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Successful Desensitization With ELX/TEZ/IVA.
J Pediatr Pharmacol Ther
October 2024
Pediatric Allergy-Immunology Department (DFKU, MAK, AB), Akdeniz University School of Medicine, Pediatric Pulmonology Department (BB, AEB), Akdeniz University School of Medicine, Antalya, Turkey.
Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) was given US Food and Drug Administration approval based on its therapeutic benefits to treat patients with cystic fibrosis (CF) who had at least 1 allele of the CF transmembrane conductance regulator (CFTR) with phenylalanine deleted at position 508 (F508del). The increase in genotyping studies has increased the frequency of use of CFTR modulators; however, severe allergic reactions to CFTR modulators have also been described. It is critical to avoid the offending medication and select alternative treatments while dealing with drug allergies.
View Article and Find Full Text PDFElexacaftor/tezacaftor/ivacaftor, a game-changer in cystic fibrosis: The Portuguese experience.
Pulm Pharmacol Ther
December 2024
Pulmonology Department, Centro Hospitalar Universitário de Lisboa Norte, ULS de Santa Maria, Lisboa, Portugal; Lisbon School of Medicine, Universidade de Lisboa, Lisboa, Portugal.
Background: Phase 3 trials of elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) combination treatment in people with cystic fibrosis (CF) with ≥1 F508del-CFTR allele showed profound short-term effects on lung function, weight, and pulmonary exacerbations (PEx). The authors conducted a 12-month study to add evidence on the real-world long-term effectiveness and safety of CFTR modulator therapy with ELX/TEZ/IVA in Portuguese CF adult population.
Methods: Ambispective, multicentre, observational, real-life study involving all the Portuguese CF Reference Centres.
Cystic fibrosis: desensitization in delayed hypersensitivity reactions to elexacaftor/tezacaftor/ivacaftor.
Front Pharmacol
June 2024
Cystic Fibrosis Unit, Pulmonology Department, Vall d'Hebron University Hospital, Barcelona, Spain.
Cystic fibrosis transmembrane conductance regulator modulators are the only available treatment for cystic fibrosis. Although elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) is well-tolerated, rash has been reported as very frequent. In severe rashes, ELX/TEZ/IVA withdrawal is necessary, leading to clinical deterioration.
View Article and Find Full Text PDFCase Report: When cystic fibrosis, elexacaftor/tezacaftor/ivacaftor therapy, and alpha1 antitrypsin deficiency get together.
Front Pediatr
April 2024
Pediatric Pulmonology and Cystic Fibrosis Unit, Department Women-Mother-Child, Service of Pediatrics, Lausanne University Hospital and University of Lausanne, Lausanne, Switzerland.
In the last 10 years, the care of patients with cystic fibrosis (CF) has been revolutionized with the introduction of (CFTR) modulator drugs, with a major impact on symptoms and life expectancy, especially considering the newest and highly effective elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) therapy. Conversely, adverse effects are relatively frequent, with some being life-threatening, such as severe hepatitis. Clinical trials on children starting CFTR modulators have reported transaminase elevations >3× upper limit of the norm in 10%-20% of patients, whereas real-life studies have reported discontinuation rates three times higher than those observed in phase 3 trials.
View Article and Find Full Text PDFImpact of CFTR modulator therapy on body composition as assessed by thoracic computed tomography: A follow-up study.
Nutrition
July 2024
Department of Endocrinology and Nutrition, Hospital Universitario de La Princesa, Instituto de Investigación Sanitaria de La Princesa, Madrid, Spain.
Objective: Treatment with cystic fibrosis transmembrane conductance regulator (CFTR) modulators in individuals with cystic fibrosis (CF) has brought a significant change in forced expiratory volume in 1 second (FEV) and clinical parameters. However, it also results in weight gain. The aim of our study is to evaluate the effect of CFTR modulator treatment on body composition, measured by computed tomography (CT).
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