4.7 Letter

Change in Lung Function after Initiation of Elexacaftor-Tezacaftor-Ivacaftor: Do Not Forget Anatomy!

Related references

Note: Only part of the references are listed.
Article Critical Care Medicine

Effects of Elexacaftor/Tezacaftor/Ivacaftor Therapy on CFTR Function in Patients with Cystic Fibrosis and One or Two F508del Alleles

Simon Y. Graeber et al.

Summary: Treatment with ELX/TEZ/IVA leads to effective improvement of CFTR function in airway and intestinal epithelia in patients with CF and one or two F508del alleles.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

Article Critical Care Medicine

Clinical Effectiveness of Elexacaftor/Tezacaftor/Ivacaftor in People with Cystic Fibrosis

David P. Nichols et al.

Summary: The study demonstrates significant improvements in lung function, respiratory symptoms, and body mass index in CF patients using ETI therapy, regardless of their familiarity with modulators or prior drug use. Significant reductions in sweat chloride concentration were also observed, correlating with improved lung function in the overall study population.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2022)

Article Critical Care Medicine

Rapid Improvement after Starting Elexacaftor-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease

Pierre-Regis Burgel et al.

Summary: An observational study in France found that the use of elexacaftor-tezacaftor-ivacaftor in CF patients led to significant improvements in lung function and weight within a short period of time, with most patients no longer requiring long-term oxygen, noninvasive ventilation, and/or enteral tube feeding. Compared to the previous two years, there was a twofold decrease in the number of lung transplantations in CF patients in 2020.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2021)

Article Cell Biology

Mucus accumulation in the lungs precedes structural changes and infection in children with cystic fibrosis

Charles R. Esther et al.

SCIENCE TRANSLATIONAL MEDICINE (2019)

Article Critical Care Medicine

Morphometric Analysis of Explant Lungs in Cystic Fibrosis

Mieke Boon et al.

AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2016)

Article Respiratory System

A morphometric study of mucins and small airway plugging in cystic fibrosis

Pierre-Regis Burgel et al.

THORAX (2007)