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威尔逊病与慢性乙型肝炎共存的见解:一项用于改进临床实践的回顾性倾向评分匹配研究。

Insights into the coexistence of Wilsons disease and chronic hepatitis B: A retrospective propensity score matched study for improving clinical practice.

作者信息

Pang Jiahui, Chen Shuru, Zeng Yingfu, Chong Yutian, Gan Weiqiang, Li Xinhua

机构信息

Department of Infectious Diseases, The Third Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong, China.

出版信息

Liver Res. 2025 Jan 2;9(2):169-177. doi: 10.1016/j.livres.2024.12.003. eCollection 2025 Jun.

DOI:10.1016/j.livres.2024.12.003
PMID:40620501
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12226794/
Abstract

BACKGROUND AND AIMS

Early and accurate diagnosis of the coexistence of Wilson's disease (WD) and chronic hepatitis B (CHB) presents a significant challenge for clinicians. The objective of this study was to retrospectively analyse the characteristics of such patients to improve clinical practice and provide a reference for clinical management.

METHODS

From January 2011 to December 2022, 35 patients with concurrent CHB and WD (CHB + WD group) were identified. A total of 127 patients with CHB (CHB group) and 168 patients with WD (WD group) were included in the control group between January 2016 and December 2021. Propensity score matching (PSM) was performed to balance the baseline values between groups. The Kaplan-Meier (K-M) survival analysis and log-rank test were performed to compare the prognoses.

RESULTS

In the cohort of 35 patients with concurrent CHB and WD, 74.3% of patients (26 patients) faced a substantial delay of up to 10 years (range: 0-40 years) in WD diagnosis following their CHB diagnosis. Twenty-three (65.7%) patients had cirrhosis at the time of WD diagnosis, and 26 (74.3%) patients experienced liver failure. The levels of serum copper and uric acid were lower in patients in the CHB + WD group than in those in the CHB group. Patients in the CHB + WD group presented higher alanine transaminase and total bile acid levels compared to those in the WD group. K-M survival analysis indicated that patients with CHB and WD had poorer outcomes than those with CHB alone; however, the outcomes were similar to those of individuals with WD alone. The optimal cut-point of serum ceruloplasmin (CP) in identifying WD in CHB patients was 0.10 g/L before PSM and after PSM.

CONCLUSIONS

The present study emphasizes the importance of clinicians being vigilant for concurrent CHB and WD diagnoses, as delays in WD diagnosis may adversely affect patient outcomes. CHB patients with serum CP below 0.10 g/L are highly recommended to screen for WD.

摘要

背景与目的

对威尔逊病(WD)和慢性乙型肝炎(CHB)共存进行早期准确诊断,对临床医生而言是一项重大挑战。本研究的目的是回顾性分析此类患者的特征,以改进临床实践,并为临床管理提供参考。

方法

2011年1月至2022年12月期间,共确定了35例CHB与WD并存的患者(CHB+WD组)。2016年1月至2021年12月期间,对照组纳入了127例CHB患者(CHB组)和168例WD患者(WD组)。进行倾向评分匹配(PSM)以平衡组间基线值。采用Kaplan-Meier(K-M)生存分析和对数秩检验比较预后。

结果

在35例CHB与WD并存的患者队列中,74.3%的患者(26例)在CHB诊断后WD诊断出现了长达10年(范围:0至40年)的显著延迟。23例(65.7%)患者在WD诊断时已出现肝硬化,26例(74.3%)患者发生了肝衰竭。CHB+WD组患者的血清铜和尿酸水平低于CHB组患者。与WD组相比,CHB+WD组患者的丙氨酸转氨酶和总胆汁酸水平更高。K-M生存分析表明,CHB与WD并存的患者预后比单纯CHB患者差;然而,其预后与单纯WD患者相似。PSM前后,CHB患者中用于识别WD的血清铜蓝蛋白(CP)最佳切点均为0.10g/L。

结论

本研究强调临床医生对CHB与WD并存诊断保持警惕的重要性,因为WD诊断延迟可能对患者预后产生不利影响。强烈建议血清CP低于0.10g/L的CHB患者筛查WD。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18c7/12226794/4b61f7d6ccb0/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18c7/12226794/1801f6a452cb/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18c7/12226794/43b7b46dec75/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18c7/12226794/4b61f7d6ccb0/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18c7/12226794/1801f6a452cb/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18c7/12226794/43b7b46dec75/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/18c7/12226794/4b61f7d6ccb0/gr3.jpg

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本文引用的文献

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A novel nomogram based on routine clinical indicators for screening for Wilson's disease.一种基于常规临床指标用于筛查肝豆状核变性的新型列线图。
Liver Res. 2023 Feb 24;7(1):82-89. doi: 10.1016/j.livres.2023.02.003. eCollection 2023 Mar.
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Noninvasive tests for liver fibrosis in 2024: are there different scales for different diseases?2024年肝纤维化的非侵入性检测:不同疾病有不同的评估标准吗?
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Hepatitis B.乙型肝炎
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Non-invasive diagnosis of cirrhosis and long-term disease monitoring by transient elastography in patients with Wilson disease.应用瞬时弹性成像技术对肝豆状核变性患者进行非侵入性诊断和长期疾病监测。
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