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囊性纤维化相关肝病中个性化CFTR调节剂治疗的启动与监测:少即是多。

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

作者信息

Vekaria Sona, Kavanagh Grace, Mulrennan Siobhain

机构信息

Department of Respiratory Medicine Sir Charles Gairdner Hospital Nedlands Western Australia Australia.

Department of Pharmacy Sir Charles Gairdner Hospital Nedlands Western Australia Australia.

出版信息

Respirol Case Rep. 2025 Mar 24;13(3):e70146. doi: 10.1002/rcr2.70146. eCollection 2025 Mar.

DOI:10.1002/rcr2.70146
PMID:40134928
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11932953/
Abstract

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 suggested treatment interruption or withdrawal depending on the severity of the derangement. Here we describe a patient with non-cirrhotic hepatic steatosis who experienced persistently elevated liver function tests due to modulator therapy but demonstrated a remarkable response to a notably low dose of ETI.

摘要

使用依列卡福妥/替扎卡福妥/依伐卡福妥(ETI)导致肝毒性已有充分记录。对于转氨酶升高但无肝硬化的患者,目前没有推荐的剂量调整或增加监测频率的算法,仅根据肝功能紊乱的严重程度建议中断治疗或停药。在此,我们描述一名患有非肝硬化性肝脂肪变性的患者,该患者因调节剂治疗导致肝功能检查持续升高,但对低剂量的ETI表现出显著反应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0bc6/11932953/2de9ee030c83/RCR2-13-e70146-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0bc6/11932953/2de9ee030c83/RCR2-13-e70146-g002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/0bc6/11932953/2de9ee030c83/RCR2-13-e70146-g002.jpg

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本文引用的文献

1
Drug-induced liver injury associated with elexacaftor/tezacaftor/ivacaftor: A pharmacovigilance analysis of the FDA adverse event reporting system (FAERS).依来曲泊帕治疗免疫性血小板减少症的有效性和安全性:一项随机、双盲、安慰剂对照的 III 期临床试验
J Cyst Fibros. 2024 May;23(3):566-572. doi: 10.1016/j.jcf.2024.01.001. Epub 2024 Jan 16.
2
Longitudinal effects of elexacaftor/tezacaftor/ivacaftor on liver tests at a large single adult cystic fibrosis centre.在一家大型成人单一囊性纤维化中心,依列卡福妥/替扎卡福妥/依伐卡福妥对肝脏检查的纵向影响。
J Cyst Fibros. 2023 Mar;22(2):256-262. doi: 10.1016/j.jcf.2023.01.007. Epub 2023 Jan 19.
3
Role of Ursodeoxycholic Acid in Treating and Preventing Idiosyncratic Drug-Induced Liver Injury. A Systematic Review.
熊去氧胆酸在治疗和预防特异质性药物性肝损伤中的作用。一项系统评价。
Front Pharmacol. 2021 Oct 27;12:744488. doi: 10.3389/fphar.2021.744488. eCollection 2021.
4
Drug-induced liver injury from elexacaftor/ivacaftor/tezacaftor.依列卡福妥/依伐卡托/替扎卡福妥所致药物性肝损伤
J Cyst Fibros. 2022 Mar;21(2):e99-e101. doi: 10.1016/j.jcf.2021.07.001. Epub 2021 Jul 16.