Results 61 to 70 of about 6,985 (222)

Impact of elexacaftor-tezacaftor-ivacaftor in lung transplantation for cystic fibrosis in the United States

JHLT Open
Background: Cystic fibrosis (CF) is an autosomal recessive condition leading to progressive lung disease and often necessitating lung transplantation. Historically, CF has been one of the leading indications for lung transplants in the United States. The
Tahuanty A. Pena, MD, MS, Brittany Wright, PharmD, Kalpaj R. Parekh, MBBS, Julia Kleney-Tait, MD, PhD   +3 more
doaj   +1 more source

Cystic fibrosis year in review 2022 [PDF]

, 2023
Remarkable medical advancements have been made for people with cystic fibrosis (CF) in recent years, with an abundance of research continuing to be conducted worldwide.
Kim, Jeeyeon, Lyman, Benjamin, Savant, Adrienne P.   +2 more
core   +2 more sources

Comparison of Single‐Breath and Multi‐Breath Xe‐MRI in the Longitudinal Assessment of Treatment in Children With Cystic Fibrosis

Journal of Magnetic Resonance Imaging, EarlyView.
ABSTRACT Background Elexacaftor/tezacaftor/ivacaftor (ETI) is a current standard therapy for pediatric cystic fibrosis (CF). Multiple‐breath washout 129Xe MRI (MBW Xe‐MRI) is improved following 1 month of treatment. However, the utility of MBW Xe‐MRI over extended ETI treatment and its comparison to single‐breath Xe‐MRI and pulmonary function tests ...
Faiyza S. Alam, Samal Munidasa, Brandon Zanette, Daniel Li, Sharon Braganza, Felix Ratjen, Giles Santyr   +6 more
wiley   +1 more source

Cystic fibrosis-related diabetes in the era of modern treatment using CFTR modulators in pediatric patients—a systematic review

Frontiers in Pediatrics
BackgroundCystic fibrosis-related diabetes (CFRD) is a common comorbidity in cystic fibrosis (CF), particularly in the pediatric population. As cystic fibrosis transmembrane conductance regulator modulators (CFTRm) become widely used, there is growing ...
Anna Pietrzykowska, Jerzy Darmoń, Katarzyna Szymocha, Wiktoria Donocik, Mateusz Tarasiewicz, Julia Stadnik, Przemysława Jarosz-Chobot   +6 more
doaj   +1 more source

Bioactive Thymosin Alpha-1 Does Not Influence F508del-CFTR Maturation and Activity. [PDF]

, 2019
Deletion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel is the most frequent mutation causing cystic fibrosis (CF). F508del-CFTR is misfolded and prematurely degraded. Recently thymosin a-1 (
Armirotti, Andrea, Braccia, Clarissa, Cholon, Deborah M, Galietta, Luis JV, Gentzsch, Martina, Guidone, Daniela, Hanrahan, John W, Lukacs, Gergely L, Matthes, Elizabeth, Pedemonte, Nicoletta, Phuan, Puay-Wah, Tomati, Valeria, Veit, Guido, Verkman, Alan S   +13 more
core  

Evaluating the Impact of Trikafta on the Quality of Life for Cystic Fibrosis Patients [PDF]

, 2022
Background: Cystic fibrosis (CF) is a severe genetic disorder that primarily impacts the digestive and pulmonary systems. The most frequent Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) mutation is F508del which accounts for 90% of CF ...
Alrwaili, Yzeed
core   +2 more sources

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

JPGN 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, Elisabetta Augustoni, Antonio Pizzol, Anna Opramolla, Cristina Chiadò, Anna Zagaria, Irene Esposito, Giulia Roberto, Alice Ponte, Andrea Evangelista, Paola Quarello, Michele Pinon, Pier Luigi Calvo   +12 more
wiley   +1 more source

Novel reaction to new cystic fibrosis medication Trikafta

Clinical Case Reports, 2021
We present a novel case of an urticaria multiforme‐type drug reaction to the new cystic fibrosis medication Trikafta (elexacaftor + tezacaftor + ivacaftor).
Julian Stashower, Patrick Carr, Virginia Miller, Barrett Zlotoff   +3 more
doaj   +1 more source

Farmacogenética en Fibrosis Quística. [PDF]

, 2017
La fibrosis quística (a partir de ahora referida como FQ) fue descrita por primera vez en 1936 como una entidad separada. También recibía el nombre de “mucoviscidosis” debido a la acumulación de espesas secreciones mucosas que bloqueaban las vías aéreas,
Oraá Pérez, Javier
core  

DEVELOPMENT AND VALIDATION OF STABILITY-INDICATING RP-UPLC METHOD FOR THE SIMULTANEOUS ESTIMATION OF TEZACAFTOR AND IVACAFTOR IN FORMULATIONS [PDF]

, 2020
Objective: Aim of the present research work was to develop a sensitive, rapid and accurate, stability-indicating RP-UPLC method for the simultaneous estimation of tezacaftor and ivacaftor in formulations.
ANJANA, C. H. K. V. L. S. N., MANEKA, S. LAKSHMI, SARAVANAKUMAR, R. T.   +2 more
core   +1 more source