well known that worsening nutritional status increases infection-related morbidity and mortality. We hypothesised that declining nutritional status was associated with a decline in immune function in A-T.Methods: Data was collected retrospectively from 2009-2019. Patients had weight and height/length recorded and bloods taken at each visit. Immunological markers were: PCV-13 vaccine response, CD4 T cells, Pneumococcal and Haemophilus influenza B antibodies.Using STATA, multi-level mixed-effects models measured the effect of weight z-scores on immune markers.Results: Multi-level estimations on 123 patients indicate weight z-score has statistically significant associations with immune dysfunction. View this table:Summary: Nutrition and immune dysfunction are linked in A-T. The next step is to determine if improving nutrition leads to improved immune function.FootnotesCite this article as: European Respiratory Journal 2020; 56: Suppl. 64, 4651.This abstract was presented at the 2020 ERS International Congress, in session “Respiratory viruses in the "pre COVID-19" era”.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only).