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Atypical causes of cholestasis

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World Journal of Gastroenterology 2014年第28期20卷 9418-9426页

作者： Ken D Nguyen vinay sundaram Walid S Ayoub Department of Internal Medicine Cedars Sinai Medical CenterLos AngelesCA 90048United States Department of Gastroenterology and Hepatology Multi Organ Transplant CenterCedars Sinai Medical CenterLos AngelesCA 90048United States

Cholestatic liver disease consists of a variety of disorders. Primary sclerosing cholangitis and primary biliary cirrhosis are the most commonly recognized cholestatic liver disease in the adult population, while biliary atresia and Alagille syndrome are commonly recognized in the pediatric population. In infants, the causes are usually congenital or inherited. Even though jaundice is a hallmark of cholestasis, it is not always seen in adult patients with chronic liver disease. Patients can have “silent” progressive cholestatic liver disease for years prior to development of symptoms such as jaundice and pruritus. In this review, we will discuss some of the atypical causes of cholestatic liver disease such as benign recurrent intrahepatic cholestasis, progressive familial intrahepatic cholestasis, Alagille Syndrome, biliary atresia, total parenteral nutrition induced cholestasis and cholestasis secondary to drug induced liver injury.

关键词： Cholestasis Benign recurrent intrahepatic cholestasis Progressive familial intrahepatic cholestasis Alagille syndrome Biliary atresia Total parenteral nutrition Drug induced liver injury

Evolution of liver transplant organ allocation policy: Current limitations and future directions

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World Journal of Hepatology 2021年第8期13卷 830-839页

作者： Alexander Polyak Alexander Kuo vinay sundaram Department of Medicine Cedars-Sinai Medical CenterLos AngelesCA 90048United States

Since the adoption of the model for end-stage liver disease(MELD)score for organ allocation in 2002,numerous changes to the system of liver allocation and distribution have been made with the goal of decreasing waitlist mortality and minimizing geographic variability in median MELD score at time of transplant without worsening post-transplant *** changes include the creation and adoption of the MELD-Na score for allocation,Regional Share 15,Regional Share for Status 1,Regional Share 35/National Share 15,and,most recently,the Acuity Circles Distribution ***,geographic differences in median MELD at time of transplant remain as well as limits to the MELD score for allocation,as etiology of liver disease and need for transplant ***-onchronic liver failure(ACLF)is a subset of liver failure where prevalence is rising and has been shown to have an increased mortality rate and need for transplantation that is under-demonstrated by the MELD *** underscores the limitations of the MELD score and raises the question of whether MELD is the most accurate,objective allocation *** to the MELD score have been proposed and studied,however MELD score remains as the current system used for *** review highlights policy changes since the adoption of the MELD score,addresses limitations of the MELD score,reviews proposed alternatives to MELD,and examines the specific implications of these changes and alternatives for ACLF.

关键词： Model for end-stage liver disease score Acute-on-chronic liver failure Regional sharing

Severe cholestatic hepatitis due to large vessel vasculitis:report of two cases

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Gastroenterology Report 2018年第1期6卷 68-71,I0003页

作者： Jason Xu Einar S.Bjo¨rnsson vinay sundaram Department of Medicine Cedars-Sinai Medical CenterLos AngelesCAUSA Department of Gastroenterology and Hepatology The National University Hospital of IcelandReykjavikIceland Faculty of Medicine University of IcelandReykjavikIceland Division of Gastroenterology and Hepatology and Comprehensive Transplant Center Cedars-Sinai Medical CenterLos AngelesCAUSA

Giant cell arteritis(GCA)is a vasculitis of medium and large sized vessels that occurs most often in people>50 years of age with associated symptoms of fever,weight loss,headache and jaw *** rheumatica(PMR),which is characterized by aching and stiffness in the shoulders,hip girdle,neck and torso,is intimately associated with GCA,and evidence suggests that GCA and PMR are two phases of the same *** occurrence of liver enzyme abnormalities in either of these conditions is ***,as these conditions occur most commonly in the elderly population who may be subject to polypharmacy,patients with elevated aminotransferases due to underlying GCA/PMR may mistakenly have their abnormal liver function tests attributed to drug-induced liver *** the potential complications of these diseases if left untreated,including ischemic stroke and blindness,early recognition and treatment are *** report two patients who developed severe cholestatic liver enzyme elevation,which had been initially attributed to drug toxicity,but was ultimately caused by large vessel vasculitis,specifically GCA and PMR.

关键词： acute liver injury polymyalgia rheumatica abnormal liver enzymes

A Case of mild idiopathic adulthood ductopenia and brief review of literature

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Gastroenterology Report 2015年第2期3卷 167-169页

作者： Aung Kaung vinay sundaram Deepti Dhall Tram T.Tran Department of Medicine Cedars-Sinai Medical CenterLos AngelesCAUSA Center for Liver Disease and Transplantation Cedars-Sinai Medical CenterLos AngelesCAUSA Department of Pathology Cedars-Sinai Medical CenterLos AngelesCAUSA

Mild idiopathic adulthood ductopenia(IAD)is a rare cholestatic disease of unknown cause and characterized by interlobular bile duct loss in less than 50%of the portal *** describe the case of a middLe-aged male who presented with persistent elevation of transaminases and alkaline *** had a normal biliary tree on endoscopic retrograde cholangiopathy and negative anti-mitochondrial *** liver biopsy specimen showed chronic biliary disease,duct loss in 4 out of 15 portal tracts and prominent *** on the work-up,he likely had mild *** transplantation would be necessary if his disease becomes progressive.

关键词： idiopathic adulthood ductopenia idiopathic cholestasis primary biliary cirrhosis small-duct primary sclerosing cholangitis

Hagen-Poiseuille's law:The link between cirrhosis,liver stiffness,portal hypertension and hepatic decompensation

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World Journal of Hepatology 2015年第1期7卷 28-32页

作者： Gerond Lake-Bakaar Muneeb Ahmed Amy Evenson Alan Bonder Salomao Faintuch vinay sundaram Liver Tumor Center Department of MedicineBeth Israel Deaconess Medical CenterHarvard Medical SchoolBostonMA 02215United States

The onset of hepatic decompensation in cirrhosis heralds an accelerated downhill course with poor outcome. The sole predictor of this decompensationin cirrhosis is increased hepatic vein to portal vein gradient hepatic venous pressure gradient(HVPG). Surrogate markers of liver function or hepatic reserve appear to be less relevant. The hepatic sinusoids become less elastic and more rigid as liver fibrosis and cirrhosis progress. We propose that the HagenPoiseuille's law,which applies to rigid,but not elastic vessels,determines the pressure-flow characteristics in the sinusoids. In the rigid cirrhotic liver,HVPG rises dramatically with any change in net surface area or radius,r4 of the vasculature that follows surgical resection. This review relates liver stiffness to the risk of decompensation in patients with cirrhosis. The liver has a unique dual blood supply comprising a low pressure portal vein and high pressure hepatic artery. We compare the complexity of autoregulation in the normal elastic liver with that in the rigid cirrhotic liver. Therapeutic modalities to reduce portal pressure may reduce the risk of hepatic decompensation and improve outcomes in cirrhosis.

关键词： Portal hypertension Liver stiffness Hagen-Poiseuille's law

Management of refractory ascites in cirrhosis:Are we out of date?

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World Journal of Hepatology 2016年第28期8卷 1182-1193页

作者： Alagappan Annamalai Lauren Wisdom Megan Herada Mazen Nourredin Walid Ayoub vinay sundaram Andrew Klein Nicholas Nissen Comprehensive Transplant Center Cedars Sinai Medical Center

Cirrhosis is a major cause of morbidity and mortality worldwide with liver transplantations as it only possible *** the face of a significant organ shortage many patients die waiting.A major complication of cirrhosis is the development of portal hypertension and *** management of ascites has barely evolved over the last hundred years and includes only a few milestones in our treatment approach,but has overall significantly improved patient morbidity and *** mainstay to ascites management includes changes in diet,diuretics,shunt procedures,and large volume *** understanding of the pathophysiology of cirrhosis and portal hypertension has significantly improved in the last couple of decades but the changes in ascites management have not seemed to mirror this newer *** herein review the history of ascites management and discuss some its current limitations.

关键词： Portal hypertension Cirrhosis Ascites Transhepatic portosystemic shunts Paracentesis

Risk factors and outcomes associated with alcohol relapse after liver transplantation

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World Journal of Hepatology 2017年第17期9卷 771-780页

作者： Jane Lim Michael P Curry vinay sundaram Department of Medicine Cedars Sinai Medical Center Department of Medicine Beth Israel Deaconess Medical Center Division of Gastroenterology and Compre-hensive Transplant Center Cedars--Sinai Medical Center

Alcoholic liver disease(ALD) is the second most common indication for liver transplantation(LT) in the United States and Europe. Unlike other indications for LT, transplantation for ALD may be controversial due to the concern for alcohol relapse and non-compliance after LT. However, the overall survival in patients transplanted for ALD is comparable or higher than in patients transplanted for other etiologies of liver disease. While the rate of alcohol use after liver transplantation does not differ among various etiologies of liver disease, alcohol relapse after transplantation for ALD has been associated with complications such as graft rejection, graft loss, recurrent alcoholic cirrhosis and reduced long-term patient survival. Given these potential complications, our review aimed to discuss risk factors associated with alcohol relapse and the efficacy of various interventions attempted to reduce the risk of alcohol relapse. We also describe the impact of alcohol relapse on post-transplant outcomes including graft and patient survival. Overall, alcohol liver disease remains an appropriate indication for liver transplantation, and long-term mortality in this group of patients is primarily attributed to cardiovascular disease or de novo malignancies rather than alcohol related hepatic complications, among those who relapse.

关键词： Cirrhosis Relapse prevention Recidivism