RT Journal Article
SR Electronic
T1 The microbiology of lung disease in ataxia telangiectasia (A-T)
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P2954
VO 40
IS Suppl 56
A1 Jayesh Bhatt
A1 Andrew Bush
YR 2012
UL http://erj.ersjournals.com/content/40/Suppl_56/P2954.abstract
AB Background: A-T is a progressive, neurodegenerative disease causing immunodeficiency, an increased risk of malignancy & respiratory system complications, like chronic sinopulmonary disease, bronchiectesis, interstitial lung disease & aspiration. The high early age mortality is due to cancer & respiratory disease. Little is known about what causes lung infections in A-T (Ped Pul 2010;45:847-59). Aim: To describe the microbiology of Respiratory tract in patients attending the UK National A-T clinic in order to guide blind antimicrobial therapy, & to inform future studies to improve the evidence base for A-T treatment. Methods: We prospectively obtained cough swabs or sputum cultures as age-appropriate & throat swabs for virology. We collected data on new treatment prescribed, current maintenance antibiotic or immune replacement therapy. Results: 56 children (68 consuls) were seen in the clinic (May 09 to January 12).We could not obtain a sample in some children (Bacteria, n= 14; Viruses, n= 33).View this table:MicrobiologyView this table:Treatment:Conclusions: Our findings are similar to smaller series (n=15) (Acta Pædia 2007;96:1021–24) but with no opportunistic infections; bacterial infection is common and current management is based on extrapolation from other lung conditions. A-T specific evidence based guidelines are urgently needed.