The First Successful Lung Transplantation in a Korean Child with Cystic Fibrosis.
10.3346/jkms.2017.32.12.2073
- Author:
Soo Ran NOH
1
;
Eun LEE
;
Jisun YOON
;
Sungsu JUNG
;
Song I YANG
;
Jinho YU
;
Soo Jong HONG
Author Information
1. Department of Pediatrics, Childhood Asthma Atopy Center, University of Ulsan College of Medicine, Asan Medical Center, Seoul, Korea. sjhong@amc.seoul.kr
- Publication Type:Case Report
- Keywords:
Cystic Fibrosis;
Lung Transplantation;
Child;
Korea
- MeSH:
Child*;
Cystic Fibrosis Transmembrane Conductance Regulator;
Cystic Fibrosis*;
Diabetes Mellitus;
Female;
Humans;
Korea;
Life Expectancy;
Lung Diseases;
Lung Transplantation*;
Lung*;
Malnutrition;
Mortality;
Pneumonia;
Pseudomonas aeruginosa;
Quality of Life;
Renal Insufficiency, Chronic;
Respiratory Insufficiency;
Sweat
- From:Journal of Korean Medical Science
2017;32(12):2073-2078
- CountryRepublic of Korea
- Language:English
-
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.
