期刊
ANNALS OF THORACIC SURGERY
卷 112, 期 6, 页码 E397-E402出版社
ELSEVIER SCIENCE INC
DOI: 10.1016/j.athoracsur.2021.02.088
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Two cases of dyskeratosis congenita in patients with progressive pulmonary diseases underwent urgent living-donor lung transplantations and recovered uneventfully. The surgeries were successful and resulted in good outcomes.
This report describes 2 cases of dyskeratosis congenita in patients with progressive pulmonary diseases who required urgent living-donor lung transplantations. In the first case, a 13-year-old boy underwent bone marrow transplantation for aplastic anemia at age of 6 years. Severe pulmonary right-to-left shunting and fibrosis developed, and his condition deteriorated with acute fibrosis exacerbation and increased intrapulmonary shunt. He underwent lung transplantation and recovered uneventfully. In the second case, a 3-year-old girl with Revesz syndrome underwent bone marrow transplantation for refractory cytopenia. At 6 years of age, she had progressive hypoxia and a brain abscess. Her respiratory condition worsened, and she recovered uneventfully after urgent lung transplantation. (C) 2021 by The Society of Thoracic Surgeons
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