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Possible drug-interaction between elexacaftor-tezacaftor-ivacaftor and repaglinide in an adult with cystic fibrosis-related diabetes.
Can J Diabetes
December 2024
Division of Endocrinology & Metabolism, Department of Medicine, Nova Scotia Health. QEII - Victoria Building, Suite 7-North-046 Victoria Building, 1276 South Park Street, Halifax, Nova Scotia, Canada, B3H 2Y9.
Changing profile of bacterial infection and microbiome in cystic fibrosis: when to use antibiotics in the era of CFTR-modulator therapy.
Eur Respir Rev
October 2024
Department of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany
The advent of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy, especially the triple therapy combining the drugs elexacaftor, tezacaftor, ivacaftor (ETI), has significantly changed the course of the disease in people with cystic fibrosis (pwCF). ETI, which is approved for the majority (80-90%) of pwCF, partially restores CFTR channel function, resulting in improved mucociliary clearance and, consequently, improved lung function, respiratory symptoms and pulmonary exacerbations. The bacterial burden of classical CF pathogens such as and is reduced without reaching eradication in the majority of infected patients.
View Article and Find Full Text PDFElexacaftor-tezacaftor-ivacaftor use after solid organ transplant.
Curr Opin Pulm Med
November 2024
Division of Pulmonary, Critical Care, and Sleep Medicine, Department of Medicine, University of Washington, Seattle, Washington, USA.
Purpose Of Review: In 2019, the United States Food and Drug Administration approved a breakthrough therapeutic for cystic fibrosis, elexacaftor-tezacaftor-ivacaftor (ETI), because of its profound effect on lung function in large phase III clinical trials. ETI acts directly on the dysfunctional protein that causes the systemic manifestations of cystic fibrosis and also leads to improvement in nonpulmonary symptoms of cystic fibrosis. Transplant recipients were excluded from the pivotal clinical trials of ETI but may stand to benefit from correction of the underlying protein defect.
View Article and Find Full Text PDFSafety and efficacy of elexacaftor/tezacaftor/ivacaftor in people with Cystic Fibrosis following liver transplantation: A systematic review.
J Cyst Fibros
May 2024
Meyer Children's Hospital IRCCS, Department of Paediatric Medicine, Cystic Fibrosis Regional Reference Center, Florence, Italy. Electronic address:
Background & Aims: Cystic Fibrosis (CF) liver disease progresses to liver failure requiring transplantation in about 3 % of patients, 0.7 % of CF patients are post liver transplant. The prognosis of CF has improved with the introduction of elexacaftor/tezacaftor/ivacaftor (ETI).
View Article and Find Full Text PDFElexacaftor/tezacaftor/ivacaftor in liver or kidney transplanted people with cystic fibrosis using tacrolimus, a drug-drug interaction study.
J Cyst Fibros
May 2024
Department of Pulmonology and Adult CF Centre, University Medical Centre Utrecht, Heidelberglaan 100, Utrecht 3584 CX, The Netherlands.
Background: The use of elexacaftor/tezacaftor/ivacaftor (ETI) in people with cystic fibrosis (pwCF) after solid organ transplantation is controversial because of potential drug-drug interactions (DDI) with tacrolimus. We aimed to improve insight into the safety and clinical benefits of co-administration of ETI and tacrolimus in liver or kidney transplanted adult pwCF.
Methods: In 5 pwCF, tacrolimus concentrations were monitored during 2 weeks before and 4 weeks after starting ETI treatment.
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