Jayesh M. Bhatt, Andrew Bush, Marjo van Gerven, Andreea Nissenkorn, Michael Renke, Lian Yarlett, Malcolm Taylor, Thomy Tonia, Adilia Warris, Stefan Zielen, Shairbanu Zinna, Peter J. F. M. Merkus
- 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.
MetadatenAuthor: | Jayesh M. Bhatt, Andrew Bush, Marjo van Gerven, Andreea NissenkornORCiD, Michael Renke, Lian Yarlett, Malcolm Taylor, Thomy Tonia, Adilia Warris, Stefan ZielenORCiDGND, Shairbanu Zinna, Peter J. F. M. Merkus |
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URN: | urn:nbn:de:hebis:30:3-422788 |
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DOI: | https://doi.org/10.1183/16000617.0066-2015 |
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ISSN: | 0905-9180 |
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ISSN: | 1600-0617 |
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Pubmed Id: | https://pubmed.ncbi.nlm.nih.gov/26621971 |
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Parent Title (English): | European respiratory review |
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Publisher: | European Respiratory Society |
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Place of publication: | Sheffield |
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Document Type: | Article |
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Language: | English |
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Date of Publication (online): | 2016/11/28 |
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Date of first Publication: | 2015/11/30 |
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Publishing Institution: | Universitätsbibliothek Johann Christian Senckenberg |
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Release Date: | 2016/11/28 |
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Volume: | 24 |
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Issue: | 138 |
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Page Number: | 17 |
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First Page: | 565 |
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Last Page: | 581 |
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Note: | Copyright © ERS 2015. ERR articles are open access and distributed under the terms of the Creative Commons Attribution Non-Commercial Licence 4.0. |
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HeBIS-PPN: | 427941164 |
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Institutes: | Medizin / Medizin |
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Dewey Decimal Classification: | 6 Technik, Medizin, angewandte Wissenschaften / 61 Medizin und Gesundheit / 610 Medizin und Gesundheit |
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Sammlungen: | Universitätspublikationen |
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Licence (English): | Creative Commons - Namensnennung-Nicht kommerziell 4.0 |
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