Impact of lumacaftor/ivacaftor on the bacterial and fungal respiratory pathogens in cystic fibrosis: a prospective multicenter cohort study in Sweden

A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden. The aim of this study was to determine the impact of luma...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	Therapeutic advances in respiratory disease 2024-01, Vol.18, p.17534666241254090
Hauptverfasser:	Al Shakirchi, Mahasin, Sorjonen, Kimmo, Hjelte, Lena, Klingspor, Lena, Bergman, Peter, Ericson, Petrea, Svedberg, Marcus, Lindberg, Ulrika, Hansen, Christine, Monestrol, Isabelle de
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Adult Aminophenols - therapeutic use Aminopyridines - administration & dosage Aminopyridines - adverse effects Aminopyridines - pharmacology Aminopyridines - therapeutic use bacteria Bacterial Infections - drug therapy Bacterial Infections - microbiology Benzodioxoles - therapeutic use Child Chloride Channel Agonists - therapeutic use cystic fibrosis Cystic Fibrosis - drug therapy Cystic Fibrosis - microbiology Cystic Fibrosis - physiopathology Cystic Fibrosis Transmembrane Conductance Regulator - genetics Drug Combinations Female fungi Fungi - isolation & purification Humans lumacaftor/ivacaftor Lung - drug effects Lung - microbiology Lung - physiopathology Male Medicin och hälsovetenskap Mycoses - drug therapy Mycoses - microbiology Pediatrics Pediatrik Prospective Studies Quinolones - pharmacology Respiratory Tract Infections - diagnosis Respiratory Tract Infections - drug therapy Respiratory Tract Infections - microbiology Sweden Time Factors Treatment Outcome Young Adult
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Beschreibung
Zusammenfassung:	A significant decline in pulmonary exacerbation rates has been reported in CF patients homozygous for F508del treated with lumacaftor/ivacaftor. However, it is still unclear whether this reduction reflects a diminished microbiological burden. The aim of this study was to determine the impact of lumacaftor/ivacaftor on the bacterial and fungal burden. The study is a prospective multicenter cohort study including 132 CF patients homozygous for F508del treated with lumacaftor/ivacaftor. Clinical parameters as well as bacterial and fungal outcomes 1 year after initiation of lumacaftor/ivacaftor were compared to data from 2 years prior to initiation of the treatment. Changes in the slope of the outcomes before and after the onset of treatment were assessed. Lung function measured as ppFEV1 (
ISSN:	1753-4666 1753-4658 1753-4666
DOI:	10.1177/17534666241254090