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Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol
We report the case of a 42‐year‐old female with a history of finger clubbing which improved during pregnancy, a history of unexplained hepatosplenomegaly, and subsequent non‐specific interstitial pneumonia with respiratory failure. Given a personal and family history of early greying of the hair, th...
Autores principales: | , , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
John Wiley & Sons, Ltd
2020
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7316582/ https://www.ncbi.nlm.nih.gov/pubmed/32607243 http://dx.doi.org/10.1002/rcr2.607 |
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author | Chambers, Daniel C. Lutzky, Viviana P. Apte, Simon H. Godbolt, David Feenstra, John Mackintosh, John |
author_facet | Chambers, Daniel C. Lutzky, Viviana P. Apte, Simon H. Godbolt, David Feenstra, John Mackintosh, John |
author_sort | Chambers, Daniel C. |
collection | PubMed |
description | We report the case of a 42‐year‐old female with a history of finger clubbing which improved during pregnancy, a history of unexplained hepatosplenomegaly, and subsequent non‐specific interstitial pneumonia with respiratory failure. Given a personal and family history of early greying of the hair, the peripheral blood monocyte telomere length was measured and was confirmed to be <1st centile, explaining the multiorgan presentation. She was treated with prednisolone, mycophenolate mofetil, and the synthetic androgen danazol with a dramatic improvement in respiratory failure and lung function. After 18 months of danazol treatment, the peripheral blood monocyte telomere length had returned to the normal range. |
format | Online Article Text |
id | pubmed-7316582 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2020 |
publisher | John Wiley & Sons, Ltd |
record_format | MEDLINE/PubMed |
spelling | pubmed-73165822020-06-29 Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol Chambers, Daniel C. Lutzky, Viviana P. Apte, Simon H. Godbolt, David Feenstra, John Mackintosh, John Respirol Case Rep Case Reports We report the case of a 42‐year‐old female with a history of finger clubbing which improved during pregnancy, a history of unexplained hepatosplenomegaly, and subsequent non‐specific interstitial pneumonia with respiratory failure. Given a personal and family history of early greying of the hair, the peripheral blood monocyte telomere length was measured and was confirmed to be <1st centile, explaining the multiorgan presentation. She was treated with prednisolone, mycophenolate mofetil, and the synthetic androgen danazol with a dramatic improvement in respiratory failure and lung function. After 18 months of danazol treatment, the peripheral blood monocyte telomere length had returned to the normal range. John Wiley & Sons, Ltd 2020-06-25 /pmc/articles/PMC7316582/ /pubmed/32607243 http://dx.doi.org/10.1002/rcr2.607 Text en © 2020 The Authors. Respirology Case Reports published by John Wiley & Sons Australia, Ltd on behalf of The Asian Pacific Society of Respirology This is an open access article under the terms of the http://creativecommons.org/licenses/by/4.0/ License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Reports Chambers, Daniel C. Lutzky, Viviana P. Apte, Simon H. Godbolt, David Feenstra, John Mackintosh, John Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol |
title | Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol |
title_full | Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol |
title_fullStr | Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol |
title_full_unstemmed | Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol |
title_short | Successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol |
title_sort | successful treatment of telomeropathy‐related interstitial lung disease with immunosuppression and danazol |
topic | Case Reports |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7316582/ https://www.ncbi.nlm.nih.gov/pubmed/32607243 http://dx.doi.org/10.1002/rcr2.607 |
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