Liver biopsy

肝活检
  • 文章类型: Case Reports
    背景:淀粉样变性是一种罕见的疾病,可以分为各种类型,最常见的类型是系统轻链类型。这种疾病的预后极差。总的来说,淀粉样变主要影响肾脏和心脏,表现为克隆浆细胞的异常增殖。肝脏是淀粉样变性影响的主要器官的情况,在这份报告中,在临床实践中并不常见。
    方法:一名62岁男性因不明原因的持续性肝功能障碍和不良治疗结果入院。他的病情持续,他出现了慢性肝衰竭,晚期伴有严重胆汁淤积,逐渐伴有肾损伤。最终,通过肝活检和病理检查诊断为肝淀粉样变性。
    结论:临床上很少发生肝淀粉样变性,肝活检和病理检查可以帮助准确有效地诊断这种情况。
    BACKGROUND: Amyloidosis is a rare disorder that can be classified into various types, and the most common type is the systemic light chain type. The prognosis of this disease is extremely poor. In general, amyloidosis mainly affects the kidneys and heart and manifests as abnormal proliferation of clonal plasma cells. Cases in which the liver is the primary organ affected by amyloidosis, as in this report, are less common in clinical practice.
    METHODS: A 62-year-old man was admitted with persistent liver dysfunction of unknown cause and poor treatment outcomes. His condition persisted, and he developed chronic liver failure, with severe cholestasis in the later stage that was gradually accompanied by renal injury. Ultimately, he was diagnosed with hepatic amyloidosis through liver biopsy and pathological examination.
    CONCLUSIONS: Hepatic amyloidosis rarely occurs in the clinic, and liver biopsy and pathological examination can assist in the accurate and effective diagnosis of this condition.
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  • 文章类型: Journal Article
    Liver biopsy is an important means of clinical diagnosis and treatment of liver diseases, but it is not easily accepted by patients because of its invasiveness. The most commonly employed liver biopsy approaches are percutaneous or transjugular. Endoscopic ultrasound-guided liver biopsy (EUS-LB), a newly emerging transjugular technique, has been widely studied and applied in recent years, but its application in China is less common. The EUS-LB has the advantages of high safety and comfort, simultaneous sampling of both liver lobes, and adequate sampling volume; however, it also has the disadvantages of high requirements for hardware, operators, and cost. This article reviews the clinical application of EUS-LB in accordance with pertinent research findings from recent years and discusses its advantages, disadvantages, and implementation feasibility.
    肝活检是肝病临床诊疗的重要手段,因其有创性不易为患者接受。常用的肝活检主要有经皮或经颈静脉肝脏穿刺途径。超声内镜下肝脏穿刺活检(EUS-LB)作为新出现的肝脏穿刺技术,近年来得到较多的研究和应用,然而在国内开展较少。EUS-LB具有较高的安全性和舒适性、可同时肝脏双叶取样、有足够的取样量等优点,但也存在对硬件和操作者要求高、成本高等不足。现根据近年来的相关研究结果对EUS-LB在临床中的应用进行综述,探讨其优缺点及开展的可行性。.
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  • 文章类型: Journal Article
    背景:以前没有对不确定期慢性乙型肝炎(CHB)患者的肝脏组织学变化进行大样本研究。
    目的:使用肝活检评估不确定期CHB患者的肝组织学变化。
    方法:收集1532例未治疗CHB患者的临床和实验室数据,从2015年1月至2021年12月,所有患者至少接受了一次肝活检.用t检验比较CHB感染的不同阶段之间的显著差异,采用多因素logistic回归分析肝脏组织学改变的危险因素。
    结果:在1532名未经治疗的CHB患者中,814例(53.13%)患者处于不确定阶段。在不确定阶段的488/814(59.95%)CHB患者中发现了显着的肝组织学变化(定义为活检评分≥G2和/或≥S2)。肝脏组织学改变在不同年龄之间有显著差异,血小板(PLT),不确定患者的丙氨酸氨基转移酶(ALT)亚组。多因素logistic回归分析提示年龄≥40岁[调整奇数风险(aOR),1.44;95%置信区间(CI):1.06-1.97;P=0.02],PLTs≤150×109/L(aOR,2.99;95CI:1.85-4.83;P<0.0001),和ALT≥正常上限(AOR,1.48;95CI:1.08,2.05,P=0.0163)是CHB患者在不确定阶段显着肝脏组织学变化的独立危险因素。
    结论:我们的结果表明,在不确定阶段,未经治疗的CHB患者中,显著的肝组织学变化并不罕见,这些患者的管理迫切需要额外的策略。
    BACKGROUND: Studies with large size samples on the liver histological changes of indeterminate phase chronic hepatitis B (CHB) patients were not previously conducted.
    OBJECTIVE: To assess the liver histological changes in the indeterminate phase CHB patients using liver biopsy.
    METHODS: The clinical and laboratory data of 1532 untreated CHB patients were collected, and all patients had least once liver biopsy from January 2015 to December 2021. The significant differences among different phases of CHB infection were compared with t-test, and the risk factors of significant liver histological changes were analyzed by the multivariate logistic regression analysis.
    RESULTS: Among 1532 untreated CHB patients, 814 (53.13%) patients were in the indeterminate phase. Significant liver histological changes (defined as biopsy score ≥ G2 and/or ≥ S2) were found in 488/814 (59.95%) CHB patients in the indeterminate phase. Significant liver histological changes were significant differences among different age, platelets (PLTs), and alanine aminotransferase (ALT) subgroup in indeterminate patient. Multivariate logistic regression analysis indicated that age ≥ 40 years old [adjust odd risk (aOR), 1.44; 95% confidence interval (CI): 1.06-1.97; P = 0.02], PLTs ≤ 150 × 109/L (aOR, 2.99; 95%CI: 1.85-4.83; P < 0.0001), and ALT ≥ upper limits of normal (aOR, 1.48; 95%CI: 1.08, 2.05, P = 0.0163) were independent risk factors for significant liver histological changes in CHB patients in the indeterminate phase.
    CONCLUSIONS: Our results suggested that significant liver histological changes were not rare among the untreated CHB patients in indeterminate phase, and additional strategies are urgently required for the management of these patients.
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  • 文章类型: Journal Article
    世界范围内几种肝脏疾病的发病率急剧增加;因此,对这些病理实体进行诊断和分期的未满足需求预示着肝活检(LB)技术的广泛应用。LB的方式是通用的,包括经皮LB,经颈静脉LB,最近一种最小侵入的方法,也就是说,EUS引导的LB(EUS-LB)。在这篇评论文章中,我们得出的结论是,EUS-LB可能是可行的,可靠,在提高诊断率方面,经皮LB和经颈静脉LB的安全替代方案,优异的采样性能,局灶性患者的不良事件得到控制,渗透,和实质性肝病。此外,已经做出了广泛的努力来优化和完善EUS-LB模式中的几个技术支柱,例如针尺寸/类型的选择,活检针的启动方式,以及通过/致动技术的选择,所有这些都旨在获得更好的标本数量和质量。与EUS引导模式相关的另一个有利方面和独特性质表明,多重筛查,监视,和干预程序可以合并为一个单一的内窥镜会话。因此,一些初步研究通过将EUS-LB与同时测量门静脉压力梯度或检查肝脏硬度相结合,阐明了临床应用价值.然而,更多研究,特别是,随机对照试验或现实世界的证据,实际上有必要阐明EUS-LB作为管理肝病的常规/常规部分的有效性和安全性。
    There is dramatically increased incidence of several liver diseases worldwide; thus, an unmet need to diagnose and stage these pathological entities heralds the wide application of liver biopsy (LB) techniques. The ways of LB are versatile, including percutaneous LB, transjugular LB, and more recently an approach of minimal invasiveness, that is, EUS-guided LB (EUS-LB). In this review article, we come to the conclusion that EUS-LB may serve as a feasible, reliable, and safe alternative to percutaneous LB and transjugular LB in terms of improved diagnostic yield, excellent sampling performance, and controlled adverse events among patients with focal, infiltrative, and parenchymal liver diseases. Furthermore, extensive efforts have been made to optimize and refine several technical pillars within EUS-LB modality such as the selection of needle size/type, priming manner of biopsy needle, and choice of pass/actuation technique, all of which aim at obtaining better specimen quantity and quality. Another advantageous aspect and unique property pertinent to EUS-guided modality indicate that multiple screening, surveillance, and intervention procedures can be combined into one single endoscopic session. Accordingly, some pilot studies have clarified the clinical usefulness by integrating EUS-LB with simultaneous measurement of portal pressure gradient or examination of liver stiffness. However, more studies, in particular, randomized controlled trials or real-world evidence, are practically warranted to elucidate the validity and safety of EUS-LB as a regular/routine part of managing liver diseases.
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  • 文章类型: Journal Article
    背景:这项研究旨在评估非侵入性血清生物标志物的诊断能力,以预测肝纤维化分期和评估乙型肝炎的进展
    方法:我们招募了433例慢性HBV感染患者,有完整的医学数据可用于研究,谁接受了经皮肝活检。使用改良的METAVIR评分评估纤维化程度。通过具有95%置信区间的接受操作者特征曲线(AUROC)下的面积来评估非侵入性血清生物标志物的预测值。
    结果:肝纤维化进展期的男性比例相对较大,肝硬化分期患者的平均年龄比非肝硬化分期患者大。我们找到了PLT,GGT,ALP,TB,在我们的队列中,FIB4和GPR与肝纤维化显着相关。GGT显示在区分肝硬化(F4)与非肝硬化阶段(F1-3)的敏感性为71.4%和特异性为76.7%,AUROC为0.775(95CI0.711-0.840)。GPR区分肝硬化(F4)与非肝硬化阶段(F1-3)的AUROC为0.794(95CI0.734-0.853),但灵敏度较低,为59.2%。此外,GGT,FIB4和GPR可以区分慢性乙型肝炎患者的晚期纤维化(F3-4)与非晚期纤维化(F1-2),AUROC为0.723(95CI0.668-0.777),0.729(95CI0.675-0.782),和0.760(95CI:0.709-0.811)。
    结论:GGT是区分肝硬化(F4)和非肝硬化阶段(F1-3)的更好的生物标志物,而GPR是识别慢性乙型肝炎患者的晚期纤维化(F3-4)和非晚期纤维化(F1-2)的更好的生物标志物。
    BACKGROUND: This study aimed to evaluate the diagnostic abilities of the non-invasive serum biomarkers to predict liver fibrosis staging and evaluate the progress of hepatitis B.
    METHODS: We enrolled 433 patients with chronic HBV infection had complete medical data available for the study, who underwent percutaneous liver biopsy. The extent of fibrosis was assessed using the modified METAVIR score. The predictive values of the non-invasive serum biomarkers were evaluated by the areas under the receiving operator characteristics curves (AUROCs) with 95% confidence intervals.
    RESULTS: The proportion of males with progressive stages of liver fibrosis was relatively larger, and the average age of patients with cirrhosis stages is older than the non-cirrhotic stages. We found PLT, GGT, ALP, TB, FIB4 and GPR to be significantly associated with liver fibrosis in our cohort. GGT showed a sensitivity of 71.4% and specificity of 76.7% in distinguishing cirrhosis (F4) from non-cirrhotic stages (F1-3), with an AUROC of 0.775 (95%CI 0.711-0.840).The AUROCs of the GPR in distinguishing cirrhosis (F4) from non-cirrhotic stages (F1-3) was 0.794 (95%CI 0.734-0.853), but it had a lower sensitivity of 59.2%. Additionally, GGT, FIB4, and GPR could differentiate advanced fibrosis (F3-4) from non-advanced fibrosis (F1-2) among individuals with chronic hepatitis B, with AUROCs of 0.723 (95%CI 0.668-0.777), 0.729 (95%CI 0.675-0.782), and 0.760 (95%CI: 0.709-0.811) respectively.
    CONCLUSIONS: GGT was a better biomarker to distinguish cirrhosis (F4) from non-cirrhotic stages (F1-3), while GPR was a better biomarker to identify advanced fibrosis (F3-4) and non-advanced fibrosis (F1-2) in patients with chronic hepatitis B.
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  • 文章类型: Journal Article
    分析原因不明的肝功能异常的临床表现,并对患者进行肝胆组织病理学检查,以评估肝活检在诊断原因不明的肝功能异常病因中的价值。
    采用方便抽样法,回顾性收集天津市第二人民医院病理科诊断为不明原因肝功能异常并接受肝活检的患者资料,中国,2022年3月至2023年7月分析肝脏病理和临床表现。
    本研究共纳入1302名患者,其中主要包括11种疾病:自身免疫性肝病(74例,5.68%),药物性肝损伤(DILI)(204例,15.67%),癌症(237例,18.20%),非酒精性脂肪性肝病(104例,7.99%),非酒精性脂肪性肝炎(74例,5.68%),病毒性肝炎(490例,37.63%),其他类型的肝炎(30例,2.30%),胆汁淤积性肝病(17例,1.31%),酒精性肝病(15例,1.15%),肝囊肿(5例,0.38%)和吉尔伯特综合征(4例,0.31%)。肝活检取样成功率100%,取(1.52±0.130)条组织条。平均手术时间为11.52分钟。经皮肝穿刺活检并没有显着增加短期肝功能指标值(血清γ-谷氨酰转肽酶,总胆红素,丙氨酸转氨酶,天冬氨酸转氨酶,碱性磷酸酶)。92例患者有少量肝包膜下积液,但是治疗后没有进展。
    超声引导下经皮肝穿刺活检对不明原因的肝功能异常具有诊断价值。病毒性肝炎,癌症和DILI是无法解释的肝功能异常的最常见原因。肝活检不会加重肝脏的器官和功能损害。
    UNASSIGNED: To analyse clinical manifestations of unexplained abnormal liver function and perform hepatobiliary histopathology procedures on patients to evaluate the value of liver biopsy in diagnosing the aetiology of unexplained abnormal liver function.
    UNASSIGNED: A convenience sampling method was used to retrospectively collect the data of patients who were diagnosed with unexplained abnormal liver function and who received liver biopsy in the Pathology Department of Tianjin Second People\'s Hospital, China, between March 2022 and July 2023 to analyse liver pathology and clinical manifestations.
    UNASSIGNED: A total of 1302 patients were included in this study, which mainly included 11 diseases: autoimmune liver disease (74 cases, 5.68%), drug-induced liver injury (DILI) (204 cases, 15.67%), cancer (237 cases, 18.20%), non-alcoholic fatty liver disease (104 cases, 7.99%), non-alcoholic steatohepatitis (74 cases, 5.68%), viral hepatitis (490 cases, 37.63%), other types of hepatitis (30 cases, 2.30%), cholestatic liver disease (17 cases, 1.31%), alcoholic liver disease (15 cases, 1.15%), hepatic cyst (5 cases, 0.38%) and Gilbert syndrome (4 cases, 0.31%). The success rate of liver biopsy sampling was 100%, and (1.52 ± 0.130) tissue strips were sampled. The average operating time was 11.52 minutes. The percutaneous liver biopsy did not significantly increase short-term liver function index values (serum γ-glutamyl transpeptidase, total bilirubin, alanine transaminase, aspartate aminotransferase, alkaline phosphatase). Ninety-two patients had a small amount of liver subcapsular fluid, but there was no progress after medical treatment.
    UNASSIGNED: Ultrasound-guided percutaneous liver biopsy has value in the diagnosis of unexplained abnormal liver function. Viral hepatitis, cancer and DILI are the most common causes of unexplained abnormal liver function. Liver biopsy does not aggravate the organic and functional impairment of the liver.
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  • 文章类型: Case Reports
    原发性骨髓纤维化(PMF)是非肝硬化门脉高压(PH)的罕见病因。在临床环境中,非肝硬化PH常被误诊为肝硬化PH。该病例报告详细介绍了一名患者,该患者表现出复发性食管静脉曲张破裂出血,最初被误诊为肝硬化。最初准备进行肝移植,患者肝活检显示无明显肝硬化,但有髓外造血(EMH)征象。在对PMF进行准确诊断后,病人接受了标准治疗,导致因食管静脉曲张引起的胃肠道反复出血近3年。
    Primary myelofibrosis (PMF) is an infrequent etiology of noncirrhotic portal hypertension (PH). In clinical settings, non-cirrhotic PH is often misdiagnosed as cirrhotic PH. This case report details a patient who exhibited recurrent esophageal variceal hemorrhage and was initially misdiagnosed with cirrhosis. Initially poised for liver transplantation, the patient\'s liver biopsy revealed no significant cirrhosis but showed signs of extramedullary hematopoiesis (EMH). Following the accurate diagnosis of PMF, the patient underwent standard treatment, leading to an absence of recurrent gastrointestinal hemorrhage due to esophageal varices for nearly three years.
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  • 文章类型: Journal Article
    背景:非酒精性脂肪性肝病(NAFLD)肝组织学NAFLD活动评分≥4,纤维化阶段F≥2被视为“处于危险中”非酒精性脂肪性肝炎(NASH)。基于国际共识,NAFLD和NASH更名为代谢功能障碍相关脂肪性肝炎(MASLD)和代谢功能障碍相关脂肪性肝炎(MASH)。分别;因此,我们引入了“高风险MASH”一词。七种非侵入性模型的诊断价值,包括FibroScan-天冬氨酸转氨酶(FAST),纤维化-4(FIB-4),天冬氨酸转氨酶与血小板比值指数(APRI),等。对于高风险MASH,很少在MASLD中进行研究和比较。
    目的:评估七种非侵入性模型作为肝活检诊断高危MASH的替代方法的临床价值。
    方法:对北京地坛医院肝活检诊断为NAFLD的309例患者进行回顾性分析。2012年1月至2020年12月。在筛选MASLD和排除标准后,279例患者被纳入并分为高危和非高危MASH组。利用每个模型的阈值,灵敏度,特异性,阳性预测值(PPV),和负预测值(NPV),被计算。构建受试者工作特征曲线以基于曲线下面积(AUROC)评价其诊断效能。
    结果:99.4%的NAFLD患者符合MASLD诊断标准。在两个队列中分析了MASLD人群:总体人群(279名患者)和接受肝脏瞬时弹性成像(FibroScan)的亚组(117名患者)。在总人口中,FIB-4显示更好的诊断效能和更高的PPV,有了敏感性,特异性,PPV,NPV,AUROC为26.9%,95.2%,73.5%,72.2%,和0.75。APRI,Forns索引,天冬氨酸转氨酶与丙氨酸转氨酶比值(ARR)显示出中等诊断功效,而S指数和γ-谷氨酰转肽酶与血小板比率(GPR)相对较弱。在子组中,FAST的诊断效能最高,其灵敏度,特异性,PPV,NPV,AUROC为44.2%,92.3%,82.1%,67.4%,0.82FIB-4AUROC为0.76。S指数和GPR对高危MASH几乎没有诊断价值。
    结论:与APRI相比,FAST和FIB-4可以代替肝活检作为高危MASH的更有效的诊断方法,Forns索引,ARR,S指数,和探地雷达;FAST优于FIB-4。
    BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) with hepatic histological NAFLD activity score ≥ 4 and fibrosis stage F ≥ 2 is regarded as \"at risk\" non-alcoholic steatohepatitis (NASH). Based on an international consensus, NAFLD and NASH were renamed as metabolic dysfunction-associated steatotic liver disease (MASLD) and metabolic dysfunction-associated steatohepatitis (MASH), respectively; hence, we introduced the term \"high-risk MASH\". Diagnostic values of seven non-invasive models, including FibroScan-aspartate transaminase (FAST), fibrosis-4 (FIB-4), aspartate transaminase to platelet ratio index (APRI), etc. for high-risk MASH have rarely been studied and compared in MASLD.
    OBJECTIVE: To assess the clinical value of seven non-invasive models as alternatives to liver biopsy for diagnosing high-risk MASH.
    METHODS: A retrospective analysis was conducted on 309 patients diagnosed with NAFLD via liver biopsy at Beijing Ditan Hospital, between January 2012 and December 2020. After screening for MASLD and the exclusion criteria, 279 patients were included and categorized into high-risk and non-high-risk MASH groups. Utilizing threshold values of each model, sensitivity, specificity, positive predictive value (PPV), and negative predictive values (NPV), were calculated. Receiver operating characteristic curves were constructed to evaluate their diagnostic efficacy based on the area under the curve (AUROC).
    RESULTS: MASLD diagnostic criteria were met by 99.4% patients with NAFLD. The MASLD population was analyzed in two cohorts: Overall population (279 patients) and the subgroup (117 patients) who underwent liver transient elastography (FibroScan). In the overall population, FIB-4 showed better diagnostic efficacy and higher PPV, with sensitivity, specificity, PPV, NPV, and AUROC of 26.9%, 95.2%, 73.5%, 72.2%, and 0.75. APRI, Forns index, and aspartate transaminase to alanine transaminase ratio (ARR) showed moderate diagnostic efficacy, whereas S index and gamma-glutamyl transpeptidase to platelet ratio (GPR) were relatively weaker. In the subgroup, FAST had the highest diagnostic efficacy, its sensitivity, specificity, PPV, NPV, and AUROC were 44.2%, 92.3%, 82.1%, 67.4%, and 0.82. The FIB-4 AUROC was 0.76. S index and GPR exhibited almost no diagnostic value for high-risk MASH.
    CONCLUSIONS: FAST and FIB-4 could replace liver biopsy as more effectively diagnostic methods for high-risk MASH compared to APRI, Forns index, ARR, S index, and GPR; FAST is superior to FIB-4.
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  • 文章类型: Case Reports
    肝脏炎性假瘤(IPT)是一种罕见的疾病,通常伪装成恶性肿瘤,导致误诊和不必要的手术切除。IgG4相关疾病(IgG4-RD)的新兴概念已获得广泛认可,涵盖IgG4相关肝IPT等实体。临床和放射学,皮质类固醇和免疫抑制疗法已被证明可以有效控制这种情况。
    一个3岁的中国男孩出现在诊所,有11个月的贫血史,不明原因的发烧,还有一个细嫩的肝脏肿块.血液检查显示慢性贫血(Hb:6.4g/L,MCV:68.6fl,MCH:19.5pg,网织红细胞:1.7%)伴有炎症反应和血清IgG4水平升高(1542.2mg/L)。腹部对比增强计算机断层扫描显示右侧叶有一个7.6厘米的低密度肿块,磁共振成像在T1加权图像上显示出轻微的低强度,在T2加权图像上显示出轻微的高强度,提示怀疑肝脏恶性肿瘤。随后的肝活检显示肿块,其特征是纤维基质和密集的淋巴浆细胞浸润。免疫组织化学分析证实了IgG4阳性浆细胞的存在,导致IgG4相关性肝IPT的诊断。在开始使用皮质类固醇和霉酚酸酯治疗后迅速消退。
    本研究强调了肝IPT的诊断方法,利用组织病理学,免疫染色,成像,血清学,器官受累,和治疗反应。早期组织学检查在临床指导中起着举足轻重的作用,避免误诊为肝肿瘤和不必要的手术干预。
    UNASSIGNED: Hepatic Inflammatory Pseudotumor (IPT) is an infrequent condition often masquerading as a malignant tumor, resulting in misdiagnosis and unnecessary surgical resection. The emerging concept of IgG4-related diseases (IgG4-RD) has gained widespread recognition, encompassing entities like IgG4-related hepatic IPT. Clinically and radiologically, corticosteroids and immunosuppressive therapies have proven effective in managing this condition.
    UNASSIGNED: A 3-year-old Chinese boy presented to the clinic with an 11-month history of anemia, fever of unknown origin, and a tender hepatic mass. Blood examinations revealed chronic anemia (Hb: 6.4 g/L, MCV: 68.6 fl, MCH: 19.5 pg, reticulocytes: 1.7%) accompanied by an inflammatory reaction and an elevated serum IgG4 level (1542.2 mg/L). Abdominal contrast-enhanced computed tomography unveiled a 7.6 cm low-density mass in the right lateral lobe, while magnetic resonance imaging demonstrated slight hypointensity on T1-weighted images and slight hyperintensity on T2-weighted images, prompting suspicion of hepatic malignancy. A subsequent liver biopsy revealed a mass characterized by fibrous stroma and dense lymphoplasmacytic infiltration. Immunohistochemical analysis confirmed the presence of IgG4-positive plasma cells, leading to the diagnosis of IgG4-related hepatic IPT. Swift resolution occurred upon initiation of corticosteroid and mycophenolate mofetil therapies.
    UNASSIGNED: This study underscores the diagnostic approach to hepatic IPT, utilizing histopathology, immunostaining, imaging, serology, organ involvement, and therapeutic response. Early histological examination plays a pivotal role in clinical guidance, averting misdiagnosis as a liver tumor and unnecessary surgical interventions.
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  • 文章类型: Journal Article
    先天性肝纤维化目前仍被认为是一种罕见的常染色体隐性遗传性疾病,该病与胆管板畸形所致的肝内胆管遗传发育障碍有关。现以1例多囊肾/多囊肝病变1基因突变致胆管炎型先天性肝纤维化患者为例,探讨该病发病原因、临床表现、诊断要点以及治疗进展,以期能够在一定程度上提高肝胆科医师对该病的认识,从而有效提高早期诊断率。.
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