Prognostic significance of pulmonary function tests in dyskeratosis congenita, a telomere biology disorder
Pulmonary fibrosis and pulmonary arteriovenous malformations are known manifestations of dyskeratosis congenita (DC), a telomere biology disorder (TBD) and inherited bone marrow failure syndrome caused by germline mutations in telomere maintenance genes resulting in very short telomeres. Baseline pu...
Main Authors: | , , , , , , |
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Format: | Article |
Language: | English |
Published: |
European Respiratory Society
2019-11-01
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Series: | ERJ Open Research |
Online Access: | http://openres.ersjournals.com/content/5/4/00209-2019.full |