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Your search term(s) "autoimmune hepatitis" returned 19 results.

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Autoimmune Hepatitis. IN: Hauser, S., ed. Mayo Clinic Gastroenterology and Hepatology Board Review. 3rd ed. New York, NY: Informa Healthcare USA. 2008. pp 391-406.

This chapter on autoimmune hepatitis is from a comprehensive textbook that provides an in-depth examination of essential knowledge in gastroenterology, hepatology, and the related areas of pathology, endoscopy, nutrition, and radiology. Autoimmune hepatitis is an inflammation of the liver of unknown cause that is associated with interface hepatitis seen on histologic examination, hypergammaglobulinemia, and autoantibodies. The chapter covers diagnosis, frequency and ethnic distribution, etiology, clinical features, physical findings, laboratory features, concurrent immune diseases, autoantibodies, classification, treatment regimens, liver transplantation, relapse, suboptimal responses, other treatments, long-term surveillance and follow up, and variant syndromes. Prednisone alone or a lower dose of prednisone in combination with azathioprine is effective in the treatment of all forms of autoimmune hepatitis. Improvement in hepatic fibrosis occurs in conjunction with reductions in liver inflammation. Corticosteroids may facilitate the disappearance of fibrosis by suppressing inflammatory activity. Liver transplantation is used as salvage therapy for decompensated disease. 4 figures. 11 tables. 10 references.

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Celiac Disease and Autoimmunity in the Gut and Elsewhere. Gastroenterology Clinics of North America. 37(2): 411-428. June 2008.

This article about celiac disease and autoimmunity in the gut and elsewhere is from an issue of Gastroenterology Clinics of North America that focuses on eosinophilic and autoimmune gastrointestinal disease. Celiac disease is a common immune-mediated enteropathy characterized by sensitivity to the wheat protein, gluten. The authors note that celiac disease is often difficult to diagnose due in large part to the silent form of the disease that affects the majority of patients. Overall mild clinical symptoms with nonspecific complaints such as fatigue, headaches, and arthralgias are common and can delay diagnosis. This article discusses the gut immunogenesis of celiac disease, the role of environmental factors, and the risk of autoimmune disease. The authors focus on the autoimmune connective tissue diseases, endocrine, and dermatologic conditions associated with celiac disease, as well as the related gut inflammatory disorders of refractory celiac disease, autoimmune enteropathy, collagenous enteritis, and collagenous colitis. Specific diseases discussed include Sjogren's syndrome, inflammatory arthritis, Addison's disease, autoimmune insulin-dependent diabetes mellitus (AIDDM), IgA deficiency, pernicious anemia, primary biliary cirrhosis, and autoimmune hepatitis. The authors conclude with a brief discussion of screening recommendations in patient populations with these immune-related illnesses. 1 figure. 2 tables. 79 references.

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Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. 188 p.

This book offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. The text includes 10 chapters: the diagnosis of cholestasis, drug-induced cholestasis, primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), overlap syndrome with autoimmune hepatitis, rare causes of cholestasis, cholestatic variants of viral disease and alcohol, cholestasis from systemic disorders, complications of cholestasis, and posttransplantation cholestasis. Specific topics include liver tests and antibodies, cross-sectional studies, liver biopsy, other diagnostic tests, endoscopic ultrasound, herbal remedies, the risk of colon cancer with inflammatory bowel disease (IBD), colon cancer posttransplant, and cholangiocarcinoma, genetic disorders, viral hepatitis, sarcoid disease, lymphoma, granulomatous disease, cystic fibrosis, rheumatologic diseases, osteoporosis, pruritus, hyperlipidemia, strictures, viral disease, and recurrent cholestatic liver disease. Each chapter begins with a brief outline and concludes with a summary and a list of references. The text concludes with a subject index.

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Cirrhosis. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2008. 8 p.

This fact sheet describes cirrhosis, a condition in which the liver slowly deteriorates and malfunctions due to chronic injury. In cirrhosis, scar tissue replaces healthy liver tissue, partially blocking the flow of blood through the liver. Scarring impairs the liver’s ability to control infections, remove bacteria and toxins from the blood, process nutrients and drugs, make proteins that regulate blood clotting, and produce bile to help absorb fats and fat-soluble vitamins. Written in nontechnical language, the fact sheet describes the causes of cirrhosis, symptoms, complications, diagnosis, measurement of severity, treatment options, and indications for liver transplantation. In the United States, heavy alcohol consumption and chronic hepatitis C have been the most common causes of cirrhosis. Obesity is becoming a common cause of cirrhosis, either as the sole cause or in combination with alcohol, hepatitis C, or both. Other causes of cirrhosis include hepatitis B, hepatitis D, and autoimmune hepatitis; diseases that damage or destroy bile ducts; inherited diseases; nonalcoholic fatty liver disease; drugs; toxins; and infections. Many people with cirrhosis have no symptoms in the early stages of the disease. As the disease progresses, symptoms may include weakness, fatigue, loss of appetite, nausea, vomiting, weight loss, abdominal pain and bloating, itching, and spiderlike blood vessels on the skin. The goals of treatment are to stop the progression of scar tissue in the liver and prevent or treat complications. Treatment for cirrhosis includes avoidance of alcohol and other drugs, nutrition therapy, and other therapies that treat specific complications or causes of the disease. A liver transplant may be considered when complications of cirrhosis cannot be controlled by treatment. Readers are referred to three resource organizations for more information: the American Liver Foundation (www.liverfoundation.org and 1–800–465–4837), Hepatitis Foundation International (www.hepfi.org or 1–800–891–0707), and the United Network for Organ Sharing (www.unos.org or 1–888–894–6361). The fact sheet briefly describes the work of the National Digestive Diseases Information Clearinghouse, which provides information about digestive diseases to people with digestive disorders and to their families, health care professionals, and the public. 1 figure.

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Diagnosis and Treatment of Autoimmune Hepatitis. Gastroenterology Clinics of North America. 37(2): 461-478.June 2008.

This article about the diagnosis and treatment of autoimmune hepatitis is from an issue of Gastroenterology Clinics of North America that focuses on eosinophilic and autoimmune gastrointestinal disease. The authors describe autoimmune hepatitis (AIH) as a hepatitis of unknown cause, known as idiopathic, characterized by inflammation of the liver, presence of auto-antibodies, and evidence of increased gamma globulins in the serum. AIH is considered to be an interaction between the immune system, auto-antigens, and unknown triggering factors. The authors provide a brief summary of the diagnosis of AIH, epidemiologic factors, the natural history of AIH, an approach to the treatment and follow-up of AIH, and the role of liver transplantation in the treatment of AIH. This immune disease affecting the liver responds well to prednisone or a combination of prednisone and azathioprine; most patients can be brought into remission, although many will require maintenance therapy with low-dose levels of these drugs. The authors stress that not all patients with AIH need to be treated, even once the diagnosis is confirmed. Drug therapy should be considered in patients who have cirrhosis if biopsy demonstrates considerable inflammation. Liver transplantation should be considered in patients who have decompensated cirrhosis from AIH or in those patients who have severe fulminant hepatitis who fail to respond to initial therapy. 3 figures. 3 tables. 60 references.

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Eosinophilic and Autoimmune Gastrointestinal Disease: New Insights and New Entities. Gastroenterology Clinics of North America. 37(2): 1-492. June 2008.

This issue of Gastroenterology Clinics of North America focuses on eosinophilic and autoimmune gastrointestinal disease. Eosinophilic esophagitis (EE) is a clinicopathologic disease characterized by upper intestinal symptoms and the finding of more than 15 or 20 eosinophils in the esophageal epithelium; these findings are unresponsive to proton pump inhibitor treatment. Eosinophils are a type of white blood cell. EE is a common disease that affects both children and adults. This issue offers 11 articles covering gut eosinophilia in food allergy and in systemic and autoimmune diseases, eosinophilic gastroenteritis in adults, the clinical manifestations of eosinophilic esophagitis in children, functional gastrointestinal disorders and the potential role of eosinophils, enteric autoantibodies and gut motility disorders, celiac disease and autoimmunity in the gut and elsewhere, autoantibodies in inflammatory bowel disease (IBD), autoimmune pancreatitis, the diagnosis and treatment of autoimmune hepatitis, and antimitochondrial antibody-negative primary biliary cirrhosis (PBC). Each article is written by experts in the field and concludes with a list of references for readers seeking more information. A subject index concludes the volume.

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Liver: Questions and Answers. IN: Hauser, S., ed. Mayo Clinic Gastroenterology and Hepatology Board Review. 3rd ed. New York, NY: Informa Healthcare USA. 2008. pp 437-456.

This section of questions and answers is from a comprehensive textbook that provides an in-depth examination of essential knowledge in gastroenterology, hepatology, and the related areas of pathology, endoscopy, nutrition, and radiology. This section helps readers review 14 chapters on the liver, covering abnormal liver tests, fulminant liver failure, chronic viral hepatitis, liver mass lesions, alcoholic liver disease, vascular diseases of the liver, portal hypertension-related bleeding, ascites, hepatorenal syndrome, metabolic liver disease, cholestatic liver disease, drug-induced liver injury, autoimmune hepatitis, nonalcoholic fatty liver disease, liver disease and pregnancy, and liver transplantation. The section consists of 43 multiple choice questions, followed by annotated answers that explain each of the correct choices.

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Overlap Syndromes with Autoimmune Hepatitis. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 85-104.

This chapter on overlap syndromes with autoimmune hepatitis is from a book that offers health care providers an overview of cholestatic liver disease; cholestasis is defined as a liver disorder characterized by impaired bile flow. The author of this chapter notes that autoimmune hepatitis, or some features thereof, may coexist with primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC). The overlapping symptoms can include significant elevations of serum aminotransferase, immunoglobulin G, and total protein concentrations, additional circulating antibiotics, and intense, plasma-cell-predominant interface inflammation––hepatitis––that is responsive to systemic immunosuppressive therapy. The chapter covers nomenclature, the interplay of PBC and autoimmune hepatitis, and the overlap between PSC and autoimmune hepatitis. The author concludes that although there is a lot of debate about how autoimmune overlap syndromes should be defined, their existence is not in doubt. The chapter is illustrated with black-and-white photographs. 3 figures. 4 tables. 74 references.

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Liver Diseases in Pregnancy. IN: Pregnancy in Gastrointestinal Diseases. 2nd ed. Bethesda, MD: American College of Gastroenterology. 2007. pp 32-53.

This chapter about liver diseases in pregnancy is from a monograph that presents updated information about pregnancy in women with gastrointestinal disorders. The authors stress that a complete understanding of the physiological changes that affect pregnancy and of the different liver diseases that occur during pregnancy is essential for early recognition and management of pregnancy-associated liver disorders. The chapter focuses on bringing readers up to date on the research in the area covered, the recommended treatments, and patient management concerns, notably issues of maternal and fetal safety. Separate sections discuss the physiological changes that affect pregnancy, diagnostic imaging tests used in pregnancy, liver disorders that are exclusive or unique to pregnancy, liver diseases that may occur during pregnancy or intercurrent liver diseases in pregnancy, and changes that occur when a woman with a pre-existing liver disease becomes pregnant. Specific conditions discussed include hepatic involvement in hyperemesis gravidarum, acute fatty liver of pregnancy (AFLP), intrahepatic cholestasis of pregnancy (IHCP), hemolysis, elevated liver enzymes and low platelets syndrome (HELLP syndrome), viral hepatitis, HIV infection, herpes simplex viral infections, cytomegalovirus infection (CMV), alcohol use, portal hypertension, autoimmune hepatitis (AIH), Wilson disease, primary biliary cirrhosis and primary sclerosing cholangitis, Budd-Chiari syndrome, gallstone disease in pregnancy, and liver transplant. The authors conclude that preventive measures, including early prenatal care, avoidance of risky behaviors that could increase a woman’s chance of acquiring infections, and cessation of smoking and drinking alcohol are vital in decreasing morbidity and mortality in pregnancy. Although liver disease in pregnancy is associated with an increased risk for morbidity and mortality, clinical outcomes have improved for both mother and baby, and pregnancy is not contraindicated in patients with liver disease or in patients who have had a liver transplant. 3 tables. 54 references.

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Autoimmune Hepatitis, From Mechanisms to Therapy. Hepatology. 43(2): S132-S144. February 2006.

This article provides a comprehensive overview of autoimmune hepatitis (AIH), a chronic inflammatory disease of the liver. The authors first review the history of the understanding of AIH, from its first description in 1950 to the use of immunosuppressive therapy for the conditions in the 1970s. In the 1970s and 1980s, several autoantibodies were identified in patients with AIH; subsequently, the molecular targets of these antibodies were identified and more precisely characterized. By now, the immunoserological and genetic heterogeneity of AIH is well established. The clinical manifestations, disease behavior, and thus, treatment outcome, may vary by racial groups, geographical regions, and genetic predisposition. The authors note that, currently, the International Autoimmune Hepatitis group is endorsing multi-center collaborative studies to more precisely define the features at disease presentation, to define prognostic indices, and outline appropriate patient care algorithms. 3 figures. 4 tables. 161 references.

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Autoimmune Hepatitis. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 105-120.

This chapter about autoimmune hepatitis is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The author describes autoimmune hepatitis as a condition of unresolving liver inflammation, which primarily affects young women. The condition is characterized by elevated liver enzymes, hypergammaglobulinemia, serum autoantibodies, interface hepatitis, plasma cell infiltrates on liver biopsy, extrahepatic manifestations, and steroid responsiveness. The chapter provides a brief historical perspective and discussion of epidemiology. It discusses pathogenesis, natural history and prognosis, clinical features, diagnosis and diagnostic tests, complications, and treatment options including medial therapy and liver transplantation. The author cautions that diagnosis of autoimmune hepatitis requires a high index of clinical suspicion because several liver conditions can mimic the disease. Most patients respond to immunosuppressive medications with improvement in clinical symptoms, biochemical liver tests, hepatic histology, and patient survival; however, relapse is common. Up to 40 percent of patients progress to cirrhosis despite therapy. The chapter includes tables and a patient care algorithm and concludes with a list of references. 1 figure. 6 tables. 19 references.

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Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. 356 p.

This user-friendly reference book provides gastroenterologists with an overview of the management of acute and chronic liver disease. The book offers 16 chapters: evaluation of the liver patient, cirrhosis and its complications, acute and chronic viral hepatitis, primary biliary cirrhosis and primary sclerosing cholangitis, autoimmune hepatitis, nonalcoholic fatty liver disease, metabolic liver disease, vascular diseases involving the liver, benign and malignant tumors of the liver, liver disease in pregnancy, postoperative jaundice, nonviral infections of the liver, hepatopulmonary syndrome, portopulmonary hypertension, liver transplantation, and drug hepatotoxicity. Each chapter presents an introduction, consideration of etiology, epidemiology, clinical presentation and symptoms, risk factors, complications, and treatment approaches. The chapters include charts and figures and conclude with a list of references. Most chapters include patient care algorithms. A subject index concludes the volume.

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Current Advances in the Treatment of Autoimmune Hepatitis. Gastroenterology and Hepatology. 2(2): 107-110. February 2006.

This article offers the answers to clinical questions on current advances in the treatment of autoimmune hepatitis, a subtype of chronic liver disease. Autoimmune hepatitis is characterized by major elevations of serum aminotransferase levels, hypergammaglobulinemia, interface hepatitis on histological examination, and autoantibodies. Topics covered include the characteristics of autoimmune hepatitis, serologic markers that support the diagnosis of autoimmune hepatitis, the initial treatment approach to patients diagnosed with autoimmune hepatitis, concerns with current treatment with corticosteroid regimens, the anticipated treatment outcomes for patients with autoimmune hepatitis, the development of new therapies with more powerful immunosuppressive and site-specific actions than prednisone or azathioprine, the use of mycophenolate, combination drug therapy for autoimmune hepatitis, and the molecular interventions being studied to treat autoimmune hepatitis, including T-cell vaccination and gene therapy. 2 figures. 7 references.

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End-Stage Liver Disease. IN: Nilsson, K.R.; Piccini, J.P., eds. Osler Medical Handbook. Philadelphia, PA: Saunders. 2006. pp. 402-417.

Chronic liver disease includes a broad differential diagnosis of infectious, autoimmune, inherited, metabolic, toxic, and acquired origins. This chapter on end-stage liver disease is from a handbook that provides the essentials of diagnosis and treatment, as well as the latest in evidence-based medicine, for residents working bedside, in-patient care. The chapter begins with a presentation of essential Fast Facts and concludes with Pearls and Pitfalls useful to the practicing internist. The body of the chapter is divided into sections: Epidemiology, Clinical Presentation, Diagnosis, and Management. Specific topics covered include the most common causes of cirrhosis in the United States, notably alcoholic liver disease and chronic hepatitis C infection; nonalcoholic fatty liver disease (NAFLD); the causes of the clinical problems associated with cirrhosis; the risk factors for hepatic encephalopathy, including infection, dehydration, hypokalemia, alkalosis, and sedating medications; the risk of liver cancer in patients with cirrhosis; autoimmune hepatitis; the diagnostic tests used to confirm metabolic and inherited causes of chronic liver disease; and the use of the Model for End-Stage Liver Disease (MELD) score to predict mortality, particularly for those patients waiting for a liver transplantation. The chapter concludes with a list of references, each labeled with a 'strength of evidence' grade to help readers determine the type of research available in that reference source. 1 figure. 2 tables. 35 references.

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Hepatobiliary Complications of Inflammatory Bowel Disease. Practical Gastroenterology. 30(8): 19-33. August 2006.

This article outlines the hepatobiliary complications that may be associated with inflammatory bowel disease (IBD, including Crohn’s disease and ulcerative colitis). The author notes that the prevalence of hepatobiliary abnormalities in IBD range from 5 percent to 15 percent. Common hepatobiliary manifestations of IBD can include chronic active hepatitis, cirrhosis, steatosis, and primary sclerosing cholangitis. The author outlines a recommended initial approach to the patient, then discusses primary sclerosing cholangitis (PSC), differential diagnosis, autoimmune hepatitis, cholelithiasis (gallstones), chronic viral hepatitis, and medication-induced hepatotoxicity (including that due to 5-ASA drugs, thiopurines, infliximab, and methotrexate). A final section considers some of the more rare hepatic complications of IBD, including liver abscess, and hepatic amyloidosis. The author concludes that PSC is the classic IBD-related liver disease and occurs most often in patients with ulcerative colitis. Readers are cautioned that patients with IBD are as likely as other patients to develop non-IBD-related liver disorders, so a diligent search for common causes of any hepatobiliary disease should be performed. 1 figure. 2 tables. 34 references.

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Liver Disease in Pregnancy. IN: Lichtenstein, G.; Reddy, K.R.; Faust, T., eds. Clinician’s Guide to Liver Disease. Thorofare, NJ: Slack Incorporated. 2006. pp 211-232.

This chapter about liver disease in pregnancy is from a user-friendly reference book that provides gastroenterologists with an overview of the management of acute and chronic liver disease. The authors stress that because early diagnosis and timely intervention can reduce perinatal and maternal morbidity and mortality, it is important to have a high index of suspicion for potential conditions affecting the liver. Gestational age is used to guide the differential diagnosis for hepatic biochemical test abnormalities during pregnancy. The chapter covers the liver in normal pregnancy, the use of imaging studies during pregnancy, intrahepatic cholestasis of pregnancy (ICP), acute fatty liver of pregnancy (AFLP), hyperemesis gravidarum, clampsia or eclampsia, the HELLP (hemolysis, elevated liver tests, low platelets) syndrome, and intercurrent liver disease in pregnancy, including cholelithiasis, Budd-Chiari syndrome, acute viral hepatitis, drug-induced liver damage, and metastases to the liver. The authors consider chronic liver disease in pregnancy, including cirrhosis, hepatitis C, autoimmune hepatitis, Wilson disease, inherited hyperbilirubinemia, and pregnancy in women who have received a liver transplant. Hyperemesis gravidarum is a first trimester condition, whereas ICP may present in the second trimester. Clampsia and the HELLP syndrome predominantly occur in the third trimester. 4 tables. 28 references.

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Evaluation of the Patient for Liver Transplantation. Hepatology. 41(6): 1-26. June 2005.

Liver transplantation is the most effective treatment for many patients with acute or chronic liver failure resulting from a variety of causes. This article presents the practice guideline from the American Association for the Study of Liver Diseases (AASLD) on the evaluation of patients for liver transplantation. These recommendations are based on a formal review and analysis of the published literature on the topic; several consensus conferences among experts; the American College of Physicians' Manual for Assessing Health Practices and Designing Practice Guidelines; guideline policies produced by professional organizations, including the AASLD and the American Gastroenterological Association; and the authors’ experience in the specified topic. Topics include the indications of liver transplantation, when evaluation for transplantation should be considered, determining the need for liver transplantation, and recipient evaluation at the transplant center. The article offers recommendations for patients with the hepatopulmonary syndrome, portopulmonary hypertension, obesity, cigarette smoking, kidney failure, extrahepatic malignancies, osteoporosis, HIV infection, surgical contraindications, and psychosocial problems. The authors discuss specific indications for liver transplantation, including chronic noncholestatic liver disorders, chronic hepatitis C, chronic hepatitis B, autoimmune hepatitis, alcoholic cirrhosis, cholestatic liver disorders, primary biliary cirrhosis, primary sclerosing cholangitis, childhood cholestatic diseases, metabolic diseases, alpha-1-antitrypsin disease, Wilson disease, nonalcoholic steatohepatitis and cryptogenic cirrhosis, hereditary hemochromatosis, neonatal hemochromatosis, tyrosinemia and glycogen storage disease, metabolic diseases with severe extrahepatic manifestations, amyloidosis and hyperoxaluria, urea cycle and branched-chain amino acid disorders, hepatic malignancies, hepatocellular carcinoma, hepatoblastoma, fibrolamellar hepatocellular carcinoma and hemangioendothelioma, cholangiocarcinoma, and fulminant hepatic failure. The article includes 76 specific recommendations for the evaluation of the patient for liver transplantation. 3 tables. 328 references.

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Autoimmune Liver Disease. IN: U.S. Department of Health and Human Services. Action Plan for Liver Disease Research. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2004. pp. 95-100.

Autoimmune liver disease occurs when the body reacts inappropriately to its own cellular components. Autoimmune liver diseases include autoimmune hepatitis, primary biliary cirrhosis (PBC), and primary sclerosing cholangitis (PSC). All three of these diseases can be severe, progressive, and can lead to cirrhosis and death from end-stage liver disease. This chapter on autoimmune liver disease is from the Action Plan for Liver Disease Research that was developed to advance research on liver and biliary diseases. The Action Plan was undertaken to identify areas of scientific opportunity to help direct research resources at the National Institutes of Health (NIH) toward practical goals in the prevention, diagnosis, and management of liver and biliary diseases. In this chapter, the authors first review the symptoms, etiology, complications, and mechanisms of injury of autoimmune liver disease, then outline recent research advances in the areas of understanding general autoimmunity, causes of autoimmune liver disease, and therapies for autoimmune liver disease. The authors then provide specific research goals in the areas of basic research on pathogenesis, as well as clinical studies of pathogenesis, etiology, and therapy. A final section considers the steps that would assist in achieving these research goals. One chart summarizes the short (0 to 3 years), intermediate (4 to 6 years), and long-term (7 to 10 years) goals of research on these topics. 3 figures. 1 table.

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Pediatric Liver Disease. IN: U.S. Department of Health and Human Services. Action Plan for Liver Disease Research. Bethesda, MD: National Digestive Diseases Information Clearinghouse. 2004. pp. 101-109.

Pediatric liver diseases include biliary atresia, metabolic disorders, intrahepatic cholestatic disorders, alpha-1 antitrypsin deficiency liver disease, nonalcoholic steatohepatitis (NASH), autoimmune hepatitis, sclerosing cholangitis, parenteral nutrition-induced liver injury, drug-induced liver injury, Wilson disease, cystic fibrosis, and various forms of viral hepatitis. Although liver disease is uncommon in children, those liver diseases that occur tend to be severe and progressive. This chapter on pediatric liver disease is from the Action Plan for Liver Disease Research that was developed to advance research on liver and biliary diseases. The Action Plan was undertaken to identify areas of scientific opportunity to help direct research resources at the National Institutes of Health (NIH) toward practical goals in the prevention, diagnosis, and management of liver and biliary diseases. In this chapter, the authors first review the types of liver disease that occur in children and their symptoms, complications, etiology, and mechanisms of injury, then outline recent research advances in the areas of pathogenesis, and prevention and therapy. The authors then provide specific research goals in the areas of pathogenesis and management of biliary atresia, the molecular pathogenesis and treatment of neonatal cholestatic syndromes, the characterization and treatment of pediatric liver diseases beyond the neonatal period, and the optimization of liver transplantation in children. A final section considers the steps that would assist in achieving these research goals. One chart summarizes the short (0 to 3 years), intermediate (4 to 6 years), and long-term (7 to 10 years) goals of research on these topics. 1 figure. 1 table.

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