Results 111 to 120 of about 3,864 (165)

CFTR modulators: from mechanism to targeted therapeutics [PDF]

People with cystic fibrosis (CF) suffer from a multi-organ disorder caused by loss-of-function variants in the gene encoding the epithelial anion channel cystic fibrosis transmembrane conductance regulator (CFTR).
Hwang, Tzyh-Chang, Sheppard, David N, Sutcliffe, Katy, Yeh, Han-I   +3 more
core   +1 more source

Effect of Modulator Therapies on Nutritional Risk Index in Adults with Cystic Fibrosis:A Prospective Cohort Study [PDF]

Background: Modulator therapies improve weight and body mass index (BMI) in cystic fibrosis (CF) patients. We aimed to compare the nutritional risk index (NRI) in adult CF patients receiving modulator (MT) or only non-modulator (conventional) therapies ...
Akman, Esen Deniz, Allegaert, Karel, Damadoğlu, Ebru, Demirkan, Kutay, Kalyoncu, Ali Fuat, Karcıoğlu, Oğuz, Yalçın, Nadir   +6 more
core   +2 more sources

The Changing Landscape of Treatment for Cystic Fibrosis Related Diabetes

Journal of Clinical & Translational Endocrinology
Objective: Patients with Cystic Fibrosis related diabetes [CFRD] are treated with insulin and high calorie diets to maintain body mass. The combined CFTR modulator elexacaftor/tezacaftor/ivacaftor [ETI] decreases pulmonary exacerbations and improves ...
Mehdia Amini, Kevin Yu, Jessica Liebich, Vaishaliben Ahir, Emily Wood, Stewart Albert, Sandeep Dhindsa   +6 more
doaj   +1 more source

Effect of the Cystic Fibrosis Drug Elexacaftor on the Murine Lower Esophageal Sphincter May Explain the GERD Symptoms of Patients [PDF]

Background: Cystic fibrosis is a life-limiting genetic disorder that primarily affects the lungs and digestive system. Cystic fibrosis (CF) is caused by over 2000 different mutations in the CFTR anion channel.
Sawyer, Xena
core  

Scedosporium infection as a late complication after start of elexacaftor/tezacaftor/ivacaftor: two case reports

Respiratory Medicine Case Reports
Highly effective modulator treatment (HEMT) has resulted in an improved prognosis for people with cystic fibrosis (pwCF). In this case report, we present two pwCF with clinical stabilization after start of HEMT who developed complicated fungal infections
P van Mourik, MD PhD, E.C. van der Hout, MD, H.D. Luijk, MD PhD, R.W. Hofland, MD PhD, A.H.W. Bruns, MD PhD, H.G.M. Heijerman, MD PhD, I. Bronsveld, MD PhD   +6 more
doaj   +1 more source

Real world study on elexacaftor‐tezacaftor‐ivacaftor impact on cholesterol levels in adults with cystic fibrosis [PDF]

, 2023
Kevin Lonabaugh, G. Li, R. List, Reyna Huang, Amber James, Andrew Barros, Lindsay Somerville, Dana Albon   +7 more
openalex   +1 more source

Personalised CFTR Modulator Treatment Initiation and Monitoring in CF‐Related Liver Disease: When Less Is More

Respirology Case Reports
Hepatotoxicity due to Elexacaftor/Tezacaftor/Ivacaftor (ETI) use has been well documented. There are no dose adjustments or increased‐frequency monitoring algorithms recommended for people who experience elevated transaminases without cirrhosis, only ...
Sona Vekaria, Grace Kavanagh, Siobhain Mulrennan   +2 more
doaj   +1 more source

CFTR Modulator Therapy in the Treatment of Cystic Fibrosis as Compared to Placebo [PDF]

Objective: To assess the efficacy of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies including ivacaftor, tezacaftor/ivacaftor, and elexacaftor/tezacaftor/ivacaftor combinations in the improvement of lung function in ...
Doody, Rebecca Leigh, Hall, Harrison James   +1 more
core   +1 more source

P049 Effects of elexacaftor/tezacaftor/ivacaftor after 6 months in Dutch people with cystic fibrosis with at least 1 F508del mutation [PDF]

, 2023
Inez Bronsveld, D. Weldam, M. Bierlaagh, Danya Muilwijk, Klaas Winter, Bert Arets, Regina W. Hofland, E. van der Hout, Harry Heijerman, Cornelis K. van der Ent   +9 more
openalex   +1 more source

ICER releases evidence report on elexacaftor/tezacaftor/ivacaftor for cystic fibrosis [PDF]

, 2020

openalex   +1 more source