Results 51 to 60 of about 3,564 (173)

Tezacaftor/ivacaftor in people with cystic fibrosis heterozygous for minimal function CFTR mutations

open access: yes, 2020
Background: Tezacaftor/ivacaftor is a CFTR modulator approved to treat people with cystic fibrosis (pwCF) who are homozygous (F/F) or heterozygous for the F508del-CFTR mutation and a residual function mutation (F/RF).
Campbell, Daniel   +7 more
core   +1 more source

Elexacaftor/Ivacaftor/Tezacaftor: First Approval

open access: yes, 2019
Compliance with Ethical StandardsFunding: The preparation of this review was not supported by any external funding. Conflict of interest: Sheridan Hoy is a salaried employee of Adis International Ltd/Springer Nature, is responsible for the article ...
Sheridan M. Hoy (4897468)
core   +1 more source

Individualized approach to elexacaftor/tezacaftor/ivacaftor dosing in cystic fibrosis, in response to self-reported anxiety and neurocognitive adverse events: A case series

open access: yesFrontiers in Pharmacology, 2023
The prevalence of mental health disorders is high among people with Cystic Fibrosis. The psychological symptoms in CF are associated with poor adherence, worse treatment outcomes, and greater health utilization/cost.
Hisham Ibrahim   +19 more
doaj   +1 more source

Lingering Identity as Chronically Ill and the Unanticipated Effects of Life-Changing Precision Medicine in Cystic Fibrosis: A Case Report

open access: yesJournal of Patient Experience, 2021
Cystic fibrosis (CF) is the leading genetic disease among Caucasians; however, advances in diagnosis and treatment have improved both quality and quantity of life for those affected.
Sigrid Ladores PhD, RN, PNP, CNE, FAAN   +1 more
doaj   +1 more source

Malassezia Folliculitis following Triple Therapy for Cystic Fibrosis

open access: yesMedicina, 2022
Triple-combination therapy with elexacaftor, tezacaftor and ivacaftor has been recently approved for cystic fibrosis patients with at least one F508del mutation in the transmembrane conductance regulator of the cystic fibrosis gene.
Federica Li Pomi   +8 more
doaj   +1 more source

Determining the Minimal Clinically Important Difference of the 40‐Item Smell Identification Test in People With Cystic Fibrosis

open access: yesInternational Forum of Allergy &Rhinology, EarlyView.
ABSTRACT Background Chronic rhinosinusitis (CRS) and olfactory dysfunction (OD) are highly prevalent among people with cystic fibrosis (PwCF) and negatively impact quality of life. The 40‐item Smell Identification Test (SIT) is widely used to assess psychophysical olfaction, but a CF‐specific minimal clinically important difference (MCID) has not been ...
Eugene Oh   +34 more
wiley   +1 more source

Pregnancy‐related effect on elexacaftor, tezacaftor and ivacaftor pharmacokinetics in women with cystic fibrosis

open access: yesBritish Journal of Clinical Pharmacology, EarlyView.
Aim The number of pregnancies among women with cystic fibrosis (wwCF) has steadily increased over the past decade. However, the pharmacokinetics (PK) of elexacaftor–tezacaftor–ivacaftor (ETI) during gestation remains uncharacterized, despite its widespread use in this population.
Paulette Magnas   +16 more
wiley   +1 more source

Impact of CFTR modulator use on outcomes in people with severe cystic fibrosis lung disease

open access: yesEuropean Respiratory Review, 2020
Drug compounds that augment the production and activity of the cystic fibrosis (CF) transmembrane regulator (CFTR) have revolutionised CF care. Many adults and some children with CF suffer advanced and severe lung disease or await lung transplantation ...
Michal Shteinberg   +1 more
doaj   +1 more source

Aplasia Cutis Congenita in the Setting of Maternal Cystic Fibrosis

open access: yesAnnals of Internal Medicine: Clinical Cases, 2022
Aplasia cutis congenita is a rare scalp defect. An affected neonate was delivered after in utero exposure beginning in the midtrimester to elexacaftor/tezacaftor/ivacaftor for maternal cystic fibrosis management. Although aplasia cutis congenita has many
Govind Kallumkal, Robert Egerman
doaj   +1 more source

Impact of cystic fibrosis transmembrane conductance regulator modulator therapies on liver stiffness and liver enzymes: An observational perspective single‐center cohort study

open access: yesJPGN Reports, EarlyView.
Abstract Objectives The efficacy of cystic fibrosis transmembrane conductance regulator (CFTR)‐modulator therapies in preventing or ameliorating cystic fibrosis liver disease (CFLD) by correcting CFTR in cholangiocytes is not well‐documented. This study aimed to assess liver function during CFTR‐modulators.
Laura Giugliano   +12 more
wiley   +1 more source

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