Bridging for lung transplantation with lumacaftor/ivacaftor
| Autor: | Pedersen, Søren Sperling, Jensen-Fangel, Søren, Jeppesen, Majbritt, Søgaard, Ole Schmeltz |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2018 |
| Předmět: |
Pulmonary and Respiratory Medicine
congenital hereditary and neonatal diseases and abnormalities medicine.medical_specialty medicine.medical_treatment Case Report Gastroenterology Cystic fibrosis Ivacaftor 03 medical and health sciences chemistry.chemical_compound 0302 clinical medicine Internal medicine medicine Lung transplantation 030212 general & internal medicine Respiratory system Young female lcsh:RC705-779 business.industry Lumacaftor lcsh:Diseases of the respiratory system respiratory system Expert Opinion medicine.disease digestive system diseases respiratory tract diseases 030228 respiratory system chemistry business medicine.drug |
| Zdroj: | Breathe, Vol 14, Iss 2, Pp e68-e71 (2018) Pedersen, S S, Jensen-Fangel, S & Jeppesen, M 2018, ' Bridging for lung transplantation with lumacaftor/ivacaftor ', Breathe, bind 14, nr. 2, s. e68-e71 . https://doi.org/10.1183/20734735.019318 Pedersen, S S, Jensen-Fangel, S, Jeppesen, M & Søgaard, O S 2018, ' Bridging for lung transplantation with lumacaftor/ivacaftor ', Breathe, vol. 14, no. 2, pp. e68-e71 . https://doi.org/10.1183/20734735.019318 Breathe |
| ISSN: | 2073-4735 1810-6838 |
| Popis: | Cystic fibrosis (CF) is a multi-organ disease caused by mutations in the cystic fibrosis transmembrane regulator (CFTR) gene, with consequent dysfunction of the CFTR channel. The most common mutation in CF is delta F508. CFTR-modulating therapy with lumacaftor/ivacaftor corrects (lumacaftor) the misfolded CFTR channel and potentiates (ivacaftor) its function at the cell surface [1]. The case of a young female cystic fibrosis patient, homozygous for delta F508 and with terminal respiratory insufficiency, who started treatment with lumacaftor/ivacaftor http://ow.ly/4I0t30kftDx |
| Databáze: | OpenAIRE |
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