The First Successful Lung Transplantation in a Korean Child with Cystic Fibrosis
Journal of Korean Medical Science
; : 2073-2078, 2017.
Article
em En
| WPRIM
| ID: wpr-158106
Biblioteca responsável:
WPRO
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.
Palavras-chave
Texto completo:
1
Índice:
WPRIM
Assunto principal:
Pneumonia
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Pseudomonas aeruginosa
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Qualidade de Vida
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Insuficiência Respiratória
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Suor
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Expectativa de Vida
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Mortalidade
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Transplante de Pulmão
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Regulador de Condutância Transmembrana em Fibrose Cística
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Fibrose Cística
Tipo de estudo:
Prognostic_studies
Limite:
Child
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Female
/
Humans
País/Região como assunto:
Asia
Idioma:
En
Revista:
Journal of Korean Medical Science
Ano de publicação:
2017
Tipo de documento:
Article