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The First Successful Lung Transplantation in a Korean Child with Cystic Fibrosis.

Authors
  • Noh, Soo Ran1
  • Lee, Eun2
  • Yoon, Jisun1, 3
  • Jung, Sungsu1, 3
  • Yang, Song I4
  • Yu, Jinho1
  • Hong, Soo Jong1, 5
  • 1 Department of Pediatrics, Childhood Asthma Atopy Center, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. , (North Korea)
  • 2 Department of Pediatrics, Chonnam National University Hospital, Gwangju, Korea. , (North Korea)
  • 3 Environmental Health Center, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. , (North Korea)
  • 4 Department of Pediatrics, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Anyang, Korea. , (North Korea)
  • 5 Environmental Health Center, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. [email protected] , (North Korea)
Type
Published Article
Journal
Journal of Korean medical science
Publication Date
Dec 01, 2017
Volume
32
Issue
12
Pages
2073–2078
Identifiers
DOI: 10.3346/jkms.2017.32.12.2073
PMID: 29115094
Source
Medline
Keywords
License
Unknown

Abstract

Cystic fibrosis (CF) is an autosomal recessive inherited multisystem disorder caused by mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Respiratory failure remains the most frequent cause of morbidity and mortality. Lung transplantation is the only option to treat end-stage lung disease. Very few cases of CF occur in Koreans. We report the case of a 12-year-old girl with respiratory failure due to CF who underwent lung transplantation. She had been diagnosed with CF 8 years previously after being treated for recurrent Pseudomonas aeruginosa pneumonia and malnutrition based on sweat chloride concentrations and the CFTR protein gene mutation test. Progression to end-stage lung disease and respiratory failure led to registration with the Korean Network for Organ Sharing. She underwent successful double lung transplantation in 2014. Although she has diabetes mellitus and chronic kidney disease, she has a better quality of life and a prolonged life expectancy.

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