Living-Donor Lung Transplantation for Dyskeratosis Congenita

Ann Thorac Surg. 2021 Dec;112(6):e397-e402. doi: 10.1016/j.athoracsur.2021.02.088. Epub 2021 Mar 19.


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.

Publication types

  • Case Reports

MeSH terms

  • Adolescent
  • Child, Preschool
  • Dyskeratosis Congenita / complications*
  • Female
  • Humans
  • Living Donors
  • Lung Diseases / etiology*
  • Lung Diseases / surgery*
  • Lung Transplantation*
  • Male
  • Postoperative Complications / surgery*