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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...

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Autores principales: Chambers, Daniel C., Lutzky, Viviana P., Apte, Simon H., Godbolt, David, Feenstra, John, Mackintosh, John
Formato: Online Artículo Texto
Lenguaje:English
Publicado: John Wiley & Sons, Ltd 2020
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.
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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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