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dc.contributorDepartament de Salut
dc.contributor.authorPrograma d'Harmonització Farmacoterapèutica
dc.date.accessioned2020-11-06T08:03:27Z
dc.date.available2020-11-06T08:03:27Z
dc.date.issued2019-10-15
dc.identifier.citationPrograma d'Harmonització Farmacoterapèutica. Lumacaftor/ivacaftor (Orkambi®) per al tractament de la fibrosi quística. Barcelona: Servei Català de la Salut; 2020.
dc.identifier.urihttp://hdl.handle.net/11351/5393
dc.descriptionLumacaftor; Ivacaftor; Cystic fibrosis; Minority disease
dc.description.abstractCystic fibrosis (CF) is an autosomal recessive disorder caused by mutations in the gene that encodes the transmembrane conductance regulator of CF (CFTR), a protein that acts as a chloride channel. Viscous secretions resulting from altered transport of chloride and sodium progressively lead to dysfunction of the exocrine glands of multiple organic systems, and mainly affect the respiratory system but also the pancreas, bile ducts, sweat glands and genitourinary system. The most common manifestations of the disease are exocrine pancreatic insufficiency in about 85-90% of cases and severe chronic obstructive pulmonary disease, the leading cause of morbidity and mortality in these patients.
dc.language.isocat
dc.publisherServei Català de la Salut
dc.rightsAttribution-NonCommercial-NoDerivatives 4.0 International
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/
dc.sourceScientia
dc.subjectFibrosi quística - Tractament
dc.subjectMedicaments - Assaigs clínics
dc.subjectAvaluació de resultats (Assistència sanitària)
dc.subject.meshCystic Fibrosis
dc.subject.mesh/drug therapy
dc.subject.meshDrug Evaluation
dc.subject.meshClinical Trials as Topic
dc.titleLumacaftor/ivacaftor (Orkambi®) per al tractament de la fibrosi quística
dc.typeinfo:eu-repo/semantics/report
dc.subject.decsfibrosis quística
dc.subject.decs/farmacoterapia
dc.subject.decsevaluación de medicamentos
dc.subject.decsensayos clínicos como asunto
dc.type.versioninfo:eu-repo/semantics/publishedVersion
dc.audienceProfessionals
dc.rights.accessrightsinfo:eu-repo/semantics/openAccess


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