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Histopathology of Wilson Disease 威尔逊病的组织病理学
Pub Date : 2020-12-17 DOI: 10.5772/INTECHOPEN.95105
N. Soylu
Wilson Disease (WD) is a genetic metabolic disease of copper metabolism. The implicated gene is ATP7B, encodes a P-type ATPase which transports copper. The resultant defective metabolism of copper results in copper accumulation in multiple tissues especially liver, eye and central nervous system. WD occurs worldwide, usually between 5 and 35 years; a wider age range is also reported. Clinical presentations are diverse and include combinations of hepatic, neurological, ophthalmic and psychiatric manifestations. Other organs or tissues may also be affected. Biochemical abnormalities such as serum ceruloplasmin and 24-h urinary copper excretion are important for the diagnosis but are not always abnormal in WD. The liver histopathology has several different patterns from mild nonspecific changes to acute fulminant hepatitis and cirrhosis. Copper histochemistry is helpful in diagnosis. Genetic testing is another diagnostic tool. It is important to diagnose WD because it is fatal when overlooked, curable when diagnosed. The diagnosis should be keep in mind at all ages in patients with hepatic disease, neurological disease, or psychiatric symptoms.
威尔逊病(WD)是一种铜代谢的遗传性代谢性疾病。与之相关的基因是ATP7B,它编码一种转运铜的p型atp酶。由此产生的铜代谢缺陷导致铜在多个组织中积聚,特别是肝脏、眼睛和中枢神经系统。WD发生在世界各地,通常在5至35岁之间;此外,还有更广泛的年龄范围的报道。临床表现多样,包括肝、神经、眼和精神表现的组合。其他器官或组织也可能受到影响。生化异常如血清铜蓝蛋白和24小时尿铜排泄对诊断很重要,但在WD中并不总是异常。肝组织病理学有几种不同的模式,从轻微的非特异性改变到急性暴发性肝炎和肝硬化。铜组织化学有助于诊断。基因检测是另一种诊断工具。诊断WD很重要,因为忽视它是致命的,诊断后可以治愈。所有年龄的肝病、神经系统疾病或精神症状患者都应牢记诊断。
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引用次数: 0
Acute on Chronic Liver Failure: Role of the Bacterial Infections 急性和慢性肝衰竭:细菌感染的作用
Pub Date : 2020-09-02 DOI: 10.5772/intechopen.93440
M. Borzio, E. Dionigi
Acute-on-chronic liver failure (ACLF) refers to a syndrome characterized by acute deterioration of liver function of a pre-existing chronic liver disease with increased short-term mortality at 3 months due to multiorgan failure. Definition of ACLF has been refined, but differences between western and eastern areas still exist. Diagnosis of ACLF as recommended by the EASL-CLIF consortium is based on the assessment of organ dysfunction. The pathogenesis of this syndrome is attributable to an exaggerated host response to inflammation, responsible for the severe haemodynamic derangement leading to multiorgan failure. ACLF is triggered by precipitating events like acute hepatitis either viral, drug-induced, toxic, or alcoholic, variceal bleeding and sepsis. Bacterial infection is currently considered the most frequent trigger of ACLF in Western countries. Cirrhotic patients, particularly if decompensated are prone to develop bacterial infection because loss of integrity of the intestinal mucosal barrier and translocation of pathogen-associated molecular patterns (PAMPs). Bacterial translocation may develop into overt infection at different sites, along with sepsis and septic shock that may lead to ACLF. Epidemiology of bacterial infection in cirrhosis has been changing and this accounts for new antibiotic regimens as empirical therapy in critically ill cirrhotic patients with bacterial infection. In this chapter, we will discuss on definition, pathogenesis, clinical aspects and therapy of bacterial infection-related ACLF.
急性伴慢性肝衰竭(ACLF)是指一种以既往存在的慢性肝病的肝功能急性恶化为特征的综合征,由于多器官衰竭,3个月时短期死亡率增加。ACLF的定义已得到完善,但东西部地区的差异仍然存在。EASL-CLIF联盟推荐的ACLF诊断是基于器官功能障碍的评估。该综合征的发病机制可归因于宿主对炎症的过度反应,导致严重的血流动力学紊乱,导致多器官功能衰竭。ACLF由突发事件触发,如病毒性、药物性、毒性或酒精性急性肝炎、静脉曲张出血和败血症。在西方国家,细菌感染目前被认为是ACLF最常见的触发因素。肝硬化患者,特别是代偿失调的患者,由于肠黏膜屏障完整性的丧失和病原体相关分子模式(PAMPs)的易位,容易发生细菌感染。细菌易位可发展为不同部位的明显感染,并伴有败血症和感染性休克,可导致ACLF。肝硬化细菌感染的流行病学一直在发生变化,这说明了新的抗生素方案作为重症肝硬化合并细菌感染患者的经验性治疗。在本章中,我们将讨论细菌感染相关ACLF的定义、发病机制、临床方面和治疗。
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引用次数: 0
Left Side Gallbladder: Clinical and Anatomical Implication 左侧胆囊:临床和解剖学意义
Pub Date : 2020-04-27 DOI: 10.5772/intechopen.92000
F. Banchini, P. Capelli
Left side gallbladder is a rare anatomical anomaly reported in the literature. It is associated with various anatomical variations of the biliary way and intrahepatic portal supply. Most of the time, it is discovered as an incidental finding during intervention for cholecystectomy, exposing patients and surgeons to high risk of complication. To prevent this, we analyze the critical aspects that must be known to perform safe interventions either in the normal setting or in the emergency setting. Different theories are proposed to describe this anomaly, but a debate is still open. Reviewing the literature and analyzing the different processes of formation, we create a classification that can explain how this anomaly can occur, dividing into four variation types.
左侧胆囊是文献中罕见的解剖异常。它与胆道和肝内门静脉供应的各种解剖变异有关。大多数情况下,它是在胆囊切除术干预过程中偶然发现的,使患者和外科医生面临高风险的并发症。为了防止这种情况发生,我们分析了在正常情况下或紧急情况下进行安全干预必须知道的关键方面。人们提出了不同的理论来描述这种异常现象,但争论仍然存在。回顾文献并分析不同的形成过程,我们创建了一个分类,可以解释这种异常是如何发生的,分为四种变异类型。
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引用次数: 1
Liver Assist Devices for Liver Failure 肝衰竭的肝辅助装置
Pub Date : 2020-03-28 DOI: 10.5772/intechopen.91287
A. Mandal, P. Garlapati, Benjamin Tiongson, Vijay Gayam
Historically, mortality rates for liver failure have been high, regardless of the type. With new advancements in liver transplantation (LTx), 1-year survival rates have improved up to 95% in most recent estimates. While some patients may live past the critical period, the majority of patients do not survive the interval period for awaiting LTx or liver regeneration. The function of the liver to detoxify and correct several biochemical parameters has been achieved to some extent through artificial liver support technology, although constant innovations are still being developed for the most optimal liver support device. The complex function of the liver makes it challenging since it does not only detoxify toxic by-products but also participates in numerous other synthetic and metabolic functions of the body. Liver support systems are divided into an artificial liver assist device (ALD) and a bioartificial liver assist device (BLD). ALDs include molecular adsorbent recirculating system (MARS), Prometheus, single-pass albumin dialysis, and selective plasma filtration therapy. These devices work as a blood purification system of the liver. On the other hand, BLD has hepatic cell lines incorporated in its equipment, which aims to function as a complex biological liver system providing support to its biochemical processes. Several clinical and randomized trials have conflicting results on the survival of the patients with acute liver failure (ALF), and the ideal liver support system still seems a far-off goal.
从历史上看,无论何种类型的肝功能衰竭,其死亡率都很高。随着肝移植(LTx)的新进展,在最近的估计中,1年生存率已提高到95%。虽然一些患者可能会活过关键期,但大多数患者无法活过等待LTx或肝脏再生的间隔期。通过人工肝支持技术,肝脏的解毒和纠正几个生化参数的功能已经在一定程度上实现,尽管最优的肝支持装置仍在不断创新。肝脏的复杂功能使其具有挑战性,因为它不仅解毒副产物,而且还参与许多其他合成和代谢功能的身体。肝支持系统分为人工肝辅助装置(ALD)和生物人工肝辅助装置(BLD)。ald包括分子吸附剂再循环系统(MARS)、普罗米修斯、单次白蛋白透析和选择性血浆过滤治疗。这些装置是肝脏的血液净化系统。另一方面,BLD在其设备中加入了肝细胞系,其目的是作为一个复杂的生物肝脏系统,为其生化过程提供支持。一些临床和随机试验对急性肝衰竭(ALF)患者的生存率有相互矛盾的结果,理想的肝支持系统似乎仍然是一个遥远的目标。
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引用次数: 2
Diagnosis and Treatment of Hepatoblastoma: An Update 肝母细胞瘤的诊断和治疗进展
Pub Date : 2020-01-07 DOI: 10.5772/intechopen.90830
Chengzhan Zhu, Dong Bingzi, Xin Chen, Q. Dong
Hepatoblastoma is a rare but the most common solid tumor in children. The incidence is gradually increasing. The international collaboration among four centers in the world has greatly improved the prognosis of hepatoblastoma. They formed the Children’s Hepatic Tumor International Collaboration (CHIC) to standardize the staging system (2017 PRETEXT system) and the risk factors for tumor stratification. Multimodal therapy has become the standard for the management of hepatoblastoma, including surgical resection, liver transplantation, chemotherapy, and so on. Surgery is the primary treatment of early stage hepatoblastoma. Three-dimensional reconstruction is helpful for preoperative evaluation of large tumors, assisting extended hepatectomy for patients in PRETEXT III or IV. Neoadjuvant therapy is useful for reducing the tumor volume and increasing the resectability. Primary liver transplantation is recommended for advanced hepatoblastoma. The lungs are the most common metastatic organ, the treatment of which is critical for the patient’s long-term survival. We reviewed the recent progress in the diagnosis and treatment of hepatoblastoma.
肝母细胞瘤是一种罕见但最常见的儿童实体瘤。发病率正在逐渐增加。国际四大中心的国际合作,极大地改善了肝母细胞瘤的预后。他们成立了儿童肝脏肿瘤国际合作组织(CHIC),标准化分期系统(2017年托普系统)和肿瘤分层的危险因素。多模式治疗已成为肝母细胞瘤治疗的标准,包括手术切除、肝移植、化疗等。手术是早期肝母细胞瘤的主要治疗方法。三维重建有助于术前对大肿瘤的评估,有助于对托普III期或IV期患者进行肝切除术。新辅助治疗有助于缩小肿瘤体积,提高可切除性。晚期肝母细胞瘤建议进行原发性肝移植。肺是最常见的转移器官,其治疗对患者的长期生存至关重要。现就近年来肝母细胞瘤的诊断和治疗进展作一综述。
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引用次数: 0
Stroke and Liver Cirrhosis: A Brief Review of Current Evidence 中风和肝硬化:当前证据的简要回顾
Pub Date : 2019-12-06 DOI: 10.5772/intechopen.90420
K. Zheng, Xiaozhong Guo, Xinhong Wang, X. Qi
Stroke and liver cirrhosis are common in our everyday clinical practice, both of which can lead to serious complications. Their association is unclear. In this chapter, we briefly summarized the epidemiology of liver cirrhosis in stroke, reviewed the current evidence regarding the association between liver cirrhosis and stroke, and discussed the potential mechanisms for explaining such an association, such as coagulopathy, hypoperfusion, cardiac diseases, diabetes, and dyslipidemia.
中风和肝硬化在我们的日常临床实践中很常见,两者都可能导致严重的并发症。他们之间的联系尚不清楚。在本章中,我们简要总结了脑卒中中肝硬化的流行病学,回顾了肝硬化与脑卒中之间关联的现有证据,并讨论了解释这种关联的潜在机制,如凝血功能障碍、灌注不足、心脏病、糖尿病和血脂异常。
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引用次数: 0
Towards the Study of Liver Failure: Protocol for a 90% Extended Hepatectomy in Mice 肝衰竭的研究:小鼠90%扩展肝切除术的方案
Pub Date : 2019-11-26 DOI: 10.5772/INTECHOPEN.90245
M. J. L. Thiebaud, Eduardo Cervantes-Alvarez, N. Navarro-Alvarez
Studies have shown that extended hepatectomy mimics post-hepatectomy liver failure (PHLF) and could also be used to study other small-for-flow syndromes. Extended hepatectomy can be defined as the removal of more than 70% of liver volume. At the molecular level, there seems to be a delayed entrance to the cell cycle, and thus liver dysfunction ensues. Therefore, there is an imperious need to study the mechanisms of such delay to understand how it can be regulated. While the classical 70% hepatectomy model to study liver regeneration has been previously described thoroughly, there are no protocols describing the surgical procedure for a 90% extended hepatectomy (90% EHx). Therefore, we here describe a detailed and reproducible protocol for such model, defining specific aspects that must be considered as well as the most common complications and troubleshooting strategies.
研究表明,延长肝切除术模拟肝切除术后肝衰竭(PHLF),也可用于研究其他小流量综合征。扩大肝切除术可定义为切除肝脏体积的70%以上。在分子水平上,似乎有一个延迟进入细胞周期,因此肝功能障碍随之而来。因此,迫切需要研究这种延迟的机制,以了解如何对其进行调节。虽然研究肝脏再生的经典70%肝切除术模型先前已被详细描述,但没有描述90%扩展肝切除术(90% EHx)的手术过程的方案。因此,我们在这里描述了这种模型的详细和可重复的协议,定义了必须考虑的特定方面以及最常见的并发症和故障排除策略。
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引用次数: 1
Microbiota, Inflammation, and Gut Barrier Dysfunction in HCC 肝细胞癌的微生物群、炎症和肠道屏障功能障碍
Pub Date : 2019-11-22 DOI: 10.5772/intechopen.89578
A. Ram, Gavin Wright, B. Vairappan
Hepatocellular carcinoma (HCC), which represents 90% of all primary liver cancers, is the fifth most common cancer and the third cause of cancer mortality rate. It is a complex disease with a poor prognosis. Incidence and mortality rates are increasing in many geographical regions, indicating a need for better management strategies. Chronic inflammation is the major driving factors for HCC development, which typically develops on the background of chronic liver disease (CLD). Currently, a large body of literature has focused on the key role of the gut-liver axis as the major pathophysiological mechanism of hepatic disease severity and HCC development. This chapter will describe the role of gut microbiota, inflammation, and intestinal barrier dysfunction-associated mechanism in the progression of HCC. In particular, enteric dysbiosis, tight junction, and inflammatory mediators in the pathogenesis of liver cancer will be discussed. Furthermore, this chapter will identify the possible potential therapeutic approach for the control of gut bacterial overgrowth, inflammation and restoration of eubiosis, and tight junction integrity in HCC.
肝细胞癌(HCC)占所有原发性肝癌的90%,是第五大常见癌症,也是癌症死亡率的第三大原因。这是一种预后不良的复杂疾病。许多地理区域的发病率和死亡率都在上升,这表明需要有更好的管理战略。慢性炎症是HCC发展的主要驱动因素,HCC通常在慢性肝病(CLD)的背景下发展。目前,大量文献关注肠-肝轴在肝病严重程度和HCC发展中的主要病理生理机制中的关键作用。本章将描述肠道菌群、炎症和肠屏障功能障碍相关机制在HCC进展中的作用。特别是,肠道失调,紧密连接和炎症介质在肝癌的发病机制将被讨论。此外,本章将确定HCC中可能的潜在治疗方法,以控制肠道细菌过度生长,炎症和益生菌恢复,以及紧密连接的完整性。
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引用次数: 2
Stereotactic Image-Guidance for Ablation of Malignant Liver Tumors 立体定向图像引导在恶性肝肿瘤消融中的应用
Pub Date : 2019-10-20 DOI: 10.5772/intechopen.89722
I. Paolucci, R. Sandu, P. Tinguely, C. Kim-Fuchs, M. Maurer, D. Candinas, S. Weber, A. Lachenmayer
Stereotactic percutaneous ablation is a rapidly advancing modality for treatment of tumors in soft solid organs such as the liver. Each year, there are about 850,000 cases of primary liver cancer worldwide. Although surgical resection still is the gold standard for most cases, only 20–30% of patients are candidates for it, due to the advanced stage of the disease. Surgery can also be a huge burden to the patient and his/her quality of life might be temporarily severely reduced due to long hospital stays, complications, and slow recovery. To overcome these disadvantages, thermo-ablation of tumors of up to 3 cm has become a more viable alternative especially in the last decade, offering a potentially equally effective but minimally invasive and tissue sparing treatment alternative. In conjunction with improved CT imaging, stereotactic image-guidance techniques and image fusion technology were introduced to increase safety, efficacy, and accuracy of this treatment. Stereotactic image-guidance leads to a simple, fast, and accurate placement of the ablation probe into the liver tumor, which is a prerequisite for a complete destruction of the tumor by ablation. More and more physicians, including surgeons, consider ablation a viable alternative to resection whenever feasible. Patients undergoing such a minimally invasive treatment benefit from a shorter hospital stays, reduced complication rates, and faster recovery.
立体定向经皮消融是一种快速发展的治疗软实体器官肿瘤的方式,如肝脏。每年,全世界约有85万例原发性肝癌病例。虽然手术切除仍然是大多数病例的金标准,但由于疾病的晚期,只有20-30%的患者适合手术切除。手术对病人来说也是一个巨大的负担,由于住院时间长、并发症和恢复缓慢,他/她的生活质量可能会暂时严重降低。为了克服这些缺点,尤其是在过去的十年中,对3厘米以内的肿瘤进行热消融已经成为一种更可行的替代方法,它提供了一种潜在的同样有效但微创和组织保留的治疗方法。结合改进的CT成像技术,立体定向图像引导技术和图像融合技术被引入,以提高该治疗的安全性、有效性和准确性。立体定向图像引导可以简单、快速、准确地将消融探头置入肝脏肿瘤中,这是消融完全破坏肿瘤的先决条件。越来越多的内科医生,包括外科医生,认为消融是切除的可行选择。接受这种微创治疗的患者受益于更短的住院时间、更低的并发症发生率和更快的恢复。
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引用次数: 5
Early Postoperative Monitoring of the Liver Graft 肝移植术后早期监测
Pub Date : 2019-10-09 DOI: 10.5772/intechopen.89094
D. P. Civantos, A. Cantero, M. R. Marcos, Francisco Fuentes Morillas, María Ángeles Santiago Triviño, M. D. Arias, M. D. Frutos, A. López
Liver transplantation (LT) is a common current technique for end-stage liver disease. Complications after the surgical procedure, though uncommon, can be of very different origin and can also be severe enough to lead to liver and multiorgan failure and finally graft loss and/or recipient’s death. Intensivists and the surgical team must be familiarized with these early complications to detect them as soon as possible in order to use the best diagnostic tools and take the best therapeutic measures to restore anatomical integrity and organ function to optimize the liver graft. In this chapter, we present an updated state of the art for efficiently tackling with all different, most usual complications that an LT patient can present during early postoperative period.
肝移植是目前治疗终末期肝病的常用技术。手术后的并发症虽然不常见,但可能有非常不同的原因,也可能严重到足以导致肝脏和多器官衰竭,最终导致移植物丢失和/或受体死亡。强化医师和手术团队必须熟悉这些早期并发症,尽早发现,以便使用最好的诊断工具,采取最好的治疗措施,恢复解剖完整性和器官功能,优化肝移植。在本章中,我们介绍了最新的技术,以有效地处理术后早期肝移植患者可能出现的所有不同的、最常见的并发症。
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引用次数: 0
期刊
Liver Pathology
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