Title |
Lung transplantation in telomerase mutation carriers with pulmonary fibrosis
|
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Published in |
European Respiratory Journal, May 2014
|
DOI | 10.1183/09031936.00060014 |
Pubmed ID | |
Authors |
Leann L. Silhan, Pali D. Shah, Daniel C. Chambers, Laurie D. Snyder, Gerdt C. Riise, Christa L. Wagner, Eva Hellström-Lindberg, Jonathan B. Orens, Juliette F. Mewton, Sonye K. Danoff, Murat O. Arcasoy, Mary Armanios |
Abstract |
Lung transplantation is the only intervention that prolongs survival in idiopathic pulmonary fibrosis (IPF). Telomerase mutations are the most common identifiable genetic cause of IPF, and at times, the telomere defect manifests in extrapulmonary disease such as bone marrow failure. The relevance of this genetic diagnosis for lung transplant management has not been examined. We gathered an international series of telomerase mutation carriers who underwent lung transplant in the USA, Australia and Sweden. The median age at transplant was 52 years. Seven recipients are alive with a median follow-up of 1.9 years (range 6 months to 9 years); one died at 10 months. The most common complications were haematological, with recipients requiring platelet transfusion support (88%) and adjustment of immunosuppressives (100%). Four recipients (50%) required dialysis for tubular injury and calcineurin inhibitor toxicity. These complications occurred at significantly higher rates relative to historic series (p<0.0001). Our observations support the feasibility of lung transplantation in telomerase mutation carriers; however, severe post-transplant complications reflecting the syndromic nature of their disease appear to occur at higher rates. While these findings need to be expanded to other cohorts, caution should be exercised when approaching the transplant evaluation and management of this subset of pulmonary fibrosis patients. |
X Demographics
Geographical breakdown
Country | Count | As % |
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Israel | 1 | 20% |
Unknown | 4 | 80% |
Demographic breakdown
Type | Count | As % |
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Members of the public | 3 | 60% |
Science communicators (journalists, bloggers, editors) | 1 | 20% |
Practitioners (doctors, other healthcare professionals) | 1 | 20% |
Mendeley readers
Geographical breakdown
Country | Count | As % |
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India | 1 | 1% |
South Africa | 1 | 1% |
Canada | 1 | 1% |
Unknown | 85 | 97% |
Demographic breakdown
Readers by professional status | Count | As % |
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Researcher | 12 | 14% |
Student > Ph. D. Student | 9 | 10% |
Student > Postgraduate | 8 | 9% |
Student > Master | 8 | 9% |
Other | 7 | 8% |
Other | 19 | 22% |
Unknown | 25 | 28% |
Readers by discipline | Count | As % |
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Medicine and Dentistry | 33 | 38% |
Biochemistry, Genetics and Molecular Biology | 5 | 6% |
Nursing and Health Professions | 5 | 6% |
Agricultural and Biological Sciences | 5 | 6% |
Pharmacology, Toxicology and Pharmaceutical Science | 3 | 3% |
Other | 5 | 6% |
Unknown | 32 | 36% |