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Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient
Werner syndrome (WS) is a progeroid or premature aging syndrome characterized by early onset of age-related pathologies and cancer. The average life expectancy of affected people is 52.8 years and tends to increase. The major causes of death are malignancy and myocardial infarction. Increased telome...
Autores principales: | , , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4695635/ https://www.ncbi.nlm.nih.gov/pubmed/26788395 http://dx.doi.org/10.1155/2015/361694 |
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author | Goletto, Tiphaine Crockett, Flora Aractingi, Selim Toper, Cecile Senet, Patricia Cadranel, Jacques Naccache, Jean-Marc |
author_facet | Goletto, Tiphaine Crockett, Flora Aractingi, Selim Toper, Cecile Senet, Patricia Cadranel, Jacques Naccache, Jean-Marc |
author_sort | Goletto, Tiphaine |
collection | PubMed |
description | Werner syndrome (WS) is a progeroid or premature aging syndrome characterized by early onset of age-related pathologies and cancer. The average life expectancy of affected people is 52.8 years and tends to increase. The major causes of death are malignancy and myocardial infarction. Increased telomere attrition and decay are thought to play a causative role in the clinical and pathological manifestations of the disease. Although telomere length, with or without germline mutation, is known to be associated with interstitial lung disease, the latter is not associated with WS. To the best of our knowledge, we report the first case describing a WS patient with fatal ILD. This case suggests that older patients with WS could develop ILD. Clinical outcome of WS patients may thus be improved by counselling them regarding smoking cessation or other exposure and by proposing antifibrotic therapy. |
format | Online Article Text |
id | pubmed-4695635 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-46956352016-01-19 Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient Goletto, Tiphaine Crockett, Flora Aractingi, Selim Toper, Cecile Senet, Patricia Cadranel, Jacques Naccache, Jean-Marc Case Rep Pulmonol Case Report Werner syndrome (WS) is a progeroid or premature aging syndrome characterized by early onset of age-related pathologies and cancer. The average life expectancy of affected people is 52.8 years and tends to increase. The major causes of death are malignancy and myocardial infarction. Increased telomere attrition and decay are thought to play a causative role in the clinical and pathological manifestations of the disease. Although telomere length, with or without germline mutation, is known to be associated with interstitial lung disease, the latter is not associated with WS. To the best of our knowledge, we report the first case describing a WS patient with fatal ILD. This case suggests that older patients with WS could develop ILD. Clinical outcome of WS patients may thus be improved by counselling them regarding smoking cessation or other exposure and by proposing antifibrotic therapy. Hindawi Publishing Corporation 2015 2015-12-16 /pmc/articles/PMC4695635/ /pubmed/26788395 http://dx.doi.org/10.1155/2015/361694 Text en Copyright © 2015 Tiphaine Goletto et al. https://creativecommons.org/licenses/by/4.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Goletto, Tiphaine Crockett, Flora Aractingi, Selim Toper, Cecile Senet, Patricia Cadranel, Jacques Naccache, Jean-Marc Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient |
title | Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient |
title_full | Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient |
title_fullStr | Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient |
title_full_unstemmed | Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient |
title_short | Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient |
title_sort | interstitial lung disease in werner syndrome: a case report of a 55-year-old male patient |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4695635/ https://www.ncbi.nlm.nih.gov/pubmed/26788395 http://dx.doi.org/10.1155/2015/361694 |
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