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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...

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Autores principales: Goletto, Tiphaine, Crockett, Flora, Aractingi, Selim, Toper, Cecile, Senet, Patricia, Cadranel, Jacques, Naccache, Jean-Marc
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
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.
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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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