RT Journal Article SR Electronic T1 Does declining nutritional status in ataxia telangiectasia cause decline in immunological function JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 4651 DO 10.1183/13993003.congress-2020.4651 VO 56 IS suppl 64 A1 Ugur, Suha A1 Bailey, Hannah A1 Bush, Andrew A1 Cliffe, Lucy A1 Mcdermott, Elizabeth A1 Payle, Andrew A1 Suri, Mohnish A1 Bhatt, Jayesh YR 2020 UL http://erj.ersjournals.com/content/56/suppl_64/4651.abstract 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).