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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| Format: | Article |
| Language: | English |
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Wiley
2015-01-01
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| Series: | Case Reports in Pulmonology |
| Online Access: | http://dx.doi.org/10.1155/2015/361694 |
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| author | Tiphaine Goletto Flora Crockett Selim Aractingi Cecile Toper Patricia Senet Jacques Cadranel Jean-Marc Naccache |
| author_facet | Tiphaine Goletto Flora Crockett Selim Aractingi Cecile Toper Patricia Senet Jacques Cadranel Jean-Marc Naccache |
| author_sort | Tiphaine Goletto |
| collection | DOAJ |
| 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 | Article |
| id | doaj-art-2eaf408d4fd045dab296c2b7b1921160 |
| institution | Kabale University |
| issn | 2090-6846 2090-6854 |
| language | English |
| publishDate | 2015-01-01 |
| publisher | Wiley |
| record_format | Article |
| series | Case Reports in Pulmonology |
| spelling | doaj-art-2eaf408d4fd045dab296c2b7b19211602025-02-03T06:11:43ZengWileyCase Reports in Pulmonology2090-68462090-68542015-01-01201510.1155/2015/361694361694Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male PatientTiphaine Goletto0Flora Crockett1Selim Aractingi2Cecile Toper3Patricia Senet4Jacques Cadranel5Jean-Marc Naccache6Service de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, FranceService de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, FranceService de Dermatologie, Hôpital Universitaire Cochin, 75014 Paris, FranceService de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, FranceService de Dermatologie, Hôpital Universitaire Tenon, 75020 Paris, FranceService de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, FranceService de Pneumologie, Hôpital Universitaire Tenon, 75020 Paris, FranceWerner 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.http://dx.doi.org/10.1155/2015/361694 |
| spellingShingle | Tiphaine Goletto Flora Crockett Selim Aractingi Cecile Toper Patricia Senet Jacques Cadranel Jean-Marc Naccache Interstitial Lung Disease in Werner Syndrome: A Case Report of a 55-Year-Old Male Patient Case Reports in Pulmonology |
| 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 |
| url | http://dx.doi.org/10.1155/2015/361694 |
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