Update on advances in cystic fibrosis towards a cure and implications for primary care clinicians
V Terlizzi, PM Farrell - Current Problems in Pediatric and Adolescent …, 2024 - Elsevier
During the past quarter century, the diagnosis and treatment of cystic fibrosis (CF) have
been transformed by molecular sciences that initiated a new era with discovery of the cystic …
been transformed by molecular sciences that initiated a new era with discovery of the cystic …
Elexacaftor-Tezacaftor-Ivacaftor: a life-changing triple combination of CFTR modulator drugs for cystic fibrosis
M Bacalhau, M Camargo, GAV Magalhães-Ghiotto… - Pharmaceuticals, 2023 - mdpi.com
Cystic fibrosis (CF) is a potentially fatal monogenic disease that causes a progressive
multisystemic pathology. Over the last decade, the introduction of CF transmembrane …
multisystemic pathology. Over the last decade, the introduction of CF transmembrane …
Effects of Lumacaftor/Ivacaftor on Cystic Fibrosis Disease Progression in Children 2 through 5 Years of Age Homozygous for F508del-CFTR: A Phase 2 Placebo …
M Stahl, J Roehmel, M Eichinger… - Annals of the …, 2023 - atsjournals.org
Rationale: Lumacaftor/ivacaftor (LUM/IVA) was shown to be safe and well tolerated in
children 2 through 5 years of age with cystic fibrosis (CF) homozygous for F508del-CFTR in …
children 2 through 5 years of age with cystic fibrosis (CF) homozygous for F508del-CFTR in …
Clinical change 2 years from start of elexacaftor‐tezacaftor‐ivacaftor in severe cystic fibrosis
KS McCoy, J Blind, T Johnson, P Olson… - Pediatric …, 2023 - Wiley Online Library
Rationale Limited published research is available on the impact of elexacaftor/tezacaftor/
ivacaftor (ETI) beyond the initial few months postdrug initiation, especially for those who …
ivacaftor (ETI) beyond the initial few months postdrug initiation, especially for those who …
One year of treatment with elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis homozygous for the F508del mutation causes a significant increase in liver …
A Castaldo, M Gelzo, P Iacotucci… - Frontiers in Molecular …, 2024 - frontiersin.org
Introduction: Modulators of cystic fibrosis transmembrane conductance regulator mutated
protein significantly improved the outcome of patients with cystic fibrosis (CF). We describe …
protein significantly improved the outcome of patients with cystic fibrosis (CF). We describe …
Structural changes in lung morphology detected by MRI after modulating therapy with elexacaftor/tezacaftor/ivacaftor in adolescent and adult patients with cystic …
V Fainardi, K Skenderaj, A Ciuni, G Milanese… - Respiratory …, 2023 - Elsevier
Introduction Elexacaftor/tezacaftor/ivacaftor (ELX/TEZ/IVA) improves CFTR function in cystic
fibrosis (CF) patients homozygous or heterozygous for F508del mutation. The aim of the …
fibrosis (CF) patients homozygous or heterozygous for F508del mutation. The aim of the …
The Role of the Dietitian
I Asseiceira, C Smith, J Calvo-Lerma - Hodson and Geddes' Cystic … - taylorfrancis.com
Nutritional management is key in the treatment of cystic fibrosis (CF) patients since
nutritional status is directly related to pulmonary function and, thus, survival. Improved …
nutritional status is directly related to pulmonary function and, thus, survival. Improved …
[引用][C] Radiological Changes After Treatment With Triple Therapy in Cystic Fibrosis. Are Bronchiectasis Irreversible?
CM Carbajal, BP Antequera… - … : Organo oficial de la …, 2023 - dialnet.unirioja.es