Living-Donor Lung Transplantation for Dyskeratosis Congenita

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

Automated summary

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.