Results 91 to 100 of about 3,103 (172)

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

Adult Diagnosis of Cystic Fibrosis: A Cause of Recurrent Pneumonia

Annals of Internal Medicine: Clinical Cases
We describe a patient presenting for evaluation of nearly annual pneumonias since the age of 9 years, eventually requiring multiple hospitalizations. Genetic testing revealed compound heterozygous mutations for 2184insA and L206W, indicative of cystic ...
Amira Elsabagh, Ashley Dean, Anthony Loschner   +2 more
doaj   +1 more source

Drug Responsiveness in Patient-Derived Rectal Organoids Correlates with Clinical Response in CF Subjects: A Real-Life Analysis

Scientia Pharmaceutica
Pharmacological modulators of CFTR have significantly changed the cystic fibrosis (CF) phenotype of subjects affected by this multi-organ disease.
Karina Kleinfelder, Paola Lecca, Roberta Valeria Latorre, Chiara Mortali, Sara Casati, Sofia Vanerio, Claudio Sorio, Paola Melotti   +7 more
doaj   +1 more source

Elexacaftor/Tezacaftor/Ivacaftor Population Pharmacokinetics in Pediatric Patients With Cystic Fibrosis

Elexacaftor/tezacaftor/ivacaftor (ETI) significantly improves treatment outcomes for people with cystic fibrosis (pwCF) with at least one F508del allele. In 2023, the Food and Drug Administration approved ETI for children with CF aged 2–5 years. However,
Harriet Corvol, Aurélie Tatopoulos, Sihem Benaboud, Véronique Houdoin, Emmanuelle Bardin, Raphael Chiron, Nathalie Stremler, Léo Froelicher‐Bournaud, Steeve Rouillon, Philippe Reix, Jeanne Languepin, Jean‐Marc Treluyer, Tiphaine Bihouee, Marie Mittaine, Frantz Foissac, Naïm Bouazza, Laurence Weiss, Françoise Troussier, Caroline Perisson, Marie‐Laure Dalphin, Gabrielle Lui, Stéphanie Bui, Isabelle Sermet‐Gaudelus, Cathy Llerena, Eric Deneuville, MODUL‐CF study group, Rames Cinthia, Sophie Ramel, Ngoc Hoa Truong, Mahassen Barboura, Michel Miralles, Muriel Laurans   +31 more
core   +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

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

sj-pdf-3-tar-10.1177_17534666241235054 – Supplemental material for Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor–tezacaftor–ivacaftor combination: a 1-year observational study

Supplemental material, sj-pdf-3-tar-10.1177_17534666241235054 for Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor–tezacaftor–ivacaftor combination: a 1-year observational study by Chiara Blardone, Simone ...
Rachele Galgani (18279158), Anna Luisa Tutino (18279167), Dario Laquintana (18279173), Alessandra Mariani (18279155), Francesco Blasi (61418), Rita Maria Nobili (18279161), Valeria Daccò (18279170), Simone Gambazza (11848347), Martina Contarini (16388017), Carmela Rizza (18279164), Andrea Gramegna (5890742), Anna Brivio (12336894), Chiara Blardone (18279152)   +12 more
core   +1 more source

Impact of CFTR modulator concentrations on clinical response in cystic fibrosis. [PDF]

Eur Respir J
Chalamalla AR, Baker E, Ryan KJ, Dowell A, Natt JR, Zemanick ET, Konstan MW, Mayer-Hamblett N, Acosta EP, Guimbellot JS, CHEC-SC investigators.   +10 more
europepmc   +1 more source

Effect of elexacaftor-tezacaftor-ivacaftor therapy on systemic inflammation in people with cystic fibrosis. [PDF]

J Bras Pneumol
Meyer VMC, Aranha Netto N, Palmeira P, Athanazio RA, Silva-Filho LVRFD.   +4 more
europepmc   +1 more source

sj-pdf-1-tar-10.1177_17534666241235054 – Supplemental material for Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor–tezacaftor–ivacaftor combination: a 1-year observational study

Supplemental material, sj-pdf-1-tar-10.1177_17534666241235054 for Perceived burden of respiratory physiotherapy in people with cystic fibrosis taking elexacaftor–tezacaftor–ivacaftor combination: a 1-year observational study by Chiara Blardone, Simone ...
Rachele Galgani (18279158), Anna Luisa Tutino (18279167), Dario Laquintana (18279173), Alessandra Mariani (18279155), Francesco Blasi (61418), Rita Maria Nobili (18279161), Valeria Daccò (18279170), Simone Gambazza (11848347), Martina Contarini (16388017), Carmela Rizza (18279164), Andrea Gramegna (5890742), Anna Brivio (12336894), Chiara Blardone (18279152)   +12 more
core   +1 more source