Outcomes of Lung Transplantation in Patients with Telomere Related Forms of Progressive Fibrosing Interstitial Lung Disease Pulmonary Fibrosis - a Systematic Review
JHLT Open(2024)
Abstract
Lung transplantation (LTX) is the last life-extending option for patients with progressive fibrosing interstitial lung diseases (fILD). Between 12 and 71% of patients with fILD are patients with underlying telomere-dysfunction (trILD) related to pathogenic telomere-related gene (TRG) variants and/or short telomere length. TrILD patients tend to have earlier disease onset, faster progression, and worse prognosis causing them to be referred for LTX more often.
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Key words
fILD,ILD,trILD,IPF,PPF,LTX,GV,TRG,TBD,ISHLT,PGD,ACR,CLAD,PRISMA,TL,HR,95% CI,OR,ECMO,CMV,WGS,WES,NGS
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