1,127 results match your criteria Clinics in Liver Disease [Journal]


The Liver in Systemic Disease.

Authors:
Jorge L Herrera

Clin Liver Dis 2019 May 22;23(2):xiii-xiv. Epub 2019 Feb 22.

Division of Gastroenterology, University of South Alabama College of Medicine, Mobile, AL 36688, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cld.2019.02.001DOI Listing
May 2019
1 Read

Obstructive Sleep Apnea and the Liver.

Clin Liver Dis 2019 May;23(2):363-382

Department of Gastroenterology and Hepatology, Digestive Disease and Surgery Institute, Cleveland Clinic Foundation, 9500 Euclid Ave, M2 Annex, Cleveland, OH 44114, USA; Department of Inflammation and Immunity, Cleveland Clinic Lerner College of Medicine, Case Western University, Cleveland, OH 44195, USA. Electronic address:

Nonalcoholic fatty liver disease (NAFLD), a disorder of altered metabolic pathways, is increasing worldwide. Recent studies established obstructive sleep apnea (OSA) and chronic intermittent hypoxia (CIH) as NAFLD risk factors. Studies have ascertained that CIH is independently related to NAFLD. Read More

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http://dx.doi.org/10.1016/j.cld.2019.01.001DOI Listing
May 2019
2 Reads

Liver Diseases During Pregnancy.

Clin Liver Dis 2019 May 26;23(2):345-361. Epub 2019 Feb 26.

Section of Hepatology, Division of Digestive Diseases, Department of Internal Medicine, Rush University Medical Center, 1725 West Harrison Street, Suite 319, Chicago, IL 60612, USA. Electronic address:

Liver diseases during pregnancy pose a unique clinical challenge because they can affect the lives of both the mother and unborn child. Although severe liver disease is rare, pregnancy-related liver disease affects approximately 3% of pregnancies and can be fatal. Timely recognition and diagnosis are essential in order to institute appropriate management strategies. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.013DOI Listing
May 2019
6 Reads

Sarcoidosis and the Liver.

Clin Liver Dis 2019 May 2;23(2):331-343. Epub 2019 Mar 2.

Division of Gastroenterology, University of South Alabama College of Medicine, UCOM 6000, 75 University Boulevard South, Mobile, AL 36688, USA. Electronic address:

Hepatic granulomas are a common finding in systemic sarcoidosis, but most patients remain asymptomatic. Elevated alkaline phosphatase is the most common sign of hepatic sarcoidosis (HS). Lacking a specific diagnostic test, the diagnosis of HS is one of exclusion. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.012DOI Listing
May 2019
1 Read

Liver Disease in Human Immunodeficiency Virus Infection.

Clin Liver Dis 2019 May 5;23(2):309-329. Epub 2019 Mar 5.

Icahn School of Medicine at Mount Sinai, 1 Gustav L. Levy Place, New York, NY 10029-6574, USA.

Liver disease in human immunodeficiency virus (HIV) remains a main cause of morbidity and mortality. Liver-related morbidity and mortality can be caused by multiple etiologic factors, including opportunistic infections, direct and indirect effects of antiretrovirals, direct and indirect effects of HIV, and viral hepatitides. These factors present with varied liver pathophysiologic mechanisms that lead to abnormalities in liver enzymes and synthetic function test, followed by distinct clinical presentations. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.011DOI Listing
May 2019
2 Reads

Hepatic Manifestations of Lymphoproliferative Disorders.

Clin Liver Dis 2019 May 2;23(2):293-308. Epub 2019 Mar 2.

Division of Gastroenterology and Hepatology, Department of Medicine, University of Pennsylvania, Philadelphia, PA 19104, USA. Electronic address:

Hepatic abnormalities in patients with lymphoproliferative disorders are common and can occur from direct infiltration by abnormal cells, bile duct obstruction, paraneoplastic syndrome, hemophagocytic syndrome, drug-induced liver injury, opportunistic infections, and reactivation of viral hepatitis. Hepatic involvement by lymphoma is often in association with systemic disease and rarely seen as a primary hepatic lymphoma. Vanishing bile duct syndrome is a well-known complication of Hodgkin disease. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.010DOI Listing
May 2019
1 Read

Intestinal Failure-Associated Liver Disease.

Clin Liver Dis 2019 May 21;23(2):279-291. Epub 2019 Feb 21.

Department of Medical and Surgical Science, Centre for Chronic Intestinal Failure, University of Bologna, St. Orsola-Malpighi Hospital, Via Massarenti, 9, 40138 Bologna, Italy.

Intestinal failure-associated liver disease (IFALD) is characterized by either liver steatosis or cholestasis and may develop in patients on long-term home parenteral nutrition for chronic intestinal failure. The pathogenesis of IFALD is multifactorial and includes gastrointestinal disease-related, parenteral nutrition-related, and systemic-related factors. Alteration of bile acid enterohepatic circulation, gut microbiome, and intestinal permeability, seem to be the main mechanisms. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.009DOI Listing
May 2019
3 Reads

Hepatic Manifestations of Cystic Fibrosis.

Clin Liver Dis 2019 May 21;23(2):263-277. Epub 2019 Feb 21.

Liver Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, 10 Center Drive, Building 10, Room 9B-16, MSC 1800, Bethesda, MD 20892, USA. Electronic address:

Cystic fibrosis liver disease (CFLD) remains the third leading cause of death in patients with cystic fibrosis. Although most patients with CFLD present in childhood, recent studies suggest a second wave of liver disease in adulthood. There are no clear guidelines for diagnosing CFLD. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.008DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6452885PMC
May 2019
2 Reads

Rheumatologic Diseases and the Liver.

Clin Liver Dis 2019 May 8;23(2):247-261. Epub 2019 Mar 8.

Division of Gastroenterology and Hepatology, Department of Internal Medicine, Howard University Hospital and College of Medicine, 2041 Georgia Avenue Northwest, Suite 5C02, Washington, DC 20060, USA. Electronic address:

A variety of rheumatologic disorders may affect the liver. There is a significant epidemiologic, genetic, and immunologic overlap between immune-mediated rheumatologic disorders and autoimmune liver diseases. There is an increased frequency of autoimmune liver diseases, such as primary biliary cholangitis, autoimmune hepatitis, primary sclerosing cholangitis, or overlap syndrome, in patients with systemic lupus erythematosus, rheumatoid arthritis, Sjögren syndrome, systemic sclerosis, vasculitis, and other immune-related diseases. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.007DOI Listing
May 2019
1 Read

Endocrine Diseases and the Liver: An Update.

Clin Liver Dis 2019 May 8;23(2):233-246. Epub 2019 Mar 8.

Department of Medicine, University of Florida Health, 4555 Emerson Street, Suite 300, Jacksonville, FL 32207, USA.

The endocrine system is a complex interconnected system of organs that control corporeal processes and function. Primary endocrine organs are involved in hormonal production and secretion but rely on a bevy of signals from the hypothalamic-pituitary axis and secondary endocrine organs, such as the liver. In turn, proper hepatic function is maintained through hormonal signaling. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.006DOI Listing
May 2019
3 Reads

Hepatobiliary Complications in Critically Ill Patients.

Clin Liver Dis 2019 May 8;23(2):221-232. Epub 2019 Mar 8.

Division of Gastroenterology and Hepatology, Northwestern Feinberg School of Medicine, 19-046 Arkes Building, 676 North Saint Clair, Chicago, IL 60611, USA.

Critically ill patients frequently present with the systemic inflammatory response syndrome, which is largely a reflection of the liver's response to injury. Underlying hepatic congestion is a major risk factor for hypoxic liver injury, the most common cause for hepatocellular injury. Cholestatic liver injury often occurs in critically ill patients due to inhibition of farnesoid X receptor (FXR), the main regulator of bile acid handling, particularly in the liver and intestines. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.005DOI Listing

The Liver in Circulatory Disturbances.

Clin Liver Dis 2019 May 8;23(2):209-220. Epub 2019 Mar 8.

Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street Southwest, Rochester, MN 55905, USA. Electronic address:

Liver diseases frequently coexist with heart disease. The causes of coexistent heart and liver disease are categorized into four groups: (1) heart disease affecting the liver, (2) liver disease affecting the heart, (3) cardiac and hepatic manifestations of a common cause, and (4) coexistent heart and liver disease with distinct causes. Discerning the cause of cardiac and liver dysfunction is important in the management of these conditions, particularly when considering surgical intervention or heart or liver transplantation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10893261183010
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http://dx.doi.org/10.1016/j.cld.2018.12.004DOI Listing
May 2019
6 Reads

Hepatic Complications of Inflammatory Bowel Disease.

Clin Liver Dis 2019 May 26;23(2):191-208. Epub 2019 Feb 26.

Division of Gastroenterology and Hepatology, University of Miami Miller School of Medicine, 1120 Northwest 14 Street #1115, Miami, FL 33136, USA.

Hepatobiliary disorders are commonly encountered in patients with inflammatory bowel disease (IBD). Although primary sclerosing cholangitis is the stereotypical hepatobiliary disorder associated with IBD, other diseases, including autoimmune hepatitis and nonalcoholic fatty liver disease, also are encountered in this population. Several agents used for treatment of IBD may cause drug-induced liver injury, although severe hepatotoxicity occurs infrequently. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.003DOI Listing
May 2019
1 Read

The Liver in Sickle Cell Disease.

Clin Liver Dis 2019 May 21;23(2):177-189. Epub 2019 Feb 21.

Institute of Liver Studies, King's College Hospital NHS Foundation Trust, Denmark Hill, London SE5 9RS, UK. Electronic address:

Patients with sickle cell disease can develop liver disease as a result of intrahepatic sickling of erythrocytes, viral hepatitis and iron overload secondary to multiple blood transfusions, and gallstone disease as a result of chronic hemolysis. The spectrum of clinical liver disease is wide and often multifactorial. Some patients develop cirrhosis that may progress to end-stage liver failure. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.002DOI Listing
May 2019
3 Reads

The Liver and Celiac Disease.

Clin Liver Dis 2019 May 1;23(2):167-176. Epub 2019 Mar 1.

Division of Gastroenterology and Hepatology, Department of Medicine, Mayo Clinic College of Medicine, 200 First Street Southwest, Rochester, MN 55905, USA. Electronic address:

Celiac disease is a multisystem disorder. Celiac hepatitis characterized by gluten-responsive mild elevation of transaminases is the more common liver manifestation of celiac disease. Celiac disease may also be associated or coexist with other chronic liver disorders. Read More

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http://dx.doi.org/10.1016/j.cld.2018.12.001DOI Listing
May 2019
2 Reads

Alcohol and Alcoholic Liver Disease.

Clin Liver Dis 2019 02;23(1):xiii-xiv

Medicine and Surgery, University of Wisconsin-Madison School of Medicine and Public Health, 600 Highland Avenue, Madison, WI 53792, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cld.2018.10.001DOI Listing
February 2019
2 Reads

Nutrition in Alcoholic Liver Disease: An Update.

Clin Liver Dis 2019 02;23(1):99-114

Section of Gastroenterology and Hepatology, Michael E. DeBakey VA Medical Center, Baylor College of Medicine, Houston, TX 77030, USA; Center for Innovations in Quality, Effectiveness and Safety (IQuESt), Michael E. DeBakey VA Medical Center, Houston, TX 77030, USA; Section of Health Services Research, Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA.

Malnutrition is a change in body composition owing to disordered nutrition associated with a decrease in function and poor clinical outcomes. Malnutrition can result from overnutrition, undernutrition and inflammatory activity. Patients with alcoholic liver disease are at increased risk for malnutrition. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10893261183008
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http://dx.doi.org/10.1016/j.cld.2018.09.012DOI Listing
February 2019
14 Reads

Acute Alcoholic Hepatitis.

Authors:
Gene Y Im

Clin Liver Dis 2019 02 26;23(1):81-98. Epub 2018 Oct 26.

Division of Liver Diseases, Icahn School of Medicine at Mount Sinai, Recanati-Miller Transplantation Institute, One Gustave Levy Place, Box 1104, New York, NY 10029, USA. Electronic address:

Alcoholic hepatitis is a unique type of alcohol-associated liver disease characterized by acute liver inflammation caused by prolonged heavy alcohol use. Treatment is mostly supportive. The short-term prognosis of acute alcoholic hepatitis depends on liver recovery, and ranges widely from rapid improvement to grim multiorgan failure despite treatment. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.005DOI Listing
February 2019
17 Reads

Pathogenesis of Alcoholic Liver Disease: An Update.

Clin Liver Dis 2019 02;23(1):71-80

Department of Surgery, Division of Abdominal Transplantation, Baylor College of Medicine, 6620 Main Street, Suite 1450, Houston, TX 77030, USA. Electronic address:

Apart from the classic knowledge that ethanol mediates its hepatotoxicity through its metabolism to acetaldehyde, a well-known hepatotoxic molecule, recent research has elucidated several key mechanisms that potentiate ethanol's damage to the liver parenchyma, such as generation of free radicals, activation of Kupffer cells, and alterations to the human bacterial and fungal microbiome. Genetic studies have suggested the role of PNPLA3 and TM6SF2 gene mutations in the progression of alcoholic liver disease. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.006DOI Listing
February 2019
6 Reads

Alcohol Use Disorders in Alcoholic Liver Disease.

Clin Liver Dis 2019 02;23(1):55-69

Department of Psychiatry, Michigan Medicine, 1500 East Medical Center Drive, SPC 5118, Ann Arbor, MI 48109, USA.

Alcohol use disorder (AUD) is common in alcoholic liver disease (ALD) and intrinsic to its pathophysiology. Optimal treatment requires a multidisciplinary team approach and a working alliance between patients and providers. Diagnosing AUD involves a combination of thorough history taking, physical examination, screening questionnaires, and alcohol biomarkers. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.004DOI Listing
February 2019
15 Reads

Adolescent Alcoholic Liver Disease.

Clin Liver Dis 2019 02 26;23(1):51-54. Epub 2018 Oct 26.

Department of Pediatrics, Texas Children's Hospital, 6701 Fannin Street, Houston, TX 77030, USA. Electronic address:

Alcohol use is common during adolescence. Adolescent alcohol use is a global problem. The risk of alcohol dependence increases based on genetic and psychosocial factors. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.003DOI Listing
February 2019
2 Reads

Epidemiology of Alcohol Consumption and Societal Burden of Alcoholism and Alcoholic Liver Disease.

Clin Liver Dis 2019 02;23(1):39-50

Division of Gastroenterology and Hepatology, Porphyria Center, University of Alabama at Birmingham, 1720 2nd Avenue South, BDB 380, Birmingham, AL 35294, USA. Electronic address:

Alcohol abuse is a major determinant of public health outcomes. Worldwide data from 2016 indicate that alcohol is the seventh leading risk factor in terms of disability-adjusted life years, an increase of more than 25% from 1990 to 2016. Understanding the epidemiology of alcoholic liver disease, including the regional variations in consumption and public policy, is an area of active research. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10893261183008
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http://dx.doi.org/10.1016/j.cld.2018.09.011DOI Listing
February 2019
14 Reads

Alcohol and the Law.

Clin Liver Dis 2019 02;23(1):25-38

Section of Gastroenterology, Department of Medicine, Baylor College of Medicine, 1 Baylor Plaza, Houston, TX 77030, USA.

In the intersection of alcohol ingestion with the law, medical ethics, and public safety, physicians are often unsure about how to proceed. Physicians' primary focus should be on patient education with an ethical and legal duty to warn the patient of the adverse effects of alcohol. Warning third parties of potential harm related to alcohol-related impairment may involve a breach of patient confidentiality; therefore it should only be undertaken after careful analysis suggests that the risk for significant harm exceeds the burden that results to the patient from warning others. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10893261183007
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http://dx.doi.org/10.1016/j.cld.2018.09.002DOI Listing
February 2019
16 Reads

Will Studies in Nonalcoholic Steatohepatitis Help Manage Alcoholic Steatohepatitis?

Clin Liver Dis 2019 02;23(1):157-165

Gastroenterology Section, VA Long Beach Healthcare System, 5901 East Seventh Street - 11G, Long Beach, CA 90822, USA. Electronic address:

Hepatic steatosis and steatohepatitis have several etiologies; the most common are alcoholic steatohepatitis (ASH) and obesity/metabolic syndrome-induced steatohepatitis, also known as nonalcoholic steatohepatitis (NASH). Although the etiology of these 2 conditions is different, they share pathways to disease progression and severity. They also have differences in physiologic pathways, and shared and divergent mechanisms can be therapeutic targets. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10893261183008
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http://dx.doi.org/10.1016/j.cld.2018.09.008DOI Listing
February 2019
14 Reads

Chronic Neurologic Effects of Alcohol.

Clin Liver Dis 2019 02;23(1):141-155

Department of Neurology, Parkinson's Disease Center and Movement Disorders Clinic, Baylor College of Medicine, 7200 Cambridge Street, 9th Floor, MS:BCM609, Houston, TX 77030, USA. Electronic address:

Chronic alcohol use induces silent changes in the structure and function of the central and peripheral nervous systems that eventually result in irreversible, debilitating repercussions. Once identified, nutritional supplementation and cessation measures are critical in preventing further neurologic damage. The proposed mechanisms of neuronal injury in chronic alcohol abuse include direct toxic effects of alcohol and indirect effects, including those resulting from hepatic dysfunction, nutritional deficiencies, and neuroinflammation. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.010DOI Listing
February 2019
3 Reads

Liver Transplantation for Alcoholic Liver Disease: An Update.

Clin Liver Dis 2019 02;23(1):127-139

6620 Main Street, Suite 1425, Houston, TX 77030, USA.

Alcoholic liver disease is a serious and increasing contributor to the global liver disease burden. Extensive selection criteria, including a minimum abstinence period, has previously been used to secure good outcomes but new research questions the effectiveness of abstinence periods and has recommended changes in integrated alcohol use treatment to effectively prevent relapse. Patients have unique health concerns, including posttransplantation risks of malignancy and metabolic complications, but overall very good long-term outcomes. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.007DOI Listing
February 2019
17 Reads

Alcohol-Associated Cirrhosis.

Authors:
Michael R Lucey

Clin Liver Dis 2019 02;23(1):115-126

Division of Gastroenterology and Hepatology, University of Wisconsin-Madison School of Medicine and Public Health, 1685 Highland Avenue Suite 4000, Madison, WI 53705-2281, USA. Electronic address:

Alcohol-associated cirrhosis (AC) contributes up to 50% of the overall cirrhosis burden in the United States. AC is typically a comorbid condition in association with alcohol-use disorder. AC is often coexistent with other conditions. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.013DOI Listing
February 2019
21 Reads

Histopathology of Alcohol-Related Liver Diseases.

Clin Liver Dis 2019 02;23(1):11-23

Department of Pathology, The Department of Veteran Affairs New York Harbor Healthcare System, 800 Poly Place, Brooklyn, NY 11209, USA.

Excessive alcohol consumption can lead to a spectrum of liver histopathology, including steatosis, steatohepatitis, foamy degeneration, fatty liver with cholestasis, and cirrhosis. Although variability in sampling and pathologist interpretation are of some concern, liver biopsy remains the gold standard for distinguishing between steatohepatitis and noninflammatory histologic patterns of injury that can also cause the clinical syndrome of alcohol-related hepatitis. Liver biopsy is not routinely recommended to ascertain a diagnosis of alcohol-related liver disease in patients with an uncertain alcohol history, because the histologic features of alcohol-related liver diseases can be found in other diseases, including nonalcoholic steatohepatitis and drug-induced liver injury. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.001DOI Listing
February 2019
2 Reads

Introduction: Alcohol and Alcoholism.

Clin Liver Dis 2019 02;23(1):1-10

Division of Abdominal Transplantation, Baylor College of Medicine, 6620 Main Street, Suite 1450, Houston, TX 77030, USA. Electronic address:

This article discusses alcohol use throughout history. The discovery and cultivation of wine and beer and distillation of spirits are explored. The article spans prehistory, Egypt, Ancient Greece, Ancient Rome, Europe, and the Americas; and the religions Judaism, Christianity, and Islam. Read More

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http://dx.doi.org/10.1016/j.cld.2018.09.009DOI Listing
February 2019
2 Reads

Advances in Pediatric Hepatology.

Authors:
Philip Rosenthal

Clin Liver Dis 2018 11;22(4):xi

Division of Pediatric Gastroenterology, Hepatology, and Nutrition, University of California, San Francisco, 550 16th Street, Mailcode 0136, San Francisco, CA 94143, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cld.2018.08.001DOI Listing
November 2018
1 Read

Liver Transplantation in Children.

Clin Liver Dis 2018 11 22;22(4):807-821. Epub 2018 Aug 22.

Pediatric Gastroenterology, Hepatology, and Nutrition, Baylor College of Medicine, Texas Children's Hospital, 6701 Fannin Street, Houston, TX 77030, USA. Electronic address:

Liver transplantation (LT) for children has excellent short- and long-term patient and graft survival. LT is a lifesaving procedure in children with acute or chronic liver disease, hepatic tumors, and a few genetic metabolic diseases in which it can significantly improve quality of life. In this article, the authors discuss the unique aspects of pediatric LT, including the indications, patient selection and evaluation, allocation, transplant surgery and organ selection, posttransplant care, prognosis, adherence, and transition of care. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.004DOI Listing
November 2018
12 Reads

Acute Liver Failure: An Update.

Clin Liver Dis 2018 11 22;22(4):773-805. Epub 2018 Aug 22.

Department of Pediatric Gastroenterology and Hepatology, University of Pittsburgh School of Medicine, Children's Hospital of Pittsburgh, 4401 Penn Avenue, Pittsburgh, PA 15224, USA.

Pediatric acute liver failure (PALF) is a dynamic, life-threatening condition of disparate etiology. Management of PALF is dependent on intensive collaborative clinical care and support. Proper recognition and treatment of common complications of liver failure are critical to optimizing outcomes. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.009DOI Listing
November 2018
16 Reads

Pediatric Liver Tumors.

Clin Liver Dis 2018 11 24;22(4):753-772. Epub 2018 Aug 24.

Department of Pediatrics, Johns Hopkins School of Medicine, 600 North Wolfe Street, CMSC 2-117, Baltimore, MD 21287, USA. Electronic address:

Although liver tumors are rare in the pediatric population, they are common in the setting of children with specific risk factors requiring increased awareness and, in some instances, screening. The evaluation of a liver mass in children is largely driven by the age at diagnosis, the presence of any medical comorbidities, and initial testing with alpha fetoprotein and imaging. Specific guidelines for the management of different tumors have been implemented in recent years such that a multidisciplinary approach is ideal and care should be provided by centers with experience in their management. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.008DOI Listing
November 2018
1 Read

Cirrhosis and Portal Hypertension in the Pediatric Population.

Clin Liver Dis 2018 11 22;22(4):735-752. Epub 2018 Aug 22.

Division of Pediatric Gastroenterology, Hepatology and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, 225 East Chicago Avenue, Box #65, Chicago, IL 60611, USA. Electronic address:

Cirrhosis is a complex process in which the architecture of the liver is replaced by structurally abnormal nodules due to cirrhosis. Cirrhosis frequently leads to the development of portal hypertension. In children, portal hypertension may be caused by a wide range of etiologies, including extrahepatic portal vein obstruction, biliary atresia, alpha 1 antitrypsin deficiency, and autoimmune hepatitis. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.007DOI Listing
November 2018
19 Reads

Nonalcoholic Liver Disease in Children and Adolescents.

Clin Liver Dis 2018 11 22;22(4):723-733. Epub 2018 Aug 22.

Department of Pediatric Gastroenterology, Hepatology, and Nutrition, University of California, San Francisco, 550 16th Street, 5th Floor, Mail Code 0136, San Francisco, CA 94143, USA.

Pediatric nonalcoholic fatty liver disease (NAFLD) is the most common cause of liver disease in children. The spectrum of NAFLD ranges from steatosis to nonalcoholic steatohepatitis (NASH) to fibrosis. Obesity rates in children continue to rise and, as a result, NAFLD in children is becoming more prevalent. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10893261183006
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http://dx.doi.org/10.1016/j.cld.2018.07.001DOI Listing
November 2018
23 Reads

Hepatitis B and C.

Clin Liver Dis 2018 11 22;22(4):703-722. Epub 2018 Aug 22.

Division of Gastroenterology, Hepatology and Nutrition, Texas Children's Hospital, Department of Pediatrics, Baylor College of Medicine, 6701 Fannin, Suite 1010, Houston, TX 77030, USA. Electronic address:

Hepatitis B virus (HBV) and hepatitis C virus (HCV) infections represent a major global public health and economic burden, with an estimated 257 million and 71 million people, respectively, having chronic infection worldwide. The natural history of HBV and HCV in children depends on age at time of infection, mode of acquisition, ethnicity, and genotype. Most children infected perinatally or vertically remain asymptomatic but are at uniquely higher risk of developing chronic viral hepatitis, progressing to liver cirrhosis and hepatocellular carcinoma (HCC), hence classifying HBV and HCV as oncoviruses. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.002DOI Listing
November 2018
9 Reads

Autoimmune Hepatitis, Sclerosing Cholangitis, and Autoimmune Sclerosing Cholangitis or Overlap Syndrome.

Clin Liver Dis 2018 11;22(4):689-702

Division of Gastroenterology, Hepatology and Nutrition, Golisano Children's Hospital, University of Rochester Medical Center, 601 Elmwood Avenue, Box 667, Rochester, NY 14642, USA; Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Florida, PO Box 100296, Gainesville, FL 32610, USA.

Autoimmune hepatitis (AIH) is characterized by elevated serum aminotransferases, immunoglobulin G, autoantibodies, and interface hepatitis, in the absence of a known diagnosis. Presentation is varied. Therapy is with immunosuppression. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S10893261183005
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http://dx.doi.org/10.1016/j.cld.2018.06.005DOI Listing
November 2018
19 Reads

Inborn Errors of Bile Acid Metabolism.

Clin Liver Dis 2018 11 22;22(4):671-687. Epub 2018 Aug 22.

Division of Pediatric Gastroenterology, Hepatology and Nutrition, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 240 Sabin Way, Cincinnati, OH 45229, USA; Division of Pathology, Cincinnati Children's Hospital Medical Center, University of Cincinnati College of Medicine, 240 Sabin Way, Cincinnati, OH 45229, USA.

Inborn errors of bile acid metabolism are rare causes of neonatal cholestasis and liver disease in older children and adults. The diagnosis should be considered in the context of hyperbilirubinemia with normal serum bile acids and made by urinary liquid secondary ionization mass spectrometry or DNA testing. Cholic acid is an effective treatment of most single-enzyme defects and patients with Zellweger spectrum disorder with liver disease. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.006DOI Listing
November 2018
14 Reads

Progressive Familial Intrahepatic Cholestasis.

Clin Liver Dis 2018 11 3;22(4):657-669. Epub 2018 Aug 3.

Institute of Liver Studies, King's College London, King's College Hospital, Denmark Hill, London SE5 9RS, UK.

Genetic cholestasis has been dissected through genetic investigation. The major PFIC genes are now described. ATP8B1 encodes FIC1, ABCB11 encodes BSEP, ABCB4 encodes MDR3, TJP2 encodes TJP2, NR1H4 encodes FXR, and MYO5B encodes MYO5B. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.003DOI Listing
November 2018
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3.660 Impact Factor

Alpha-1-Antitrypsin Deficiency Liver Disease.

Clin Liver Dis 2018 11 22;22(4):643-655. Epub 2018 Aug 22.

Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, Saint Louis University School of Medicine, 1465 South Grand Boulevard, St Louis, MO 63104, USA; Department of Biochemistry and Molecular Biology, Saint Louis University School of Medicine, 1465 South Grand Boulevard, St Louis, MO 63104, USA. Electronic address:

In homozygous ZZ alpha-1-antitrypsin (AAT) deficiency, the liver synthesizes large quantities of AAT mutant Z, which folds improperly during biogenesis and is retained within the hepatocytes and directed into intracellular proteolysis pathways. These intracellular polymers trigger an injury cascade, which can lead to liver injury. This is highly variable and not all patients develop liver disease. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.010DOI Listing
November 2018
2 Reads

Alagille Syndrome.

Clin Liver Dis 2018 11 22;22(4):625-641. Epub 2018 Aug 22.

Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, 3401 Civic Center Boulevard, Philadelphia, PA 19104, USA; Department of Pediatrics, Perelman School of Medicine, University of Pennsylvania, 3400 Civic Center Boulevard, Philadelphia, PA 19104, USA. Electronic address:

Alagille syndrome is a complex multisystem autosomal dominant disorder with a wide variability in penetrance of clinical features. A majority of patients have pathogenic mutations in either the JAG1 gene, encoding a Notch pathway ligand, or the receptor NOTCH2. No genotype-phenotype correlations have been found in any organ system. Read More

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http://dx.doi.org/10.1016/j.cld.2018.06.001DOI Listing
November 2018
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Primary Biliary Cholangitis: A New Era.

Clin Liver Dis 2018 08 31;22(3):xiii-xiv. Epub 2018 Mar 31.

Division of Hepatology, University of Miami Miller School of Medicine, 1500 Northwest 12th Avenue, Suite 1101, Miami, FL 33136, USA. Electronic address:

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http://dx.doi.org/10.1016/j.cld.2018.03.012DOI Listing
August 2018
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Current Status of Liver Transplantation for Primary Biliary Cholangitis.

Clin Liver Dis 2018 08;22(3):613-624

Division of Gastroenterology and Hepatology, Mayo Clinic, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA. Electronic address:

Primary biliary cholangitis (PBC) is an autoimmune cholestatic liver disease diagnosed with elevated alkaline phosphatase in the presence of antimitochondrial antibody. With the introduction and widespread use of ursodeoxycholic acid the proportion of PBC patients undergoing liver transplant (LT) has decreased. However, up to 40% of patients are ursodeoxycholic acid nonresponders and require second-line treatment or progress to end-stage liver disease requiring LT. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.011DOI Listing
August 2018
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Overlap Syndrome of Autoimmune Hepatitis and Primary Biliary Cholangitis.

Clin Liver Dis 2018 08;22(3):603-611

Department of Digestive Diseases, Yale University, New Haven, CT, USA. Electronic address:

Overlap syndrome of autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) is typically defined as concomitant or serial presentation with clinical features of both of these 2 distinct diseases. The Paris criteria and variations of the International Autoimmune Hepatitis group scoring systems for the diagnosis of AIH have been used to diagnose overlap syndrome. If left untreated, patients with overlap syndrome will have higher rates of portal hypertension, gastrointestinal bleeding, ascites, death, and need for liver transplant. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.010DOI Listing
August 2018
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Antimitochondrial Antibody-Negative Primary Biliary Cholangitis: Is It Really the Same Disease?

Clin Liver Dis 2018 08;22(3):589-601

Division of Gastroenterology and Hepatology, Mayo Clinic, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA; Office of the Provost, Arizona State University, 550 North 3rd Street, Phoenix, AZ 85004, USA. Electronic address:

Antimitochondrial antibody (AMA)-negative primary biliary cholangitis (PBC) is a term reserved for patients with clinical and histopathological findings consistent with PBC but without positive AMA. There does not seem to be a natural progression from AMA negativity to positivity. Antinuclear and antismooth muscle antibodies are frequently found in the absence of histologic autoimmune hepatitis features. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.009DOI Listing
August 2018
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Liver Biopsy in Primary Biliary Cholangitis: Indications and Interpretation.

Clin Liver Dis 2018 08;22(3):579-588

Division of Liver Pathology Research, Center for Liver Diseases, Inova Fairfax Hospital, 3300 Gallows Road, Falls Church, VA 22042, USA. Electronic address:

Primary biliary cholangitis is a disease characterized by immune-mediated bile duct destruction, followed by inflammation, scarring, and the development of chronic cholestasis and a slow progression to cirrhosis over the course of years. Liver biopsy has traditionally been used in conjunction with clinical evaluation and serologic autoantibody testing to establish the diagnosis, but it is no longer required in typical cases with positive antimitochondrial antibodies. Biopsy remains essential, however, in antimitochondrial antibody-negative patients or suspected overlap syndrome with autoimmune hepatitis, and if an adequate biopsy is performed precise staging is possible for assessment of prognosis. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.008DOI Listing
August 2018
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Natural History of Primary Biliary Cholangitis in the Ursodeoxycholic Acid Era: Role of Scoring Systems.

Clin Liver Dis 2018 08;22(3):563-578

Division of Gastroenterology and Hepatology, Stanford University, Alway Building, Room M211, 300 Pasteur Drive, Stanford, CA 94305, USA. Electronic address:

Primary biliary cholangitis (PBC) is a chronic disease that progresses to end-stage liver disease. Ursodeoxycholic acid (UDCA), the standard treatment for PBC for several decades, is associated with improved survival without liver transplantation. Approximately 40% of patients do not respond to UDCA. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.007DOI Listing
August 2018
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Individualizing Care: Management Beyond Medical Therapy.

Clin Liver Dis 2018 08;22(3):545-561

Division of Gastroenterology and Hepatology, Department of Medicine and Surgery, University of Milan Bicocca, Piazza dell'Ateneo Nuovo, 1, 20126 Milan, Italy; Academic Department of Medical Genetics, University of Cambridge, Cambridge, UK. Electronic address:

The evolving research landscape, with advances in the omics technologies, availability of large-scale patient cohorts, and forthcoming availability of novel drugs in primary biliary cholangitis (PBC), is creating a unique opportunity for developing a precision medicine (PM) program. PM has potential to change the paradigm of management. Diagnostic work-up of PBC patients may include information on genetic variants and molecular signature to define a particular subtype of disease and provide an estimate of treatment response and survival. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.006DOI Listing
August 2018
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Chronic Complications of Cholestasis: Evaluation and Management.

Authors:
David N Assis

Clin Liver Dis 2018 08 28;22(3):533-544. Epub 2018 May 28.

Department of Medicine, Section of Digestive Diseases, Yale University School of Medicine, 333 Cedar Street, 1080 LMP, New Haven, CT 06510, USA. Electronic address:

Patients with primary biliary cholangitis (PBC) are at risk for various harmful consequences of chronic cholestasis. These include fat-soluble vitamin deficiency, even in the setting of macronutrient sufficiency, as well as metabolic bone disease, including osteoporosis with fractures. Hyperlipidemia is often present and less commonly associated with risk of cardiovascular event; however, the long-term effect of new emerging therapies for PBC remains to be determined. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.014DOI Listing
August 2018
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Understanding and Treating Pruritus in Primary Biliary Cholangitis.

Clin Liver Dis 2018 08 17;22(3):517-532. Epub 2018 May 17.

Division of Hepatology, Schiff Center for Liver Diseases, University of Miami Miller School of Medicine, Miami, FL, USA.

Pruritus is a common symptom with primary biliary cholangitis. Research has focused on refining understanding of the neurohumoral pathways involved in transduction of pruritus from peripheral cutaneous receptors to the central nervous system, and identifying modulating drugs. Current treatments have variable efficacy and safety. Read More

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http://dx.doi.org/10.1016/j.cld.2018.03.005DOI Listing
August 2018
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