ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia.

Bhatt, Jayesh M; Bush, Andrew; van Gerven, Marjo; Nissenkorn, Andreea; Renke, Michael; Yarlett, Lian; Taylor, Malcolm; Tonia, Thomy; Warris, Adilia; Zielen, Stefan; Zinna, Shairbanu; Merkus, Peter J F M (2015). ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia. European respiratory review, 24(138), pp. 565-581. European Respiratory Society 10.1183/16000617.0066-2015

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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.

Item Type:

Journal Article (Original Article)

Division/Institute:

04 Faculty of Medicine > Pre-clinic Human Medicine > Institute of Social and Preventive Medicine (ISPM)

UniBE Contributor:

Tonia, Thomai

Subjects:

600 Technology > 610 Medicine & health
300 Social sciences, sociology & anthropology > 360 Social problems & social services

ISSN:

0905-9180

Publisher:

European Respiratory Society

Language:

English

Submitter:

Doris Kopp Heim

Date Deposited:

12 Feb 2016 16:47

Last Modified:

05 Dec 2022 14:52

Publisher DOI:

10.1183/16000617.0066-2015

PubMed ID:

26621971

BORIS DOI:

10.7892/boris.77450

URI:

https://boris.unibe.ch/id/eprint/77450

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