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[Research progresses in ex vivo liver perfusion technology]. [离体肝脏灌注技术的研究进展]。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20250917-00390
Q Y Liu, Q Zhao, X S He

Liver transplantation is the standard therapy for end-stage liver disease, but the long-standing shortage of donor livers has constrained its advancement. The use of standard donor criteria expansion partially alleviates the supply-demand imbalance but increases postoperative complication risks. Extracorporeal mechanical perfusion mitigates ischemia-reperfusion injury, extends preservation time, and enables functional assessment and partial repair of the liver under ex vivo settings. Current clinical evidence confirms that short-term mechanical perfusion positively improves outcomes, but it still has limitations in terms of functional evaluation and deep repair. Therefore, the exploration of prolonged mechanical perfusion has possibilities for the restoration of organ function. The concept of "organ medicine" has enabled the breakthrough application of mechanical perfusion technology, originating from organ transplantation, to multiple disciplines, such as organ research, education, and therapy. Additionally, advancements in transforming research results and industrial upgrading are anticipated to develop into a strategic technology for a new round of medical revolution and industrial transformation.

肝移植是终末期肝病的标准治疗方法,但供体肝脏的长期短缺制约了其发展。使用标准供体标准扩大部分缓解了供需不平衡,但增加了术后并发症的风险。体外机械灌注减轻缺血-再灌注损伤,延长保存时间,并在离体环境下实现肝脏的功能评估和部分修复。目前的临床证据证实,短期机械灌注能积极改善预后,但在功能评估和深度修复方面仍有局限性。因此,延长机械灌注的探索为器官功能的恢复提供了可能。“器官医学”的概念使得源于器官移植的机械灌注技术在器官研究、教育、治疗等多学科的突破性应用。此外,研究成果转化和产业升级的进展有望发展成为新一轮医疗革命和产业转型的战略技术。
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引用次数: 0
[Changes in hepatic phase Ⅱ detoxification enzymes and their mechanism in metabolic associated steatohepatitis (MASH) induced by MCD diet in mice]. [MCD饮食诱导小鼠代谢性脂肪性肝炎(MASH)中肝脏期Ⅱ解毒酶的变化及其机制]。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20240728-00351
J Q Gao, B Zuo, C Q Pi, M Xiao, J X Wang, W J Tao, Y He
<p><p><b>Objective:</b> To investigate the changes in hepatic phase II detoxification enzymes and their mechanism in metabolic associated steatohepatitis (MASH) induced by a methionine-choline-deficient (MCD) diet in mice. <b>Methods:</b> Ten C57BL/6J mice were randomly divided into two groups, with five mice in each group, and fed with a control diet (NCD group) and a methionine-choline-deficient diet (MCD group) for four consecutive weeks to establish the MASH model in mice. Mice body weight was recorded weekly. Mice peripheral blood and liver tissue samples were collected after four weeks. The liver histopathological changes were observed by hematoxylin-eosin staining and Sirius red staining in liver tissue. The levels of plasma alanine aminotransferase (ALT), aspartate aminotransferase (AST) and triglycerides were measured by an automatic biochemical analyzer. Triglyceride and total cholesterol were used to evaluate the lipid accumulation condition in the liver of mice with Oil red O staining. Real-time fluorescence quantitative PCR was used to detect the expression of liver inflammatory factors interleukin (IL)-1β and monocyte chemoattractant protein-1 (MCP-1) condition. Transcriptome sequencing and bioinformatics were used to analyze the changes in gene expression profiles in the liver of mice and screen differentially expressed genes. The expression conditions of phase Ⅱ detoxification enzymes glutathione S-transferase mu 4 (GSTM4), dihydronicotinamide riboside:quinone oxidoreductases (NQO-2), sulfotransferase 1β1 (SULT1β1), and uridine diphosphate glucuronosyltransferase 2 family, polypeptide A3(UGT2A3) were verified by real-time fluorescent quantitative PCR. Plasma malondialdehyde content, total antioxidant capacity (T-AOC), plasma and liver glutathione content were determined using commercial kits. The expression of nuclear factor E2-related factor 2 (Nrf2), GSTM4, and UGT1A6 was examined by Western blotting. The independent sample <i>t</i>-test was used for comparison between the groups. <b>Results:</b> The body weight of mice in the MCD group showed a gradual downward trend, while the body weight of mice in the NCD group did not change significantly following four weeks of different dietary feeding. The MCD group mice liver had yellow-white appearance with round edges. The liver/body mass index was significantly lower in the NCD group (<i>t</i>=3.216, <i>P</i><0.01). Hematoxylin-eosin staining showed that hepatocytes in the MCD group had an occurrence of fatty degeneration accompanied by inflammatory cell infiltration, with a higher NAFLD activity score (NAS) compared to the NCD group (<i>t</i>=7.155, <i>P</i><0.001). Sirius red staining showed that the the liver of the MCD group had mildly increased periportal fibers. Plasma biochemical tests indicated that plasma ALT, AST, and triglyceride levels were significantly higher in the MCD group than those in the NCD group (<i>t</i>=8.920, <i>P</i><0.001; <i>t</i>=6.696, <i>P</i><0.001; <i
目的:探讨蛋氨酸胆碱缺乏(MCD)诱导小鼠代谢性脂肪性肝炎(MASH)时肝脏II期解毒酶的变化及其机制。方法:将10只C57BL/6J小鼠随机分为2组,每组5只,连续4周饲喂对照饲粮(NCD组)和蛋氨酸-胆碱缺乏饲粮(MCD组)建立小鼠MASH模型。每周记录小鼠体重。4周后采集小鼠外周血和肝组织样本。采用苏木精-伊红染色和天狼星红染色观察肝组织病理变化。采用全自动生化分析仪测定血浆丙氨酸转氨酶(ALT)、天冬氨酸转氨酶(AST)和甘油三酯水平。采用油红O染色法,用甘油三酯和总胆固醇评价小鼠肝脏脂质积累情况。采用实时荧光定量PCR检测肝脏炎症因子白细胞介素(IL)-1β和单核细胞趋化蛋白-1 (MCP-1)的表达情况。利用转录组测序和生物信息学分析小鼠肝脏中基因表达谱的变化,筛选差异表达基因。采用实时荧光定量PCR验证Ⅱ期解毒酶谷胱甘肽s -转移酶4 (GSTM4)、二氢烟酰胺核苷:醌氧化还原酶(NQO-2)、硫代转移酶1β1 (SULT1β1)、尿苷二磷酸葡萄糖醛基转移酶2家族多肽A3(UGT2A3)的表达情况。采用商品化试剂盒测定血浆丙二醛含量、总抗氧化能力(T-AOC)、血浆和肝脏谷胱甘肽含量。Western blotting检测核因子e2相关因子2 (Nrf2)、GSTM4、UGT1A6的表达。组间比较采用独立样本t检验。结果:MCD组小鼠的体重呈逐渐下降趋势,而NCD组小鼠的体重在四周的不同膳食喂养后没有明显变化。MCD组小鼠肝脏呈黄白色,边缘圆润。NCD组肝/体质量指数明显低于对照组(t=3.216, Pt=7.155, Pt=8.920, Pt=6.696, Pt=3.904, Pt=7.405, Pt=3.559, Pt=2.562, 2.391, Pt=2.498, 3.570, 3.768, 4.166, Pt=3.601, Pt=11.93, Pt=3.635, Pt=2.872, Pt=3.175, 3.064, Pt=3.385, 2.990, 2.168, p)。MCD饮食诱导MASH小鼠肝脏多相Ⅱ解毒酶表达降低,抗氧化能力下降,其机制与下调上游调节因子Nrf2蛋白表达有关。
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引用次数: 0
[Advances in liver transplantation technology]. 【肝移植技术进展】。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20250919-00394
Z J Zhu

Liver transplantation techniques have made significant advances in recent years in surgical procedures, expanding liver donor sources and perioperative management, leading to markedly improved patient survival rates. This review focuses on key recent developments in the field of liver transplantation, summarizing practical achievements and technical breakthroughs in areas such as machine perfusion techniques and utilization of marginally viable donors, minimally invasive liver transplantation techniques, and the clinical application of small-and ultra-small-size grafts. Furthermore, it explores the current challenges encountered in clinical practice and analyzes future research priorities and developmental trends.

近年来,肝移植技术在外科手术、扩大肝供体来源和围手术期管理方面取得了重大进展,显著提高了患者的生存率。本文综述了近年来肝移植领域的重要进展,总结了机器灌注技术和边际存活供体的利用、微创肝移植技术、小尺寸和超小尺寸肝移植的临床应用等方面的实践成果和技术突破。此外,它探讨了目前在临床实践中遇到的挑战,并分析了未来的研究重点和发展趋势。
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引用次数: 0
[Study on the role and mechanism of quercetin promoting autophagy in HCCLM3 cells via STING signaling]. [槲皮素通过STING信号通路促进HCCLM3细胞自噬作用及机制研究]。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20240411-00197
L Chen, G Z Wu, Y Zhu, T T Zhang

Objective: To explore the effects of quercetin on autophagy and proliferation in HBV-positive liver cancer HCCLM3 cells based on STING signaling and its underlying mechanism. Methods: HCCLM3 cells were treated with quercetin (50 μmol/L or 100 μmol/L), designated as the 50 μmol/L group and 100 μmol/L group, respectively. The inhibitory effect of quercetin on HCCLM3 cells was detected using the CCK-8 method. A scratch assay was conducted to assess the impact of quercetin on the migration ability of HCCLM3 cells. A CCK8 and ROS kit was used to detect the effect of quercetin on the levels of reactive oxygen species in HCCLM3 cells. Western blotting was employed to measure the effect of quercetin on the expression of STING signaling and autophagy-related proteins in HCCLM3 cells. RNA interference technology was used to assess the effects of STING signaling inhibition on the expression of autophagy-related proteins and reactive oxygen species levels in HCCLM3 cells. The combined effects of STING activators and quercetin on HCCLM3 cell proliferation and autophagy were evaluated. The t-test was used to detect data differences between two groups, while ANOVA was employed for comparisons among multiple groups, followed by the SNK-q test for further pairwise comparisons. Results: Compared with the control group, quercetin (50 μmol/L and 100 μmol/L groups) significantly inhibited HCCLM3 cell survival activity in a dose-dependent manner (control group: 100%; 50 μmol/L group: 75.25%; 100 μmol/L group: 50.36%, P<0.01 ). Quercetin inhibited HCCLM3 cell migration in a dose-dependent manner (>2 h, control group: 187.16 μm; 50 μmol/L group: 145.22 μm; 100 μmol/L group: 88.21 μm, P<0.01), which significantly increased intracellular reactive oxygen species (ROS) levels in HCCLM3 cells (control group: 1.00; 50 μmol/L group: 1.565; 100 μmol/L group: 2.175, P<0.01). The phosphorylation level of STING was significantly increased (P<0.01), and the expression of autophagy-related protein microtubule-related protein 1A/1B light chain 3 (LC3) protein was significantly promoted (P<0.01). Compared with the quercetin group, the cell viability of the small interfering-STING+quercetin group was increased (quercetin group: 56.3%; small interfering-STING+quercetin group: 85.7%, P<0.05), while the expression of autophagy-related protein LC3 was decreased. Compared with the quercetin group, the cell viability of the quercetin+STING activator group was further decreased (quercetin group: 56.7%; quercetin+STING activator group: 35.4%, P<0.01), and the expression levels of STING and autophagy protein LC3 were significantly increased (P<0.05). Conclusions: STING signaling-regulated cell autophagy mediates the inhibitory effect of quercetin on the proliferation of HCCLM3 cells, and this effect is enhanced after administration of the STING agonist.

目的:探讨槲皮素基于STING信号通路对hbv阳性肝癌HCCLM3细胞自噬和增殖的影响及其机制。方法:用槲皮素(50 μmol/L或100 μmol/L)处理HCCLM3细胞,分别作为50 μmol/L组和100 μmol/L组。采用CCK-8法检测槲皮素对HCCLM3细胞的抑制作用。采用划痕法评估槲皮素对HCCLM3细胞迁移能力的影响。采用CCK8和ROS试剂盒检测槲皮素对HCCLM3细胞活性氧水平的影响。Western blotting检测槲皮素对HCCLM3细胞STING信号和自噬相关蛋白表达的影响。采用RNA干扰技术评估STING信号抑制对HCCLM3细胞自噬相关蛋白表达及活性氧水平的影响。观察STING激活剂联合槲皮素对HCCLM3细胞增殖和自噬的影响。两组间比较采用t检验,多组间比较采用方差分析,进一步两两比较采用SNK-q检验。结果:与对照组相比,槲皮素(50μmol / L和100μmol / L组)显著地抑制HCCLM3细胞生存活动方式存在剂量依赖的相关性(对照组:100%;50μmol / L组:75.25%;100μmol / L组:50.36%,P2 h,对照组:187.16μm; 50μmol / L组:145.22μm;结论:STING信号调节的细胞自噬介导槲皮素对HCCLM3细胞增殖的抑制作用,且STING激动剂对HCCLM3细胞的抑制作用增强。
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引用次数: 0
[Advances in minimally invasive surgical techniques for liver transplant recipients]. [微创肝移植手术技术进展]。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20250916-00387
Z Feng, X F Zhang, Y Lyu

In recent years, minimally invasive surgical techniques, especially laparoscopic and robot-assisted surgery, have fundamentally changed the standard procedure for living donor liver retrieval and are gradually expanding into the core area of recipient surgery. This review systematically examines the latest advancements in the application of minimally invasive techniques in surgery for liver transplant recipients, focusing on the current status of the integrated application of innovative technologies such as laparoscopy, robot-assisted surgery, and magnetic anastomosis. In addition, this article delves into the potential and evidence-based basis for improving postoperative recovery and reducing surgical complications, and analyze the technical complexity, ethical considerations, equipment dependence, and training challenges faced in achieving full minimally invasiveness in key stages of donor liver implantation. Lastly, the future development directions are discussed, emphasizing that innovation in technological instruments, establishment of standardized training systems, deep multidisciplinary integration, and assemblage with emerging technologies are key pathways to safely and efficiently advance this field, ultimately aiming to provide optimized surgical treatment options for more patients with end-stage liver disease.

近年来,微创手术技术,特别是腹腔镜和机器人辅助手术,从根本上改变了活体供肝回收的标准程序,并逐渐扩展到受体手术的核心领域。本文系统综述了微创技术在肝移植受者手术中的最新应用进展,重点介绍了腹腔镜、机器人辅助手术、磁吻合等创新技术的综合应用现状。此外,本文还深入探讨了提高术后恢复和减少手术并发症的潜力和循证基础,并分析了在供肝植入关键阶段实现完全微创所面临的技术复杂性、伦理考虑、设备依赖性和培训挑战。最后,讨论了未来的发展方向,强调创新技术手段、建立规范化培训体系、多学科深度融合、与新兴技术结合是安全、高效推进该领域的关键途径,最终为更多终末期肝病患者提供优化的手术治疗选择。
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引用次数: 0
[The logic of survival in patients with acute liver failure]. 【急性肝功能衰竭患者的生存逻辑】。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20250711-00274
H L Weng

Massive hepatic necrosis (MHN) is defined as hepatocyte death exceeding 70% in the entire liver. Acute liver failure induced by MHN can lead to death in nearly half of patients. However, the vast majority of lost hepatocyte conditions can recover in about half of these patients. Hepatocyte function restoration and gradual differentiation into mature hepatocytes primarily rely on the rapid activation and proliferation of liver progenitor cells (LPCs) in these survived patients. This paper mainly describes the cellular and molecular mechanisms by which LPCs restore liver function and rescue patients following the occurrence of MHN.

大面积肝坏死(MHN)是指整个肝脏中肝细胞死亡超过70%。由MHN引起的急性肝衰竭可导致近一半患者死亡。然而,绝大多数失去肝细胞的情况可以在大约一半的患者中恢复。在这些存活的患者中,肝细胞功能的恢复和逐渐分化为成熟的肝细胞主要依赖于肝祖细胞(LPCs)的快速激活和增殖。本文主要阐述LPCs在MHN发生后恢复肝功能和抢救患者的细胞和分子机制。
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引用次数: 0
[Clinical outcomes of small-size grafts in auxiliary liver transplantation for the treatment of portal hypertension]. [辅助肝移植治疗门静脉高压症小尺寸移植物的临床疗效]。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20250919-00396
H F Ju, L Wei, L Y Sun, W Qu, Z G Zeng, H M Zhang, Y L Tan, J Wang, F X Xie, Z J Zhu

Objective: To evaluate the safety and efficacy of using small and ultra-small sized grafts for in situ auxiliary liver transplantation in the treatment of portal hypertension. Methods: A prospective single-arm cohort study was conducted. Patients who underwent liver transplantation at Beijing Friendship Hospital from December 2014 to July 2025 were included. Intraoperative portal vein pressure was routinely monitored, with the target regulation for portal vein blood flow set at<15 mmHg (1 mmHg=0.133 kPa) and follow-up continued until September 2025. The primary endpoints were the patient's status and graft survival. The secondary endpoints were small-for-size syndrome and perioperative complications. The small-for-size syndrome was graded according to the 2023 International Liver Transplantation Society consensus statement. Results: A total of 33 cases were enrolled. Among them, 22 had ultra-small size grafts, 11 had small-size grafts, 28 had living donor grafts, and five had split grafts. The graft-to-recipient weight ratio in living donor liver transplantation was 0.31%~0.79%, while in split liver transplantation it was 0.45%~1.02%. Intraoperative portal vein pressure of ≥15 mmHg was observed in 11 cases, who underwent portal vein blood flow adjustment via splenic artery ligation (2 cases), partial splenectomy (8 cases), and/or restrictive portocaval shunting (1 case), after which all patients achieved the target portal vein pressure. All cases completed at least one month of follow-up, with 28 cases following for more than one year, and the median follow-up period was 36.5 months. Early-stage postoperative small-for-size syndrome occurred in eight cases (24.2%, 8/33), all classified as grade A, with improvements following supportive treatment. Severe complications (Clavien-Dindo≥Ⅲ) occurred in three cases (9.1%, 3/33). The one-year survival rate was 92.9% (26/28). The overall survival rate at the end of follow-up was 90.9% (30/33). No patients experienced graft loss or death due to small-for-size syndrome. Graft tissue tested negative for hepatitis B core antibody and covalently closed circular DNA, and hepatitis B surface antigen seroconversion was achieved following second-stage residual liver resection and under a combined strategy of potent nucleos(t)ide analogs and hepatitis B immunoglobulin in ten cases of hepatitis B-related disease. Conclusions: With standardized portal vein blood flow monitoring and individualized portal vein blood flow adjustment, in situ auxiliary liver transplantation can safely and effectively use small and even ultra-small sized grafts, thereby significantly expanding graft sources and ensuring donor and recipient safety. These findings warrant further validation and promotion in multicenter controlled studies.

目的:评价小尺寸和超小尺寸肝原位辅助移植治疗门静脉高压症的安全性和有效性。方法:采用前瞻性单臂队列研究。纳入2014年12月至2025年7月在北京友谊医院行肝移植手术的患者。术中常规监测门静脉压力,设定门静脉血流调节目标。结果:共入组33例。其中超小型移植物22例,小型移植物11例,活体供体移植物28例,分裂移植物5例。活体肝移植的移植物重量比为0.31%~0.79%,裂体肝移植的移植物重量比为0.45%~1.02%。术中门静脉压力≥15mmhg 11例,分别行脾动脉结扎术(2例)、脾部分切除术(8例)、限制性门静脉分流术(1例)调整门静脉血流,术后门静脉压力均达到目标。所有病例均完成了至少1个月的随访,其中28例随访1年以上,中位随访时间为36.5个月。术后早期小尺寸综合征8例(24.2%,8/33),均为A级,经支持治疗后有所改善。严重并发症(Clavien-Dindo≥Ⅲ)3例(9.1%,3/33)。1年生存率为92.9%(26/28)。随访结束时总生存率为90.9%(30/33)。没有患者因小尺寸综合征发生移植物丢失或死亡。移植组织的乙肝核心抗体和共价闭合环状DNA检测呈阴性,在10例乙肝相关疾病中,在第二阶段残肝切除和强效核苷类似物和乙肝免疫球蛋白联合策略下,乙肝表面抗原血清转化实现。结论:通过标准化门静脉血流监测和个体化门静脉血流调节,原位辅助肝移植可以安全有效地使用小尺寸甚至超小尺寸的移植物,从而显著扩大移植物来源,保证供受体安全。这些发现值得在多中心对照研究中进一步验证和推广。
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引用次数: 0
[A review of research progress in integrated traditional Chinese and Western Medicine for liver diseases]. [中西医结合治疗肝病研究进展综述]。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20250116-00028
Q Zhang, B Tian, C Shan, Z H Cao, C J Xu, Z J Hu, X L Qi

Liver disease is a major global health issue, severely impacting patients' quality of life and life expectancy. Integrated Traditional Chinese and Western Medicine demonstrates unique advantages in the field of liver disease treatment. Therefore, this article elaborates on the research progress of integrated traditional Chinese and Western medicine in viral hepatitis, metabolic dysfunction-associated steatotic liver disease, cirrhosis, and liver cancer.

肝病是一个重大的全球健康问题,严重影响患者的生活质量和预期寿命。中西医结合在肝病治疗领域具有独特优势。为此,本文就中西医结合治疗病毒性肝炎、代谢功能障碍相关性脂肪变性肝病、肝硬化、肝癌的研究进展作一综述。
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引用次数: 0
[Current research progress status in identifying hepatocellular ballooning degeneration in nonalcoholic steatohepatitis]. [鉴别非酒精性脂肪性肝炎肝细胞球囊变性的研究进展现状]。
Q3 Medicine Pub Date : 2025-11-20 DOI: 10.3760/cma.j.cn501113-20240604-00284
H R Xie, L Wei

Nonalcoholic steatohepatitis (NASH) is a chronic liver disease associated with metabolic syndrome, such as obesity, diabetes, and hyperlipidemia, and is a progressive form of nonalcoholic fatty liver disease with pathological features including steatosis, lobular inflammation, and hepatocyte damage (ballooning degeneration). Hepatocellular ballooning degeneration is an important pathological feature of NASH and is closely related to the prognosis of NASH patients. Accurate identification of hepatocellular ballooning degeneration is of great significance for diagnosing NASH and assessing therapeutic response. This article reviews the current status and identification methods of hepatocellular ballooning degeneration in NASH, including invasive and noninvasive.

非酒精性脂肪性肝炎(NASH)是一种与代谢综合征(如肥胖、糖尿病和高脂血症)相关的慢性肝脏疾病,是一种进行性非酒精性脂肪性肝病,其病理特征包括脂肪变性、小叶炎症和肝细胞损伤(球囊变性)。肝细胞球囊变性是NASH的重要病理特征,与NASH患者的预后密切相关。准确识别肝细胞球囊变性对NASH的诊断和评价治疗效果具有重要意义。本文综述了NASH肝细胞球囊变性的现状及鉴别方法,包括侵袭性和非侵袭性。
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引用次数: 0
[Recommendations from the European Association for the Study of the Liver and the European Reference Network for Rare Liver Diseases Clinical Practice Guidelines for hepatolenticular degeneration]. [来自欧洲肝脏研究协会和欧洲罕见肝病参考网络关于肝豆状核变性临床实践指南的建议]。
Q3 Medicine Pub Date : 2025-10-20 DOI: 10.3760/cma.j.cn501113-20250408-00130
S Tang, W Hou, S J Zheng

Wilson disease (WD), also known as hepatolenticular degeneration, is an autosomal recessive inherited disorder of copper metabolism that primarily affects the liver, brain, and other organs. The diagnostic criteria include clinical features, biochemical tests (plasma ceruloplasmin, 24-hour urinary copper, liver copper content), and molecular genetic analysis. The Leipzig scoring system, supplemented by the use of exchangeable copper, is recommended for diagnosis. Pharmacotherapy mainly includes chelating agents (such as penicillamine and trientine) and zinc salts. Chelating agent therapy is recommended only for patients with severe liver disease. Patient monitoring is primarily based on clinical symptoms, liver biochemical indices, and copper metabolism parameters (such as 24-hour urinary copper and exchangeable copper) to identify poor adherence as well as over-treatment or under-treatment situations. The diagnosis and treatment of acute liver failure with WD is extremely challenging, as the diagnosis is difficult and medical treatment cannot save life. The role of liver transplantation has been clearly recognized in the treatment of acute liver failure with WD, and it may also be considered in cases with neurological involvement.

肝豆状核变性(WD),也称为肝豆状核变性,是一种铜代谢常染色体隐性遗传疾病,主要影响肝脏、脑和其他器官。诊断标准包括临床表现、生化检查(血浆铜蓝蛋白、24小时尿铜、肝铜含量)和分子遗传学分析。莱比锡评分系统,辅以使用可交换的铜,被推荐用于诊断。药物治疗主要包括螯合剂(如青霉胺和曲恩汀)和锌盐。螯合剂治疗仅推荐用于严重肝病患者。患者监测主要根据临床症状、肝脏生化指标、铜代谢参数(如24小时尿铜、可交换铜)来识别依从性差、治疗过度或治疗不足的情况。急性肝功能衰竭合并WD的诊断和治疗极具挑战性,因为诊断困难,药物治疗不能挽救生命。肝移植在治疗伴有WD的急性肝衰竭中的作用已经得到了明确的认识,在神经系统受累的情况下也可以考虑肝移植。
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引用次数: 0
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中华肝脏病杂志
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