ERS statement on the multidisciplinary respiratory management of ataxia telangiectasia

  • 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.

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Author:Jayesh M. Bhatt, Andrew Bush, Marjo van Gerven, Andreea Nissenkorn, Michael Renke, Lian Yarlett, Malcolm Taylor, Thomy Tonia, Adilia Warris, Stefan ZielenORCiDGND, Shairbanu Zinna, Peter J. F. M. Merkus
Pubmed Id:
Parent Title (English):European respiratory review
Publisher:European Respiratory Society
Place of publication:Sheffield
Document Type:Article
Date of Publication (online):2016/11/28
Date of first Publication:2015/11/30
Publishing Institution:Universitätsbibliothek Johann Christian Senckenberg
Release Date:2016/11/28
Page Number:17
First Page:565
Last Page:581
Copyright © ERS 2015. ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0.
Institutes:Medizin / Medizin
Dewey Decimal Classification:6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit
Licence (English):License LogoCreative Commons - Namensnennung-Nicht kommerziell 4.0