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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. Summary: Nutrition and immune dysfunction are linked in A-T. The next step is to determine if improving nutrition leads to improved immune function. |
