TY - JOUR T1 - ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia JF - European Respiratory Review JO - EUROPEAN RESPIRATORY REVIEW SP - 565 LP - 581 DO - 10.1183/16000617.0066-2015 VL - 24 IS - 138 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 Y1 - 2015/12/01 UR - http://err.ersjournals.com/content/24/138/565.abstract N2 - 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 ER -