Aug 2026· Pediatric Pulmonology· Vol 61· 0 citations· 11 references
Medicine
TL;DR
Clinical practices regarding LFT monitoring and ETI dose adjustments in relation to hepatotoxicity and LFT abnormalities in PwCF with and without baseline CFLD or elevations in LFTs across adult and pediatric CF Foundation accredited care centers and affiliate programs in the United States are described.
Abstract
Elexacaftor/tezacaftor/ivacaftor (ETI) has significantly improved lung function and the clinical course of people with cystic fibrosis (PwCF) but also can cause elevations in liver function tests (LFTs). While the prescribing information (PI) provides some recommendations for holding ETI or hepatically adjusting doses based on certain criteria, clinicians are often faced with clinical scenarios that do not meet those criteria (e.g. PwCF with CF liver disease [CFLD, now termed CF hepatobiliary involvement (CFHBI)] that do not meet Child‐Pugh Class B or C criteria). The purpose of this survey was to describe clinical practices regarding LFT monitoring and ETI dose adjustments in relation to hepatotoxicity and LFT abnormalities in PwCF with and without baseline CFLD or elevations in LFTs across adult and pediatric CF Foundation accredited care centers and affiliate programs (CFF‐ACCAP) in the United States.
Elexacaftor/Tezacaftor/Ivacaftor (ETI) and other highly effective modulator therapies have transformed outcomes for people with cystic fibrosis (pwCF), yielding significant pulmonary and extrapulmonary benefits. Although lung transplant (LTx) rates have declined dramatically in the post ETI era, a substantial number of...
John C. Kennedy, A. Taddesse, A. Coppolino et al.· Journal of Cystic Fibrosis· 0 citations
Abstract RATIONALE Prior research has demonstrated that people with cystic fibrosis (PwCF) in British Columbia (BC) had worse health outcomes if they traveled >360 min to reach their CF clinic compared to those who traveled shorter distances. The status of geographic disparities in CF has not been assessed since the in...
Carolina Bevanda, B. Quon· Canadian Journal of Respirat...· 0 citations
The clinical response to elexacaftor/tezacaftor/ivacaftor (ETI) in people with cystic fibrosis carrying the rare cystic fibrosis transmembrane conductance regulator (CFTR) variant W1282R has not been well characterized. We retrospectively analyzed 12 people with cystic fibrosis treated with ETI for 3–36 months in addit...
E. Kondratyeva, A. Efremova, Y. Melyanovskaya et al.· International Journal of Mol...· 0 citations
Objective: To describe real-world clinical outcomes associated with elexacaftor/
tezacaftor/ivacaftor (ETI) in people with cystic fibrosis (pwCF) followed at a pediatric
referral center in Brazil. Methods: This was a retrospective analysis nested within a
prospective cohort study conducted between February of 2023 an...
Renata Wrobel Folescu Cohen, Paloma Fernandes Coelho, Leonardo Yllel Rocha et al.· Respiratory Research & C...· 0 citations
HFE-related hemochromatosis (HC) is caused by hepcidin dysregulation and is characterized by excessive iron absorption and accumulation in the liver, heart, and endocrine glands, which leads to complications including arthropathy, cirrhosis, and hepatocellular carcinoma. Standard of care (SoC) typically involves phlebo...
Jeremy Shearman, D. Girelli, Dominik J. Schaer et al.· Clinical Hematology Internat...· 1 citation
BACKGROUND
Improved health among people with cystic fibrosis (PwCF) taking highly effective modulator therapies (HEMTs), particularly elexacaftor/tezacaftor/ivacaftor (ETI), has altered the trajectory toward lung transplant (LTx). PwCF now face greater uncertainty about clinical outcomes and LTx timing.
OBJECTIVES
To...
Melissa J. Basile, D. Young, S. Knapp et al.· Heart & lung : the journal o...· 0 citations
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