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镰状细胞性肝病患者对羟基脲的反应：一例报告

Response to Hydroxyurea in a Patient With Sickle Cell Hepatopathy: A Case Report.

作者信息

Safhi Maha A, Baghdadi Rana M, Al-Marzouki Adel F, Al-Sayes Fatin

机构信息

Medicine, King Abdulaziz University Faculty of Medicine, Jeddah, SAU.

Internal Medicine, King Abdulaziz University Faculty of Medicine, Jeddah, SAU.

出版信息

Cureus. 2021 Dec 23;13(12):e20649. doi: 10.7759/cureus.20649. eCollection 2021 Dec.

DOI:10.7759/cureus.20649
PMID:34976545
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8712197/
Abstract

Sickle cell hepatopathy is an underreported entity lacking clear management guidelines. This case highlights the potential role of hydroxyurea (HU) in improving the hepatic dysfunction seen among patients with sickle cell disease (SCD). We herein present the clinical course of a patient prior to and after the initiation of hydroxyurea with an emphasis on long-term outcomes and the patterns of liver injury over a 15-year time course.

摘要

镰状细胞性肝病是一种报告不足且缺乏明确管理指南的疾病。本病例突出了羟基脲（HU）在改善镰状细胞病（SCD）患者肝功能障碍方面的潜在作用。我们在此介绍一名患者在开始使用羟基脲之前和之后的临床病程，重点关注15年时间跨度内的长期结局和肝损伤模式。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/d6ad8826200a/cureus-0013-00000020649-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/c013533ec0fa/cureus-0013-00000020649-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/cb00d07f715a/cureus-0013-00000020649-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/781bb056a97d/cureus-0013-00000020649-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/d6ad8826200a/cureus-0013-00000020649-i04.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/c013533ec0fa/cureus-0013-00000020649-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/cb00d07f715a/cureus-0013-00000020649-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/781bb056a97d/cureus-0013-00000020649-i03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/2bb8/8712197/d6ad8826200a/cureus-0013-00000020649-i04.jpg

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本文引用的文献

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Sickle Hepatopathy.镰状细胞性肝病
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2
Rare but Lethal Hepatopathy-Sickle Cell Intrahepatic Cholestasis and Management Strategies.罕见但致命的肝病——镰状细胞性肝内胆汁淤积症及管理策略
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How we treat sickle hepatopathy and liver transplantation in adults.成人镰状细胞肝病的治疗方法和肝移植。
Blood. 2014 Apr 10;123(15):2302-7. doi: 10.1182/blood-2013-12-542076. Epub 2014 Feb 24.
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Reversal of hepatic and renal failure from sickle cell intrahepatic cholestasis.镰状细胞性肝内胆汁淤积所致肝肾功能衰竭的逆转
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Hydroxyurea for the treatment of sickle cell anemia.羟基脲用于治疗镰状细胞贫血。
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Hepatic dysfunction in sickle cell disease: a new system of classification based on global assessment.镰状细胞病中的肝功能障碍：基于整体评估的新分类系统
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Sickle cell hepatopathy: clinical presentation, treatment, and outcome in pediatric and adult patients.镰状细胞性肝病：儿童和成人患者的临床表现、治疗及预后
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Resolution of chronic hepatic sequestration in a patient with homozygous sickle cell disease receiving hydroxyurea.接受羟基脲治疗的纯合子镰状细胞病患者慢性肝内瘀血的消退
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Sickle cell hepatopathy.镰状细胞性肝病
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