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Heritable Chronic Cholestatic Liver Diseases:A Review

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Journal of Clinical and Translational Hepatology 2024年第8期12卷 726-738页

Chronic cholestasis due to heritable causes is usually diagnosedin ***,many cases can present andsurvive into *** time course varies considerablydepending on the underlying *** data usuallyreveal elevated conjugated hyperbilirubinemia,alkalinephosphatase,and gamma-glutamyl *** be asymptomatic;however,when present,the typicalsymptoms are pruritus,jaundice,fatigue,and *** diagnostic methods and management requireddepend on the underlying *** development of genome-wide associated studies has allowed the identificationof specific genetic mutations related to the pathophysiologyof cholestatic liver *** aim of this review was tohighlight the genetics,clinical pathophysiology,presentation,diagnosis,and treatment of heritable etiologies of chroniccholestatic liver disease.

关键词： Chronic cholestasis Cholestatic liver disease Congenital hepatic fibrosis Caroli disease Caroli syndrome Alagille syndrome Biliary atresia

Focal Nodular Hyperplasia: A Comprehensive Review with a Particular Focus on Pathogenesis and Complications

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Journal of Clinical and Translational Hepatology 2024年第2期12卷 182-190页

作者： Jaimy Villavicencio Kim george Y.wu Division of Gastroenterology-Hepatology University of Connecticut Health CenterFarmingtonCTUSA

Focal nodular hyperplasia is a benign tumor of the liver that is often found incidentally with *** purpose of this review is to discuss the pathophysiology,rare complications that can occur due to these lesions,and management options.A literature review was performed on clinical trials and case reports involving focal nodular hyperplasia complications and management of these,as well as the proposed pathogenesis underlying these *** exposure to oral contraceptive pills and endogenous hormones have been thought to play a role in the development of these lesions,this has not been *** recently,they are thought to arise as a consequence of a vascular anomaly causing alterations in the expression of angiopoietin *** are rare,but previous cases have reported associated pain,rupture and compression of nearby structures(hepatic vein,stomach,biliary system).Resection of focal nodular hyperplasia is not usually ***,if there is associated pain with no other identifiable cause or presence of a large or growing lesion with risk of causing a complication,then surgical resection,radiofrequency ablation or arterial embolization should be considered.

关键词： Focal nodular hyperplasia Oral contraceptives Liver lesion

Subnormal Serum Liver Enzyme Levels:A Review of Pathophysiology and Clinical Significance

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Journal of Clinical and Translational Hepatology 2024年第4期12卷 428-435页

作者： Elham M.Youssef george Y.wu Biochemistry Department National Research CenterCairoEgypt Department of Medicine Division of Gastroenterology-HepatologyUniversity of Connecticut Health CenterFarmingtonCTUSA

Subnormal levels of liver enzymes,below the lower limit of normal on local laboratory reports,can be useful *** instance,subnormal levels of aminotransferases can be observed in vitamin B6 deficiency and chronic kidney *** alkaline phosphatase levels may indicate the presence of hypophosphatasia,Wilson's disease,deficiencies of divalent ions,or *** levels of gamma glutamyl transferase may be seen in cases of acute intrahepatic cholestasis,the use of certain medications,and in bone ***,subnormal levels of 5'-nucleotidase have been reported in lead poisoning and nonspherocytic hemolytic *** aim of this review is to bring attention to the fact that subnormal levels of these enzymes should not be ignored as they may indicate pathological conditions and provide a means of early diagnosis.

关键词： Transaminases Vitamin B6 Celiac disease Renal insufficiency Hepatolenticular Degeneration Crohn disease Malnutrition Clofibrate Bone diseases Lead poisoning Anemia Hemolytic

Etiologies of Splenic Venous Hypertension:A Review

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Journal of Clinical and Translational Hepatology 2024年第6期12卷 594-606页

作者： Jasmine Tidwell Bianca Thakkar george Y.wu Department of Medicine University of Connecticut Health CenterFarmingtonCTUSA Division of Gastroenterology-Hepatology University of Connecticut Health CenterFarmingtonCTUSA

Splenic venous hypertension or left-sided portal hypertension is a rare condition caused by an obstruction of the splenic ***,it presents with upper gastrointestinal bleeding in the absence of liver *** can be classified based on the mechanism of development of splenic vein hypertension:compression,stenosis,inflammation,thrombosis,and surgically decreased splenic venous *** is established by various imaging modalities and should be suspected in patients with gastric varices in the absence of esophageal varices,splenomegaly,or *** management and prognosis vary depending on the underlying etiology but generally involve reducing splenic venous *** aim of this review was to summarize the etiologies of splenic venous hypertension according to the mechanism of development.

关键词： Splenic venous hypertension Left-sided portal hypertension Sinistral portal hypertension Splenic vein thrombosis Gastric varices Hematemesis Upper gastrointestinal bleeding

Effects of Transjugular Intrahepatic Portosystemic Shunt on Renal and Pulmonary Function in Hepatic Decompensation with and without Hepatorenal and Hepatopulmonary Syndromes:A Review

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Journal of Clinical and Translational Hepatology 2024年第9期12卷 780-791页

作者： Ahmed H.Abdelwahed Moataz Aboeldahb george Y.wu Department of Medicine Division of Gastroenterology-HepatologyUniversity of Connecticut Health CenterFarmingtonCTUSA Department of Gastroenterology and Hepatology Mayo ClinicRochesterMNUSA

Cirrhosis is often characterized by decreased liver function,ranging from a compensated,typically asymptomatic phase to a decompensated phase characterized by the appearance of ascites or variceal bleeding,and ultimately hepatorenal syndrome(HRS)or hepatopulmonary syndrome(HPS).The latter two complications are associated with a poor prognosis and limited treatment *** cases of ascites or variceal bleeding resistant to medical therapy,transjugular intrahepatic portosystemic shunt(TIPS)is effective and *** blood by TIPS diverts portal blood to the systemic circulation,potentially increasing systemic blood volume and benefiting renal ***,TIPS could also divert nitric oxide to the systemic circulation,potentially worsening systemic hypotension and perfusion,which could be detrimental to renal *** evidence indicates that TIPS often improves renal function in patients with portal hypertension,with or without *** studies have shown persistently decreased renal function after ***,these data are insufficient to support a recommendation for the use of TIPS specifically for *** patients without pre-existing HPS,TIPS does not appear to significantly affect pulmonary gas *** of TIPS in HPS have been inconsistent;some studies have shown improvement,but effects were *** studies have shown a persistent decline in pulmonary function after *** evidence supports the need for large randomized controlled trials to investigate the beneficial effects of TIPS for *** pulmonary function data are less clear regarding TIPS for *** aim of the current report was to review the literature regarding the effects of TIPS on renal and pulmonary function in hepatic decompensation,with or without the development of HRS or HPS.

关键词： Cirrhosis Hepatorenal syndrome Hepatopulmonary syndrome Transjugular intrahepatic portosystemic shunt

Genetics of Gallstone Disease and Their Clinical Significance: A Narrative Review

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Journal of Clinical and Translational Hepatology 2024年第3期12卷 316-326页

作者： Christopher J.Costa Minh Thu T.Nguyen Haleh Vaziri george Y.wu Department of Medicine University of Connecticut Health CenterFarmingtonCTUSA Division of Gastroenterology and Hepatology University of Connecticut Health CenterFarmingtonCTUSA

Gallstone(GS)disease is common and arises from a combination of genetic and environmental *** genetic abnormalities specifically leading to cholesterol GSs are rare,there are clinically significant gene variants associated with cholesterol *** contrast,most bilirubin GSs can be attributed to genetic *** pathogenesis of cholesterol and bilirubin GSs differs *** GSs are notably influenced by genetic variants within the ABC protein superfamily,including ABCG8,ABCG5,ABCB4,and ABCB11,as well as genes from the apolipoprotein family such as ApoB100 and ApoE(especially the E3/E3 and E3/E4 variants),and members of the MUC ***,bilirubin GSs are associated with genetic variants in highly expressed hepatic genes,notably UGT1A1,ABCC2(MRP2),ABCC3(MRP3),CFTR,and MUC,alongside genetic defects linked to hemolytic anemias and conditions impacting *** genetic cases constitute a small portion of GS disease,recognizing genetic predisposition is essential for proper diagnosis,treatment,and genetic counseling.

关键词： Gallstones Cholelithiasis ATP-binding cassette transporters ABCG8 protein Human UDP-glucuronosyltransferase A1

Pathogenesis of Thrombocytopenia in Chronic HCV Infection:A Review

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Journal of Clinical and Translational Hepatology 2020年第2期8卷 184-191页

作者： Sarah Rawi george Y wu Department of Medicine Division of Gastroenterology-HepatologyUniversity of Connecticut Health CenterFarmingtonCTUSA

A large proportion of patients with chronic hepatitis C have associated thrombocytopenia (TCP). Due to bleeding risks, TCP, when severe, can limit diagnostic and therapeutic procedures, treatments, and increases risk of complications, especially excessive bleeding. It is important to understand the mechanisms that cause TCP in order to manage it. In general, TCP can be due to increased destruction or decreased production. Proposed mechanisms of increased destruction include autoantibodies to platelets and hyper-splenism with sequestration. Proposed mechanisms of de-creased production include virus-induced bone marrow suppression and decreased TPO production. Autoantibodies directed against platelet surface antigens have demonstrated an inverse correlation with platelet counts. Hypersplenism with sequestration involves the interaction of portal hyper-tension, splenomegaly, and platelet destruction. Decreased production mechanisms involve appropriate and inappropri-ate levels of TPO secretion. There is limited evidence to support viral-induced bone marrow suppression. In contrast, there is strong evidence to support low levels of TPO in liver failure as a major cause of TCP. TPO-agonists, specifically eltrombopag, have been shown in hepatitis C patients to increase platelet counts without reducing portal hypertension or splenomegaly. We conclude that TCP in hepatitis C virus-induced liver disease is often multifactorial, but an under-standing of the mechanisms can lead to judicious use of new drugs for treatment.

关键词： Thrombocytopenia Chronic hepatitis Autoantibodies Hypersplenism Thrombopoietin

Hepatitis C and HIV co-infection:a review

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World Journal of Gastroenterology 2002年第4期8卷 577-579页

作者： Irena Maier george Y. wu Department of Medicine Division of Gastroenteroiogy-Hepatology University of Connecticut Health CenterFarmingtonCTUSA Department of Medicine Division of Gastroenterology-Hepatology University of Connecticut Health CenterFarmingtonCTUSA

Co-infection with hepatitis C virus and humanimmunodeficiency virus is common in certainpopulations. Among HCV(+) persons, 10 % are alsoHIV (+), and among HIV (+) persons, 25 % are alsoHCV(+). Many studies have shown that in intravenousdrug users, co-infection prevalence can be as high as90-95 %. There is increasing evidence supporting theconcept that people infected with HIV have a muchmore rapid course of their hepatitis C *** of co-infection is often challenging becausehighly active anti-retroviral therapy (HAART) therapyis frequently hepatotoxic, especially in the presence ofHCV. The purpose of this review is to describe theeffects that HIV has on hepatitis C, the effects thathepatitis C has on HIV, and the treatment options inthis challenging population.

关键词： Antiretroviral Therapy, Highly Active Antiviral Agents Female HIV Infections Hepatitis C Humans Immunity, Cellular Interferons Male Ribavirin

HEV and HBV Dual Infection:A Review

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Journal of Clinical and Translational Hepatology 2020年第3期8卷 313-321页

作者： Myra Nasir george Y.wu Department of Medicine Division of Gastroenterology-HepatologyUniversity of Connecticut Health CenterFarmingtonCTUSA

Hepatitis E virus(HEV)is a global health problem,affecting about 20 million people *** is significant overlap of hepatitis B virus(HBV)and HEV endemicity in many Asian countries where dual infections with HEV and HBV can *** the clinical course of HEV is largely self-limited,HEV superinfection in patients with chronic hepatitis B(CHB)can result in acute exacerbation of underlying *** superinfection in patients with CHB-related cirrhosis has been identified as a risk factor for decompensated cirrhosis and an independent predictor of *** acute HEV infection in pregnancy can cause fulminant liver failure,the few studies on pregnant patients with dual HBV and HEV infection have shown a subclinical *** is a risk factor for the development of chronic HEV infection,which can be managed by decreasing the dose of immune-suppressants and administering *** for HEV has been developed and is in use in China but its efficacy in patients with CHB has yet to be established in the *** this review,we appraise studies on dual infection with HEV and HBV,including the effect of HEV superinfection and coinfection in CHB,management strategies used and the role of active vaccination in the prevention of HEV.

关键词： Hepatitis B Hepatitis E Coinfection Superinfection Chronic hepatitis B