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Hyperbilirubinemia and Gilbert's syndrome in Cystic Fibrosis patients treated with elexacaftor/tezacaftor/ivacaftor.

作者信息

Terlizzi V, Timpano S, Salvi M, Tosco A, Castaldo A, Fevola C, Leonetti G, Vitullo P, Sepe A, Badolato R, Salvatore D

机构信息

Department of Paediatric Medicine, Cystic Fibrosis Regional Reference Center, Meyer Children's Hospital IRCCS, Florence, Italy.

Cystic Fibrosis Regional Support Center, Department of Pediatrics, ASST Spedali Civili Brescia, University of Brescia, Brescia, Italy.

出版信息

J Cyst Fibros. 2023 Nov;22(6):1130-1132. doi: 10.1016/j.jcf.2023.06.013. Epub 2023 Jul 1.

DOI:10.1016/j.jcf.2023.06.013
PMID:37400299
Abstract
摘要

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Hyperbilirubinemia and Gilbert's syndrome in Cystic Fibrosis patients treated with elexacaftor/tezacaftor/ivacaftor.接受依列卡福妥/替扎卡福妥/依伐卡福妥治疗的囊性纤维化患者的高胆红素血症和吉尔伯特综合征
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Regarding the article entitled "Effect of elexacaftor/tezacaftor/ivacaftor on annual rate of lung function decline in people with cystic fibrosis".关于题为《依列卡福妥/替扎卡福妥/依伐卡托对囊性纤维化患者肺功能年下降率的影响》的文章。
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Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: a double-blind, randomised, phase 3 trial.在纯合子 F508del 突变的囊性纤维化患者中,elexacaftor 加 tezacaftor 加 ivacaftor 联合治疗方案的疗效和安全性:一项双盲、随机、3 期临床试验。
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引用本文的文献

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Blood platelet reduction after elexacaftor/tezacaftor/ivacaftor treatment in people with cystic fibrosis may depend on systemic inflammation reduction.在接受依列卡福/替扎卡福/依伐卡福治疗的囊性纤维化患者中,血小板减少可能取决于全身炎症的减轻。
Sci Rep. 2025 Jul 29;15(1):27571. doi: 10.1038/s41598-025-12333-8.
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Hemoglobin Levels in Children Treated for Cystic Fibrosis with CFTR Modulators: A Single Center Retrospective Study.接受CFTR调节剂治疗的囊性纤维化儿童的血红蛋白水平:一项单中心回顾性研究。
J Clin Med. 2025 Jul 9;14(14):4856. doi: 10.3390/jcm14144856.
3
Liver biochemical indexes and cholesterol metabolism in cystic fibrosis patients with F508del/CFTR variant genotype after elexacaftor/tezacaftor/ivacaftor treatment.
囊性纤维化 F508del/CFTR 变异基因型患者接受依伐卡托/泰他卡托/艾氟卡托治疗后的肝生化指标和胆固醇代谢。
Sci Rep. 2024 Jul 29;14(1):17422. doi: 10.1038/s41598-024-68511-7.
4
Cystic fibrosis year in review 2023.囊性纤维化 2023 年年鉴
Pediatr Pulmonol. 2024 Dec;59(12):3106-3116. doi: 10.1002/ppul.27190. Epub 2024 Jul 26.
5
Case Series: Hyperbilirubinemia under elexacaftor/tezacaftor/ivacaftor in the presence of Gilbert's syndrome.病例系列报告:在存在吉尔伯特综合征的情况下,使用 elexacaftor/tezacaftor/ivacaftor 治疗导致高胆红素血症。
BMC Pulm Med. 2024 Jul 1;24(1):307. doi: 10.1186/s12890-024-03114-6.
6
Changes in vitamins and trace elements after initiation of highly effective CFTR modulator therapy in children and adults with cystic fibrosis - a real-life insight.囊性纤维化儿童和成人开始高效CFTR调节剂治疗后维生素和微量元素的变化——基于现实生活的洞察
Mol Cell Pediatr. 2024 May 8;11(1):4. doi: 10.1186/s40348-024-00178-6.
7
One year of treatment with elexacaftor/tezacaftor/ivacaftor in patients with cystic fibrosis homozygous for the F508del mutation causes a significant increase in liver biochemical indexes.在携带F508del突变纯合子的囊性纤维化患者中,使用依列卡福/替扎卡福/艾伐卡福治疗一年会导致肝脏生化指标显著升高。
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