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Wilson's disease:Revisiting an old friend

World Journal of Hepatology 2021年第6期13卷 634-649页

作者： Ana Lucena-Valera Domingo Perez-Palacios Rocio Muñoz-Hernandez Manuel Romero-Gómez Javier Ampuero Department of Gastroenterology Hospital Universitario Virgen del RocioSevilla 41013Spain Seliver Group Instituto de Biomedicina de Sevilla(IBiS)Hospital Universitario Virgen del Rocío/CSIC/Universidad de SevillaSevillaEspaña Department of Unit of Digestive diseases Hospital Universitario Virgen del RocioSevilla 41014Spain

Wilson's disease(WD)is a rare condition caused by copper accumulation primarily in the liver and secondly in other organs,such as the central nervous system.It is a hereditary autosomal recessive disease caused by a deficiency in the ATP7B transporter.This protein facilitates the incorporation of copper into ceruloplasmin.More than 800 mutations associated with WD have been described.The onset of the disease frequently includes manifestations related to the liver(as chronic Liver disease or acute liver failure)and neurological symptoms,although it can sometimes be asymptomatic.Despite it being more frequent in young people,WD has been described in all life stages.Due to its fatal prognosis,WD should be suspected in all patients with unexplained biochemical liver abnormalities or neurological or psychiatric symptoms.The diagnosis is established with a combination of clinical signs and tests,including the measurement of ceruloplasmin,urinary copper excretion,copper quantification in liver biopsy,or genetic assessment.The pharmacological therapies include chelating drugs,such as D-penicillamine or trientine,and zinc salts,which are able to change the natural history of the disease,increasing the survival of these patients.In some cases of end-stage Liver disease or acute liver failure,liver transplantation must be an option to increase survival.In this narrative review,we offer an overview of WD,focusing on the importance of clinical suspicion,the correct diagnosis,and treatment.

关键词： Wilson´s disease Copper ATP7B Ceruloplasmin Chelator Liver disease

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Application of the woodchuck animal model for the treatment of hepatitis B virus-induced liver cancer

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World Journal of Gastrointestinal Oncology 2021年第6期13卷 509-535页

作者： Manasa Suresh Stephan Menne Department of Microbiology and Immunology Georgetown University Medical CenterWashingtonDC 20057United States

This review describes woodchucks chronically infected with the woodchuck hepatitis virus(WHV)as an animal model for hepatocarcinogenesis and treatment of primary liver cancer or hepatocellular carcinoma(HCC)induced by the hepatitis B virus(HBV).Since laboratory animal models susceptible to HBV infection are limited,woodchucks experimentally infected with WHV,a hepatitis virus closely related to HBV,are increasingly used to enhance our understanding of virus-host interactions,immune response,and Liver disease progression.A correlation of severe liver pathogenesis with high-level viral replication and deficient antiviral immunity has been established,which are present during chronic infection after WHV inoculation of neonatal woodchucks for modeling vertical HBV transmission in humans.HCC in chronic carrier woodchucks develops 17 to 36 mo after neonatal WHV infection and involves liver tumors that are comparable in size,morphology,and molecular gene signature to those of HBV-infected patients.Accordingly,woodchucks with WHV-induced liver tumors have been used for the improvement of imaging and ablation techniques of human HCC.In addition,drug efficacy studies in woodchucks with chronic WHV infection have revealed that prolonged treatment with nucleos(t)ide analogs,alone or in combination with other compounds,minimizes the risk of Liver disease progression to HCC.More recently,woodchucks have been utilized in the delineation of mechanisms involved in innate and adaptive immune responses against WHV during acute,self-limited and chronic infections.Therapeutic interventions based on modulating the deficient host antiviral immunity have been explored in woodchucks for inducing functional cure in HBV-infected patients and for reducing or even delaying associated Liver disease sequelae,including the onset of HCC.Therefore,woodchucks with chronic WHV infection constitute a well-characterized,fully immunocompetent animal model for HBV-induced liver cancer and for preclinical evaluation of the safety and efficacy of new mod

关键词： Woodchuck Hepatitis B virus Chronic infection Liver disease Hepatocellular carcinoma Cancer treatment

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COVID-19 and comorbidities of hepatic diseases in a global perspective

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World Journal of Gastroenterology 2021年第13期27卷 1296-1310页

作者： Aqsa Ahmad Syeda Momna Ishtiaq Junaid Ali Khan Rizwan Aslam Sultan Ali Muhammad Imran Arshad Institute of Microbiology University of Agriculture FaisalabadFaisalabad 38040PunjabPakistan Institute of Physiology and Pharmacology University of Agriculture FaisalabadFaisalabad 38040PunjabPakistan

The worldwide outbreak of coronavirus disease 2019(COVID-19) has challenged the priorities of healthcare system in terms of different clinical management and infection transmission, particularly those related to hepatic-disease comorbidities. Epidemiological data evidenced that COVID-19 patients with altered liver function because of hepatitis infection and cholestasis have an adverse prognosis and experience worse health outcomes. COVID-19-associated liver injury is correlated with various Liver diseases following a severe acute respiratory syndrome-coronavirus type 2(SARS-CoV-2) infection that can progress during the treatment of COVID-19 patients with or without pre-existing Liver disease. SARS-CoV-2 can induce liver injury in a number of ways including direct cytopathic effect of the virus on cholangiocytes/hepatocytes, immune-mediated damage, hypoxia, and sepsis. Indeed, immediate cytopathogenic effects of SARSCoV-2 via its potential target, the angiotensin-converting enzyme-2 receptor, which is highly expressed in hepatocytes and cholangiocytes, renders the liver as an extra-respiratory organ with increased susceptibility to pathological outcomes. But, underlying COVID-19-linked Liver disease pathogenesis with abnormal liver function tests(LFTs) is incompletely understood. Hence, we collated COVID-19-associated liver injuries with increased LFTs at the nexus of pre-existing Liver diseases and COVID-19, and defining a plausible pathophysiological triad of COVID-19, hepatocellular damage, and Liver disease. This review summarizes recent findings of the exacerbating role of COVID-19 in pre-existing Liver disease and vice versa as well as international guidelines of clinical care, management, and treatment recommendations for COVID-19 patients with Liver disease.

关键词： Liver disease COVID-19 Pathophysiology Epidemiology Prophylaxis

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Probiotics in hepatology:An update

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World Journal of Hepatology 2021年第9期13卷 1154-1166页

作者： Roman Maslennikov Vladimir Ivashkin Irina Efremova Elena Poluektova Elena Shirokova Department of Internal Medicine Gastroenterology and HepatologySechenov UniversityMoscow 119435Russia Scientific Community for Human Microbiome Research Moscow 119435Russia Department of Internal Medicine Consultative and Diagnostic Center of the Moscow City Health DepartmentMoscow 107564Russia

The gut-liver axis plays an important role in the pathogenesis of various Liver diseases.Probiotics are living bacteria that may be used to correct disorders of this axis.Notable progress has been made in the study of probiotic drugs for the treatment of various Liver diseases in the last decade.It has been proven that probiotics are useful for hepatic encephalopathy,but their effects on other symptoms and syndromes of cirrhosis are poorly studied.Their effectiveness in the treatment of metabolic associated fatty Liver disease has been shown both in experimental models and in clinical trials,but their effect on the prognosis of this disease has not been described.The beneficial effects of probiotics in alcoholic Liver disease have been shown in many experimental studies,but there are very few clinical trials to support these findings.The effects of probiotics on the course of other Liver diseases are either poorly studied(such as primary sclerosing cholangitis,chronic hepatitis B and C,and autoimmune hepatitis)or not studied at all(such as primary biliary cholangitis,hepatitis A and E,Wilson's disease,hemochromatosis,storage diseases,and vascular Liver diseases).Thus,despite the progress in the study of probiotics in hepatology over the past decade,there are many unexplored and unclear questions surrounding this topic.

关键词： Gut-liver axis Pathogenesis Gut dysbiosis Gut microbiota Gut microbiome Liver disease Probiotics Hepatic encephalopathy Cirrhosis Metabolic associated fatty Liver disease Alcoholic Liver disease Primary sclerosing cholangitis

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The Complement System in Liver diseases

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Cellular & Molecular Immunology 2006年第5期3卷 333-340页

作者： Xuebin Qin Bin Gao Laboratory for Translational Research Harvard Medical School Cambridge MA 02139 USA Section on liver Biology Laboratory of Physiologic Studies National Institute on Alcohol Abuse and Alcoholism National Institutes of Health Bethesda MD 20892 USA Laboratory for Translational Research Harvard Medical School One Kendall Square Building 600 3rd Floor Cambridge MA 02139 USA.

The complement system plays an important role in mediating both acquired and innate responses to defend against microbial infection, and in disposing immunoglobins and apoptotic cells. The liver （mainly hepatocytes） is responsible for biosynthesis of about 80-90% of plasma complement components and expresses a variety of complement receptors. Recent evidence from several studies suggests that the complement system is also involved in the pathogenesis of a variety of liver disorders including liver injury and repair, fibrosis, viral hepatitis, alcoholic Liver disease, and liver ischemia/reperfusion injury. In this review, we will discuss the potential role of the complement system in the pathogenesis of Liver diseases. Cellular ＆ Molecular Immunology.

关键词： immunity complement Liver disease

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Current understanding of mesenchymal stem cells in Liver diseases

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World Journal of Stem Cells 2021年第9期13卷 1349-1359页

作者： Mu-Chen Wu Qing-Hua Meng Department of Medical Oncology You An HospitalCapital Medical UniversityBeijing 100069China

Liver diseases caused by various factors have become a significant threat to publichealth worldwide. liver transplantation has been considered as the only effectivetreatment for end-stage Liver diseases;however, it is limited by the shortage ofdonor organs, postoperative complications, long-term immunosuppression, andhigh cost of treatment. Thus, it is not available for all patients. Recently,mesenchymal stem cells (MSCs) transplantation has been extensively explored forrepairing hepatic injury in various Liver diseases. MSCs are multipotent adultprogenitor cells originated from the embryonic mesoderm, and can be found inmesenchymal tissues including the bone marrow, umbilical cord blood, adiposetissue, liver, lung, and others. Although the precise mechanisms of MSC transplantationremain mysterious, MSCs have been demonstrated to be able toprevent the progression of liver injury and improve liver function. MSCs can selfrenewby dividing, migrating to injury sites and differentiating into multiple celltypes including hepatocytes. Additionally, MSCs have immune-modulatoryproperties and release paracrine soluble factors. Indeed, the safety and effectivenessof MSC therapy for Liver diseases have been demonstrated in animals.However, pre-clinical and clinical trials are largely required to confirm its safetyand efficacy before large scale clinical application. In this review, we will explorethe molecular mechanisms underlying therapeutic effects of MSCs on liverdiseases. We also summarize clinical advances in MSC-based therapies.

关键词： Mesenchymal stem cell Liver disease Clinical trial Treatment Safety Efficacy

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Progressive Neuronal Degeneration of Childhood with liver's disease (Alpers' disease) Clinical Features and Neuropathological Studies of 4 Sibling

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中国临床神经科学 2000年第Z1期8卷 95-页

作者： Yupu Guo Zhong Guo Haowen Liu Mingwe Wang Hongwei Duan Shufang Gao Haitao Ren Department of Neurology Peking Union Medical College Hospital Beijing100730 Department of Neurology Peking Union Medical College Hospital Beijing100730 Department of Neurology Peking Union Medical College Hospital Beijing100730 Department of Neurology Peking Union Medical College Hospital Beijing100730 Department of Neurology Peking Union Medical College Hospital Beijing100730 Department of Neurology Peking Union Medical College Hospital Beijing100730 Department of Neurology Peking Union Medical College Hospital Beijing100730

We report four siblings of a family with Alpers' disease. Three of four siblings occurred diarrhea and myoclonus at the ages of 7 to 8 years old. During the disease evolution, symptoms of subacute encephalopathy such as headache, visual disturbance, cortical blindness, progressive seizures and mental retardation were presented at the ages of 15 to 20 years old. Downhill progression led them to death in multiple organ failure within six to eight months of onset. CT showed hypodensity lesions in the bilateral oc cipital and temporal lobes. Spongiform changes, which characterized by diffuse neuronal degeneration or loss and astrocytosis, were most severe in the gray matter. White matter was slightly involved, while basal ganglia, pons, brain stem and cerebellum were not involved. Physical examination of the only live brother of the four siblings showed short status (165 cm), arched feet and improper nose-pointed test of the left side. Muscle biopsy of him showed a large amount of Red-Ragged (RR) fibers and abnormal mitochondria. Clinical features and pathological findings of autopsy in all the four siblings were consistent with progressive neuronal degeneration of childhood with Liver disease (PNDC) - Alpers' disease. The muscle biopsy showed the characteristic findings of mitochondrial myopathy. Our report confirmed the classification of late onset Alpers' disease as a mitochondrial disorders.

关键词： neuronal degeneration multiple organ failure mental retardation examination of Liver disease basal ganglia

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Simultaneous embolization of a spontaneous porto-systemic shunt and intrahepatic arterioportal fistula:A case report

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World Journal of Clinical Cases 2021年第31期9卷 9577-9583页

作者： Guo-Feng Liu Xiao-Ze Wang Xue-Feng Luo Department of Gastroenterology and Hepatology Sichuan University-University of Oxford Huaxi Joint Centre for Gastrointestinal CancerWest China HospitalSichuan UniversityChengdu 610041Sichuan ProvinceChina

BACKGROUND Hepatic encephalopathy(HE)is a frequent and debilitating complication of chronic Liver disease.Recurrent HE is strongly linked with spontaneous portosystemic shunts(SPSSs).Intrahepatic arterioportal fistulas(IAPFs)occur rarely but pose a major clinical challenge and may lead to or worsen portal hypertension.Herein,we present a rare case of recurrent HE secondary to a SPSS combined with an IAPF.CASE SUMMARY A 63-year-old female with primary biliary cirrhosis presented with recurrent disturbance of consciousness for 4 mo.SPSS communicating the superior mesenteric vein with the inferior vena cava and IAPF linking the intrahepatic artery with the portal vein were found on contrast-enhanced abdominal computed tomography.The patient did not respond well to medical treatment.Therefore,simultaneous embolization of SPSS and IAPF was scheduled.After embolization,the symptoms of HE showed obvious resolution.CONCLUSION The presence of liver vascular disorders should not be neglected in patients with chronic Liver disease,and interventional therapy is a reasonable choice in such patients.

关键词： Spontaneous portosystemic shunts Intrahepatic arterioportal fistula Endovascular embolization Hepatic encephalopathy Liver disease Case report

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Role of endoscopic ultrasound in the field of hepatology: Recent advances and future trends

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World Journal of Hepatology 2021年第11期13卷 1459-1483页

作者： Jahnvi Dhar Jayanta Samanta Department of Gastroenterology Post Graduate Institute of Medical Education and ResearchChandigarh 160012India

The role of endoscopic ultrasound(EUS)as a diagnostic and therapeutic modality for the management of various gastrointestinal diseases has been expanding.The imaging or intervention for various Liver diseases has primarily been the domain of radiologists.With the advances in EUS,the domain of endosonologists is rapidly expanding in the field of hepatology.The ability to combine endoscopy and sonography in one hybrid device is a unique property of EUS,together with the ability to bring its probe/transducer near the liver,the area of interest.Its excellent spatial resolution and ability to provide real-time images coupled with several enhancement techniques,such as contrast-enhanced(CE)EUS,have facilitated the growth of EUS.The concept of“Endo-hepatology”encompasses the wide range of diagnostic and therapeutic procedures that are now gradually becoming feasible for managing various Liver diseases.Diagnostic advancements can enable a wide array of techniques from elastography and liver biopsy for liver parenchymal diseases,to CE-EUS for focal liver lesions to portal pressure measurements for managing various liver conditions.Similarly,therapeutic advancements range from EUS-guided eradication of varices,drainage of bilomas and abscesses to various EUS-guided modalities of liver tumor management.We provide a comprehensive review of all the different diagnostic and therapeutic EUS modalities available for the management of various Liver diseases.A synopsis of all the technical details involving each procedure and the available data has been tabulated,and the future trends in this area have been highlighted.

关键词： Endoscopic ultrasound Liver disease Elastography Varices liver tumor liver biopsy

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Traditional Chinese medicine diagnosis and treatment based on systematics

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iliver 2023年第4期2卷 181-187页

作者： Zhanhong Wang Danli Wang Wen Liu Zhongxia Wang The State Key Laboratory of Management and Control for Complex Systems Institute of AutomationChinese Academy of SciencesBeijing 100190 School of Artificial Intelligence University of Chinese Academy of SciencesBeijing 100049China Beijing Hospital of Traditional Chinese Medicine Capital Medical UniversityBeijing 100010China Department of Nutrition Fifth Medical CenterPLA General HospitalBeijing 100039China

Background:Traditional Chinese Medicine(TCM)is a well-established medical system with a long history.However,the overall concept and systematic thinking in TCM are not comprehensively understood and applied in its inheritance and development.Objective This study aims to provide a basic theory for TCM diagnosis using systematics as the guiding principle.Using modern scientific and technological achievements,we aim to explore a new TCM diagnosis method.Methods:We analyzed previous studies on TCM diagnosis and treatment,and reviewed clinical research on TCM diagnosis and treatment from the viewpoint of systematics.We propose a new process model based on systematics for TCM diagnosis and treatment.This is a generalized model that summarizes the process of“establishing an image to express meaning”.Results:The proposed model was implemented in the clinical practice of TCM.We monitored the detailed treatment process of patients in the Department of Liver diseases at Beijing Hospital.One patient underwent a treatment program that lasted 1 year and 45 days,consisting of 12 iterative cycles,each guided by the proposed diagnostic model and tailored to the patient's evolving condition.This case study validates the effectiveness of the proposed model in the diagnosis and treatment of Liver disease in TCM.The therapeutic efficacy has been validated through the examination of both TCM indicators and Western medical auxiliary parameters.Among these,the TCM indicators consist of 2 components:tongue diagnosis and pulse diagnosis.Meanwhile,the Western medical auxiliary indicators encompass a range of assessments,including whole blood cell analysis,professional liver function examination,a series of liver function assessments,a high-sensitivity hepatitis B pentathlete test,as well as color Doppler ultrasound evaluations of the liver,bile duct,pancreas,spleen,and assessments of liver elasticity,among other related examination parameters.In conclusion,it is evident that the syndrome of liver depression and spleen deficiency has signif

关键词： Traditional Chinesemedicine Systematics Diagnosis and treatment model Liver disease

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