TY  - JOUR
A1  - Bhatt, Jayesh M.
A1  - Bush, Andrew
A1  - Gerven, Marjo van
A1  - Nissenkorn, Andreea
A1  - Renke, Michael
A1  - Yarlett, Lian
A1  - Taylor, Malcolm
A1  - Tonia, Thomy
A1  - Warris, Adilia
A1  - Zielen, Stefan
A1  - Zinna, Shairbanu
A1  - Merkus, Peter J. F. M.
T1  - ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia
T2  - European respiratory review
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.
Y1  - 2015
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/42278
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-422788
SN  - 0905-9180
SN  - 1600-0617
N1  - Copyright © ERS 2015. ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
VL  - 24
IS  - 138
SP  - 565
EP  - 581
PB  - European Respiratory Society
CY  - Sheffield
ER  -