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Trasplante de pulmón; Fibrosis pulmonar; Trastornos de los telómeros Trasplantament pulmonar; Fibrosi pulmonar; Trastorns dels telòmers Lung transplantation; Pulmonary fibrosis; Telomere disorders 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. This study was funded by Instituto de Salud Carlos III through project PI18/00367 (Co-funded by European Regional Development Fund, ERDF, a way to build Europe), Spanish Society of Respiratory (SEPAR), Barcelona Respiratory Network (BRN), and Fundació Ramón Pla Armengol. RP laboratory was funded by grants PI20-00335 (Fondo de Investigaciones Sanitarias, Instituto de Salud Carlos III, Spain supported by FEDER funds). MM-M was funded by grants PI18/00367 (Fondo de Investigaciones Sanitarias, ISCIII, Spain, supported by FEDER funds), AC19/00006 (Projects of International Programs, ISCIII, Spain, supported by FEDER funds), Cohorte FPI CIBERES-ISCIII, Barcelona Respiratory Network-Fundation Ramon Pla Armengol, Spanish Society of Respiratory (SEPAR), and Catalan Society of Respiratory (SOCAP-FUCAP). CF was funded by Ministerio de Ciencia e Innovación (grant RTC-2017-6471-1; AEI/FEDER, UE), and by Cabildo Insular de Tenerife (CGIEU0000219140).

Altres ajuts: FEDER/FIS/PI09/02444 Altres ajuts: FEDER/FIS/PI12/00511 Abstract. Regenerative capacity of skeletal muscles resides in satellite cells, a self-renewing population of muscle cells. Several studies are investigating epigenetic mechanisms that control myogenic proliferation and differentiation to find new approaches that could boost regeneration of endogenous myogenic progenitor populations. In recent years, a lot of effort has been applied to purify, expand and manipulate adult stem cells from muscle tissue. However, this population of endogenous myogenic progenitors in adults is limited and their access is difficult and invasive. Therefore, other sources of stem cells with potential to regenerate muscles need to be examined. An excellent candidate could be a population of adult stromal cells within fat characterized by mesenchymal properties, which have been termed adipose-derived stem cells (ASCs). These progenitor adult stem cells have been successfully differentiated in vitro to osteogenic, chondrogenic, neurogenic and myogenic lineages. Autologous ASCs are multipotent and can be harvested with low morbidity; thus, they hold promise for a range of therapeutic applications. This review will summarize the use of ASCs in muscle regenerative approaches.