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Alagille Syndrome. Valencia, CA: Children’s Liver Association for Support Services. 2006. 2 p.

This brochure describes Alagille syndrome, a multi-system hereditary disorder that often presents with symptoms involving the liver during infancy and early childhood. The classic syndrome involves five distinct findings: chronic cholestasis (reduced bile flow), congenital heart disease, bone defects, eye findings, and typical facial features. The management of children with Alagille syndrome is aimed primarily at preventing complications and treating symptoms. Treatment includes vitamin supplementation, drug therapy for pruritus (itching), and liver transplantation. The brochure concludes with a description of the work and goals of the Children’s Liver Association for Support Services (CLASS), a non-profit organization dedicated to addressing the needs of families coping with childhood liver disease and transplantation. The brochure also includes a form with which readers can request to be placed on the mailing list of CLASS.

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Biliary Atresia. Valencia, CA: Children’s Liver Association for Support Services. 2006. 2 p.

Biliary atresia is a progressive inflammatory process that begins soon after birth. In this condition, bile is trapped inside the liver and rapidly causes damage and scarring to the liver cells. This brochure discusses the diagnosis, treatment, and possible complications related to this pediatric liver disease called biliary atresia. The usual history of the disease is a full term infant who appears normal at birth but develops jaundice after the age of two to three weeks. The infant has yellow eyes and skin, light colored stools and dark urine caused by the buildup of bilirubin in the blood. Once the diagnosis is confirmed, the preferred treatment is to remove the blocked biliary ducts outside the liver and attach the small intestine directly to the liver at the spot where bile is found or expected to drain (this operation is called the Kasai procedure). Bile flow is re-established in approximately 80 percent of infants who are operated on when younger than three months of age. Therefore, early diagnosis is very important. The author notes that liver transplantation also plays an important role in the long-term treatment of biliary atresia. The brochure concludes with a description of the work and goals of the Children’s Liver Association for Support Services (CLASS), a non-profit organization dedicated to addressing the needs of families coping with childhood liver disease and transplantation.

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Approach to the Patient with Abnormal Liver Tests and Fulminant Liver Failure. IN: Hauser, S., ed. Mayo Clinic Gastroenterology and Hepatology Board Review. 3rd ed. New York, NY: Informa Healthcare USA. 2008. pp 283-292.

This chapter on managing patients with abnormal liver tests and fulminant liver failure 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 chapter includes a general discussion of commonly used liver tests; the differential diagnosis and discussion of diseases characterized by an increase in hepatocellular enzyme levels; differential diagnosis and discussion of diseases characterized by an increase in cholestatic enzyme levels; the evaluation of patients who have jaundice; and the management of patients who have fulminant liver failure. In general, a patient with liver test abnormalities that are less than twice normal may be followed as long as the patient is asymptomatic and the albumin level, prothrombin time, and bilirubin concentration are normal. Clinical syndromes that contribute to abnormal liver tests include acute hepatitis, chronic hepatitis, cholestasis without hepatitis or jaundice, jaundice, and cirrhosis or portal hypertension. Fulminant liver failure can be defined as the presence of acute liver failure, including the development of hepatic encephalopathy, within 8 weeks after the onset of jaundice in a patient without a previous history of liver disease. Liver transplantation has revolutionized the management of fulminant liver failure, which without transplantation has a low survival rate. The author stresses that transplantation should be performed when a poor outcome is anticipated, before the patient has uncontrolled sepsis or prolonged periods of increased intracranial pressure. The chapter includes diagnostic algorithms for evaluating patients who have abnormal liver tests. 4 figures. 6 tables. 7 references.

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Primary Sclerosing Cholangitis. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 67-84. Available from Humana Press. 999 Riverview Dr, Suite 208, Totowa, NJ 07512. Email: humana@humanapr.com. Website: www.humanapress.com. Price: $89.95.

This chapter on primary sclerosing cholangitis (PSC) 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. Primary sclerosing cholangitis is a chronic, progressive cholestatic liver disease characterized by fibrosis of the intrahepatic and extrahepatic bile ducts. PSC is often associated with inflammatory bowel disease (IBD). The chapter covers diagnosis, pathogenesis, natural history, malignancy risk, and management of patients with PSC. The author notes that liver biopsy may be useful in helping establish the diagnosis of PSC, particularly if the patient’s cholangiograms are normal. PSC is a progressive disease that slowly advances over time and may shorten life expectancy. The most severe complication that can develop is bile duct cancer. No effective medical therapy is yet available for the underlying disease; however, liver transplantation is an option for patients with end-stage liver disease. 4 figures. 1 table. 91 references.

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Cirrhosis: What You Need to Know About This Potentially Life-Threatening Liver Condition. New York, NY: American Liver Foundation. 2007. 2 p.

This brochure from the American Liver Foundation reviews the physiology of the liver and the problems associated with cirrhosis, which is defined as the replacement of normal liver tissue with nonliver scar tissue. As cirrhosis continues, the liver is left without enough functioning tissue to perform properly. The liver is needed to convert food into nutrients and stored energy, detoxify substances that are harmful to the body, process medications, store vitamins and minerals, and make bile, which is used for the digestion of fats. Written in a question-and-answer format, the brochure discusses the causes of cirrhosis; alcoholism and cirrhosis; other causes of cirrhosis, including chronic viral hepatitis, nonalcoholic steatohepatitis (NASH), and bile duct disease; inherited diseases and cirrhosis; the symptoms and complications of cirrhosis; diagnostic tests that may be used to confirm cirrhosis; and treatment options. In the early stages of alcoholic cirrhosis, an effective treatment is abstaining from alcohol and following a nutritious diet. In advanced stages, cirrhosis is a life-threatening condition that can only be treated with liver transplantation. The back cover of the brochure lists facts about cirrhosis in a summarized format. Contact information for the American Liver Foundation is provided.

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Ascites, Hepatorenal Syndrome, and Encephalopathy. IN: Hauser, S., ed. Mayo Clinic Gastroenterology and Hepatology Board Review. 3rd ed. New York, NY: Informa Healthcare USA. 2008. pp 351-362.

This chapter on ascites, hepatorenal syndrome, and hepatic encephalopathy 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. The author describes these conditions as the three main complications that occur with portal hypertension and hepatic synthetic dysfunction of cirrhosis as liver disease progresses. For each condition, the chapter covers pathogenesis, patient evaluation, and treatment strategies, including dealing with refractory conditions. The chapter also covers spontaneous bacterial peritonitis and the prognostic indicators for survival in cirrhosis. Hepatorenal syndrome requires the presence of five conditions for diagnosis: chronic or acute liver disease with advanced liver failure and portal hypertension; low glomerular filtration rate; absence of shock, bacterial infection, nephrotoxic drugs, or significant fluid loss; no sustained improvement in renal function following diuretic withdrawal or plasma volume expansion; and proteinuria of less than 500 milligrams per deciliter and no ultrasonographic evidence of obstructive uropathy or parenchymal renal disease. The only proven treatment for hepatorenal syndrome is liver transplantation. 1 figure. 1 table. 9 references.

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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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Cholestasis Post Liver Transplantation. IN: Lindor, K.; Talwalkar, J., eds. Cholestatic Liver Disease. Totowa, NJ: Humana Press. 2008. pp 171-182.

This chapter on cholestasis that occurs after liver transplantation 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 chapter covers biliary complications, preservation or reperfusion injury and ABO incompatibility, small-for-size syndrome, hepatic artery thrombosis, infectious complications, drug-induced acute cellular rejection, chronic rejection, and recurrent disease, including primary biliary cirrhosis (PBC), primary sclerosing cholangitis (PSC), and viral hepatitis. The authors caution that cholestasis can occur anytime throughout the posttransplant period, may be intrahepatic or extrahepatic in origin, and has a very broad differential diagnosis. Careful diagnostic imaging of the biliary tree is an important first step in the workup, followed by liver biopsy if clinically indicated. 1 table. 50 references.

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Diagnosis and Long-Term Management of Wilson Disease. Gastroenterology and Hepatology. 3(1): 27-29. January 2007.

This article answers common questions that physicians may have regarding the diagnosis and long-term management of patients with Wilson disease. The article begins with a review of the pathophysiology of Wilson disease, an inherited disorder of copper metabolism. Dietary copper is absorbed by the gut and accumulates in the liver, leading to oxidative damage within the liver cells; this damage leads to the development of steatohepatitis, followed by fibrosis and cirrhosis. The author describes the natural course of the disease, the typical initial presentation of patients with Wilson disease, treatment options for these patients, salvage therapy other than transplantation for patients with advanced chronic Wilson disease, the prioritization of patients with Wilson disease for liver transplantation, the long-term prognosis of Wilson disease patients who respond to standard medical therapy, and associated conditions and complications in older patients with Wilson disease. 4 references.

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Endoscopic Management of Primary Sclerosing Cholangitis: State of the Art. American Journal of Gastroenterology. 102:S32-S37 p. 2007.

Primary sclerosing cholangitis (PSC) is a chronic, idiopathic disorder of the bile ducts that progresses from inflammation to fibrotic stricturing in the bile ducts, which in turn leads to biliary obstruction and, ultimately, chronic liver disease. This article brings readers up-to-date on the use of endoscopy in the management of patients with PSC. Fifty to 80 percent of patients with PSC have coexisting inflammatory bowel disease (IBD), more commonly presenting as ulcerative colitis (UC) than Crohn’s disease. The author discusses the clinical presentation of PSC, diagnostic approaches, the medical and surgical treatments used to manage PSC, cholangiographic findings and endoscopic management, and managing strictures. The author describes endoscopic retrograde cholangiopancreatography (ERCP). ERCP is a technique that has increasingly-limited purely diagnostic application in the management of PSC but is of great use in tissue acquisition and in the treatment of cholestatic symptoms in the long-term management of the complications of PSC, notably those due to biliary strictures and bile duct stones. Liver transplantation remains the only definitive treatment for PSC. 3 figures. 32 references.

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