The First Successful Lung Transplantation in a Korean Child with Cystic Fibrosis.
Soo Ran NohEun LeeJisun YoonSungsu JungSong I YangJinho YuSoo-Jong HongPublished in: Journal of Korean medical science (2018)
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.
Keyphrases
- cystic fibrosis
- respiratory failure
- extracorporeal membrane oxygenation
- pseudomonas aeruginosa
- acute respiratory distress syndrome
- chronic kidney disease
- lung function
- mechanical ventilation
- biofilm formation
- end stage renal disease
- protein protein
- acinetobacter baumannii
- amino acid
- mental health
- health information
- metabolic syndrome
- healthcare
- copy number
- glycemic control
- gene expression
- escherichia coli
- intensive care unit
- weight loss
- dna methylation
- adipose tissue
- muscular dystrophy
- insulin resistance