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Clinical outcomes of lung transplant recipients with telomerase mutations.

ABSTRACT: Successful lung transplantation for patients with pulmonary fibrosis from telomerase mutations may be limited by systemic complications of telomerase dysfunction, including myelosuppression, cirrhosis, and malignancy. We describe clinical outcomes in 14 lung transplant recipients with telomerase mutations.Subjects underwent lung transplantation between February 2005 and April 2014 at 5 transplant centers. Data were abstracted from medical records, focusing on outcomes reflecting post-transplant treatment effects likely to be complicated by telomerase mutations.The median age of subjects was 60.5 years (interquartile range = 52.0-62.0), 64.3% were male, and the mean post-transplant observation time was 3.2 years (SD ± 2.9). A mutation in telomerase reverse transcriptase was present in 11 subjects, a telomerase RNA component mutation was present in 2 subjects, and an uncharacterized mutation was present in 1 subject. After lung transplantation, 10 subjects were leukopenic and 5 did not tolerate lymphocyte anti-proliferative agents. Six subjects developed recurrent lower respiratory tract infections, 7 developed acute cellular rejection (A1), and 4 developed chronic lung allograft dysfunction. Eight subjects developed at least 1 episode of acute renal failure and 10 developed chronic renal insufficiency. In addition, 3 subjects developed cancer. No subjects had cirrhosis. At data censorship, 13 subjects were alive.The clinical course for lung transplant recipients with telomerase mutations is complicated by renal disease, leukopenia with intolerance of lymphocyte anti-proliferative agents, and recurrent lower respiratory tract infections. In contrast, cirrhosis was absent, acute cellular rejection was mild, and development of chronic lung allograft dysfunction was comparable to other lung transplant recipients. Although it poses challenges, lung transplantation may be feasible for patients with pulmonary fibrosis from telomerase mutations.

SUBMITTER: Tokman S

PROVIDER: S-EPMC5382798 | biostudies-literature | 2015 Oct

REPOSITORIES: biostudies-literature

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Clinical outcomes of lung transplant recipients with telomerase mutations.

Tokman Sofya S Singer Jonathan P JP Devine Megan S MS Westall Glen P GP Aubert John-David JD Tamm Michael M Snell Gregory I GI Lee Joyce S JS Goldberg Hilary J HJ Kukreja Jasleen J Golden Jeffrey A JA Leard Lorriana E LE Garcia Christine K CK Hays Steven R SR

The Journal of heart and lung transplantation : the official publication of the International Society for Heart Transplantation 20150511 10

<h4>Background</h4>Successful lung transplantation for patients with pulmonary fibrosis from telomerase mutations may be limited by systemic complications of telomerase dysfunction, including myelosuppression, cirrhosis, and malignancy. We describe clinical outcomes in 14 lung transplant recipients with telomerase mutations.<h4>Methods</h4>Subjects underwent lung transplantation between February 2005 and April 2014 at 5 transplant centers. Data were abstracted from medical records, focusing on o ...[more]

PMID: 26169663

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Project description:Medication nonadherence after liver transplantation (LT) is associated with adverse clinical outcomes such as graft rejection and graft loss. Few studies have examined nonadherence and its impact on clinical outcomes in LT. The study objectives were (1) to evaluate medication understanding (with treatment knowledge and demonstrated regimen use scores) and medication adherence or nonadherence to entire regimens among LT recipients and (2) to examine associations of these exposures with clinical outcomes. We conducted a 2-site study of 105 recipients between 2011 and 2012 at 2 transplant centers in Chicago, IL and Atlanta, GA. Data were collected via detailed, in-person interviews and medical record reviews. Study participants were middle-aged and predominantly male; 15% of the sample had limited literacy. On average, patients were taking 11 medications [standard deviation (SD)?=?4], and 39% had undergone a medication change within the last month. The average scores for the entire medication regimen were 86% (SD?=?22%) for treatment knowledge and 78% (SD?=?22%) for demonstrated regimen use. The mean score for self-reported nonadherence to the entire regimen was 14% (SD?=?20%), whereas 32% of the patients were nonadherent according to tacrolimus levels. In multivariate analyses, lower income, less time since transplantation, a higher number of medications, and limited literacy were inversely associated with treatment knowledge scores (all P?<?0.05), whereas limited literacy was associated with nonadherence according to tacrolimus levels (P?<?0.05). In multivariate models, higher scores for treatment knowledge [incidence rate ratio (IRR)?=?0.85, 95% confidence interval (CI)?=?0.74-0.97] and demonstrated regimen use (IRR?=?0.87, 95% confidence interval?=?0.77-0.98) were independently associated with 15% and 13% reductions in the number of posttransplant rehospitalizations, respectively. Inadequate treatment knowledge and improper regimen use may be significant determinants of unintentional nonadherence among LT recipients and are associated with adverse clinical outcomes.

Project description:Background and objectivesEmerging evidence from recently published observational studies and an individual patient data meta-analysis shows that mammalian target of rapamycin inhibitor use in kidney transplantation is associated with increased mortality. Therefore, all-cause mortality and allograft loss were compared between use and nonuse of mammalian target of rapamycin inhibitors in patients from Australia and New Zealand, where mammalian target of rapamycin inhibitor use has been greater because of heightened skin cancer risk.Design, setting, participants, & measurementsOur longitudinal cohort study included 9353 adult patients who underwent 9558 kidney transplants between January 1, 1996 and December 31, 2012 and had allograft survival ?1 year. Risk factors for all-cause death and all-cause and death-censored allograft loss were analyzed by multivariable Cox regression using mammalian target of rapamycin inhibitor as a time-varying covariate. Additional analyses evaluated mammalian target of rapamycin inhibitor use at fixed time points of baseline and 1 year.ResultsPatients using mammalian target of rapamycin inhibitors were more likely to be white and have a history of pretransplant cancer. Over a median follow-up of 7 years, 1416 (15%) patients died, and 2268 (24%) allografts were lost. There was a higher risk of all-cause mortality with time-varying mammalian target of rapamycin inhibitor use (hazard ratio, 1.47; 95% confidence interval, 1.23 to 1.76) as well as in the fixed time model analyses comparing mammalian target of rapamycin inhibitor use at baseline (hazard ratio, 1.54; 95% confidence interval, 1.22 to 1.93) and 1 year (hazard ratio, 1.63; 95% confidence interval, 1.32 to 2.01). Time-varying mammalian target of rapamycin inhibitor use was associated with higher risk of death because of malignancy (hazard ratio, 1.37; 95% confidence interval, 1.09 to 1.71). There were no statistically significant differences in the risk of all-cause (hazard ratio, 0.98; 95% confidence interval, 0.85 to 1.12) and death-censored (hazard ratio, 0.85; 95% confidence interval, 0.69 to 1.03) allograft loss between the mammalian target of rapamycin inhibitor use and nonuse groups in the time-varying model as well as the fixed time models.ConclusionsMammalian target of rapamycin inhibitor use was associated with a higher risk of all-cause mortality but not allograft loss.

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Clinical outcomes of lung transplant recipients with telomerase mutations.

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Clinical outcomes of lung transplant recipients with telomerase mutations.

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