PT - JOURNAL ARTICLE AU - Jayesh M. Bhatt AU - Andrew Bush AU - Marjo van Gerven AU - Andreea Nissenkorn AU - Michael Renke AU - Lian Yarlett AU - Malcolm Taylor AU - Thomy Tonia AU - Adilia Warris AU - Stefan Zielen AU - Shairbanu Zinna AU - Peter J.F.M. Merkus 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