Lung Transplant Improves Survival and Quality of Life Regardless of Telomere Dysfunction
Planas-Cerezales, Lurdes (Universitat de Barcelona)
Arias-Salgado, Elena G. (Instituto de Salud Carlos III)
Berastegui García, Cristina (Hospital Universitari Vall d'Hebron. Institut de Recerca)
Montes-Worboys, Ana (Universitat de Barcelona)
González-Montelongo, Rafaela (Instituto Tecnológico y de Energías Renovables (Santa Cruz de Tenerife, Illes Canàries))
Lorenzo-Salazar, José. M. (Instituto Tecnológico y de Energías Renovables (Santa Cruz de Tenerife, Illes Canàries))
Vicens-Zygmunt, Vanesa (Universitat de Barcelona)
Garcia-Moyano, Marta (Hospital Universitario de Cruces (Barakaldo, País Basc))
Dorca, Jordi (Universitat de Barcelona)
Flores, Carlos (Instituto de Salud Carlos III)
Perona, Rosario (Instituto de Salud Carlos III)
Román, Antonio (Hospital Universitari Vall d'Hebron. Institut de Recerca)
Molina-Molina, María (Instituto de Salud Carlos III)
Universitat Autònoma de Barcelona

Data:	2021
Resum:	Introduction: Fibrotic interstitial lung diseases (ILDs) are the first indication for lung transplantation (LT). Telomere dysfunction has been associated with poor post-transplant outcomes. The aim of the study was to evaluate the morbi-mortality and quality of life in fibrotic ILDs after lung transplant depending on telomere biology. Methods: Fibrotic ILD patients that underwent lung transplant were allocated to two arms; with or without telomere dysfunction at diagnosis based on the telomere length and telomerase related gene mutations revealed by whole-exome sequencing. Post-transplant evaluation included: (1) short and long-term mortality and complications and (2) quality of life. Results: Fifty-five percent of patients that underwent LT carried rare coding mutations in telomerase-related genes. Patients with telomere shortening more frequently needed extracorporeal circulation and presented a higher rate of early post-transplant hematological complications, longer stay in the intensive care unit (ICU), and a higher number of long-term hospital admissions. However, post-transplant 1-year survival was higher than 80% regardless of telomere dysfunction, with improvement in the quality of life and oxygen therapy withdrawal. Conclusions: Post-transplant morbidity is higher in patients with telomere dysfunction and differs according to elapsed time from transplantation. However, lung transplant improves survival and quality of life and the associated complications are manageable.
Ajuts:	Generalitat de Catalunya PT17/0015/0024 Instituto de Salud Carlos III PI18/00367 Instituto de Salud Carlos III PI20-00335 Instituto de Salud Carlos III AC19/00006
Drets:	Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original.
Llengua:	Anglès
Document:	Article ; recerca ; Versió publicada
Matèria:	Interstitial lung disease ; Pulmonary fibrosis ; Genetics ; Telomere shortening ; Telomere disorders ; Lung transplantation
Publicat a:	Frontiers in Medicine, Vol. 8 (july 2021) , ISSN 2296-858X

DOI: 10.3389/fmed.2021.695919
PMID: 34395476

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