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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...
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| Published in: | ERJ Open Res |
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| Main Authors: | , , , , , , |
| Format: | Artigo |
| Language: | Inglês |
| Published: |
European Respiratory Society
2019
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| Subjects: | |
| Online Access: | https://ncbi.nlm.nih.gov/pmc/articles/PMC6856494/ https://ncbi.nlm.nih.gov/pubmed/31754622 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1183/23120541.00209-2019 |
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