Lumacaftor/ Ivacaftor improves exercise tolerance in patients with Cystic Fibrosis and severe airflow obstruction

Autor:	Theeba Thiruchelvam, John D. Brannan, Kim Cookson, Peter A. B. Wark, Douglas J. Dorahy
Rok vydání:	2018
Předmět:	Male Cystic Fibrosis medicine.medical_treatment Aminopyridines Cystic Fibrosis Transmembrane Conductance Regulator Quinolones Aminophenols Cystic fibrosis Ivacaftor chemistry.chemical_compound 0302 clinical medicine DLCO 030212 general & internal medicine Exercise Tolerance medicine.diagnostic_test Lumacaftor Homozygote Middle Aged Respiratory Function Tests Drug Combinations Lung transplantation Female medicine.drug Research Article Pulmonary and Respiratory Medicine Spirometry Adult medicine.medical_specialty Genotype Walk Test 03 medical and health sciences Young Adult Internal medicine medicine Plethysmograph Humans Benzodioxoles lcsh:RC705-779 business.industry Australia lcsh:Diseases of the respiratory system medicine.disease Clinical trial 030228 respiratory system chemistry Six minute walk test Mutation business
Zdroj:	BMC Pulmonary Medicine BMC Pulmonary Medicine, Vol 19, Iss 1, Pp 1-8 (2019)
ISSN:	1471-2466 1261-9000
Popis:	Background Treatment of patients with Cystic Fibrosis homozygous for the Phe508del gene, with Lumacaftor /Ivacaftor (LUM/IVA) improves outcomes in patients with FEV1 > 40% predicted. We set out to observe the most sensitive clinical measure that would change with treatment in terms of exercise capacity or lung function in adults with severe lung disease as defined by an FEV1
Databáze:	OpenAIRE
Externí odkaz:	https://explore.openaire.eu/search/publication?articleId=doi_dedup___::560c1bf50191fe2f2a7e58898f30d553 https://pubmed.ncbi.nlm.nih.gov/31208380 Zobrazit plný text záznamu Full text from SpringerLink