PT  - JOURNAL ARTICLE
AU  - Bhatt, Jayesh M.
AU  - Bush, Andrew
AU  - van Gerven, Marjo
AU  - Nissenkorn, Andreea
AU  - Renke, Michael
AU  - Yarlett, Lian
AU  - Taylor, Malcolm
AU  - Tonia, Thomy
AU  - Warris, Adilia
AU  - Zielen, Stefan
AU  - Zinna, Shairbanu
AU  - Merkus, Peter J.F.M.
TI  - ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia
AID  - 10.1183/16000617.0066-2015
DP  - 2015 Dec 01
TA  - European Respiratory Review
PG  - 565--581
VI  - 24
IP  - 138
4099  - http://err.ersjournals.com/content/24/138/565.short
4100  - http://err.ersjournals.com/content/24/138/565.full
SO  - EUROPEAN RESPIRATORY REVIEW2015 Dec 01; 24
AB  - Ataxia telangiectasia (A-T) is a rare, progressive, multisystem disease that has a large number of complex and diverse manifestations which vary with age. Patients with A-T die prematurely with the leading causes of death being respiratory diseases and cancer. Respiratory manifestations include immune dysfunction leading to recurrent upper and lower respiratory infections; aspiration resulting from dysfunctional swallowing due to neurodegenerative deficits; inefficient cough; and interstitial lung disease/pulmonary fibrosis. Malnutrition is a significant comorbidity. The increased radiosensitivity and increased risk of cancer should be borne in mind when requesting radiological investigations. Aggressive proactive monitoring and treatment of these various aspects of lung disease under multidisciplinary expertise in the experience of national multidisciplinary clinics internationally forms the basis of this statement on the management of lung disease in A-T. Neurological management is outwith the scope of this document.Complex respiratory manifestations in ataxia telangiectasia require regular, proactive multidisciplinary management http://ow.ly/Solqe