Keywords: Chapter 14, Liver
Abscess of the liver:
Focal suppurative inflammation of the liver. May be pylephlebitis due to ascending infection that enters the liver through the portal veins, or cholangitis, if the infection had reached the liver through the bile ducts.
Alcoholic hepatitis:
Acute febrile disease caused by alcohol abuse. Histologically the liver shows hepatocellular necrosis infiltrates of leukocytes, bilestasis and perivenular fibrosis. Hepatocytes contain Mallory hyaline.
Alcoholic liver disease:
A spectrum of liver diseases caused by excessive chronic intake of alcohol. Includes fatty liver, alcoholic hepatitis, and cirrhosis.
Alpha1-antitrypsin deficiency:
An autosomal recessive disorder characterized by childhood cirrhosis and emphysema. AAT accumulates in liver cells in form of PAS positive globules.
Ascites:
Accumulation of serous fluid (transudate) in the abdominal cavity.
Bile lake:
Morphologic change in the liver caused by longstanding cholestasis. It consists of bile filled spaces in which the liver cells were destroyed by prolonged exposure to detergent action of bile.
Bile stones:
Stones found in the biliary system. May be composed of cholesterol or pigment (bilirubin salts). Bile stones also contain calcium soaps and mucin.
Bile:
Greenish-yellow viscous fluid secreted by the liver, composed of bile sales, bilirubin and its derivatives (e.g. Biliverdin), cholesterol and mucus. It is secreted into the small intestine where it emulsifies the fats. Bile is also stored in the gallbladder.
Biliary atresia:
A congenital developmental disorder characterized by occlusion or incomplete development of bile ducts.
Bilirubin:
Yellow pigment derived from heme. Bilirubin formed from hemolyzed red blood cells is bound to albumin ("unconjugated bilirubin"). In the liver it is conjugated to glucuronic acid and excreted in bile.
Budd-Chiari syndrome:
Occlusion of the hepatic vein and its tributaries. Typically caused by thrombosis due to hematologic diseases (polycythemia, leukemia) tumors, but also trauma, pregnancy or contraceptives.
Cholangiocarcinoma:
Primary malignant tumor of biliary epithelium. May develop inside the liver or in the extrahepatic bile ducts.
Cholangitis, suppurative:
Ascending bacterial infection of the bile ducts. Usually caused by the enteric bacteria such as E. Coli.
Cholecystitis:
Inflammation of the gallbladder, may be acute or chronic.
Cholelithiasis:
Stones within the biliary system, most often in the gallbladder or extrahepatic bile ducts.
Cholestasis:
Stagnation of bile in the liver, induced as bile plugs in the bile canaliculi or brown pigmentation of liver cells. Longstanding cholestasis may produce so called feathery degeneration of liver cells and "bile lakes" which replace the destroyed hepatocytes. Pathogenetically classified as either intrahepatic or extrahepatic cholestasis.
Cirrhosis:
End stage liver disease in which the liver appears nodular and fibrosed. Cirrhosis occurs in several morphologic forms (e.g., Micronodular macronodular). Cirrhosis may be an outcome and or complication of viral hepatitis, alcohol abuse, drug toxicity, hereditary metabolic diseases (AAT deficiency, Wilson disease). Unless treated by transplantation cirrhosis has an invariably lethal outcome.
Crigler-Najjar disease:
Autosomal recessive enzyme deficiency interfering with the conjugation of bilirubin in liver cells. Presents as neonatal jaundice complicated by encephalopathy. Death usually occurs during the first year of life.
Drug induced liver disease:
Anyone of several forms of liver injury by drugs. May present as cholestasis, viral hepatitis like disease, fatty change, peliosis hepatis.
Dubin-Johnson syndrome:
Autosomal recessive chronic or intermittent jaundice, due to defective intracellular transport of bilirubin. Pathologically characterized by accumulation of brown granular pigment in liver cells and Kupffer cells.
Extrahepatic bile duct carcinoma:
Malignant tumor of the common bile duct or hepatic ducts. Histologically an adenocarcinoma. Equally common in males and females.
Fatty liver:
Accumulation of fat in liver cells resulting in enlargement of the liver. The liver is large, yellow and greasy. The fatty change can be caused by obesity, diabetes, alcoholism, drugs (e.g. Tetracycline).
Focal nodular hyperplasia:
A hyperplastic lesion of the liver composed of hepatocellular nodules and fibrous septa. Typically contains a central scar from which the septa radiate toward the periphery.
Galactosemia:
An autosomal recessive deficiency of galactose-1-phosphate uridyl transferase that results in excessive accumulation of galactose in tissues. Typically the disease presents with jaundice, hepatosplenomegaly and hypoglycemia, cataracts and mental deficiency.
Gallbladder carcinoma:
Malignant tumor of the gallbladder. In 90% of cases adenocarcinoma, in 10% squamous cell carcinoma. Typically associated with cholelithiasis. Most common in women. Found in 2% of all cholecystectomy specimens.
Gilbert disease:
Autosomal dominant inconjugated hyperbilirubinemia of unknown pathogenesis. Affects 5-10% of population, more often in males. Typically occurs after puberty. Does not cause any pathologic changes in the liver. Jaundice is the only symptom, but it requires no treatment.
Hemangioma:
Benign tumor composed of blood vessels. The most common liver tumor found at autopsy, however, clinically it is usually silent.
Hemochromatosis, hereditary:
Autosomal recessive disorder of iron absorption resulting in excessive accumulation of iron in the body. Liver contains large amounts of iron in form of hemosiderin, which accumulates in hepatocytes, Kupffer cells and bile ducts. Excessive accumulation of iron leads to cirrhosis, diabetes, skin pigmentation and heart failure.
Hemosiderosis (iron overload):
Excessive accumulation of iron due to transfusion, medication, dietary overload etc. May produce the same pathologic changes as hereditary hemochromatosis.
Hepatic adenoma:
Benign tumor composed of liver cells.
Hepatic artery:
A branch of the celiac artery that provides the arterial blood to the liver.
Hepatic coma:
End stage of hepatic encephalopathy, marked by loss of consciousness, tremor, hyperreflexia and asterixis. It is thought to represent intoxication with metabolites such as ammonia, GABA, mercaptan, etc., that cannot be detoxified by the failing liver.
Hepatic encephalopathy:
Metabolic brain injury caused by liver failure. If untreated will progress to hepatic coma and death.
Hepatic necrosis:
Severe form of hepatitis characterized by widespread liver cell necrosis involving large parts of the lobule. Occurs in several forms such as bridging necrosis, massive confluent necrosis, massive hepatic necrosis (acute yellow atrophy).
Hepatic vein:
Main conduit for the outflow of blood from the liver into the inferior vena cava.
Hepatitis A virus:
RNA enterovirus that causes relatively mild self limited liver disease.
Hepatitis B virus:
Doublestranded DNA hepadna virus which may cause acute or chronic hepatitis.
Hepatitis C virus:
RNA flavivirus which may cause acute or chronic hepatitis.
Hepatitis D virus:
Defective RNA virus which acts as helper to the HBV.
Hepatitis E virus:
RNA virus that causes acute hepatitis.
Hepatitis chronic:
Inflammation of the liver that lasts more than 6 months. May be cause by viruses, drugs, immune factors or inborn metabolic disorders (e.g., Wilson disease, AAT deficiency)
Hepatitis, autoimmune (lupoid) hepatitis:
An autoimmune multisystemic disease of young and middle-aged women characterized by liver inflammation, hypergammaglobulinemia. Responds favorably to corticosteroids.
Hepatitis, viral:
Liver infection most often caused by one of the major hepatotropic viruses (A,B,C,D,E, F); but also by other viruses.
Hepatocellular carcinoma:
Malignant tumor composed of liver cells. Usually develops in cirrhotic liver especially often in cirrhosis caused by hepatitis B and C, AAT deficiency and hemochromatosis.
Hepatorenal syndrome:
Oliguric renal failure secondary to the hormonal and circulatory changes that accompany cirrhosis. The kidney shows no pathologic changes.
Hyperbilirubinemia:
Increased concentration of bilirubin in blood (more than 1mg/dl).
Hypoalbuminemia:
Decreased concentration of albumin in blood (normal 3.5-5.0 gm/dl) typically found in chronic liver disease because of decreased production. Oncotic edema is the most prominent consequence of hypoalbuminemia.
Icterus:
Synonym for jaundice.
Jaundice, physiologic neonatal:
Hyperbilirubinemia encountered in neonates whose liver cannot efficiently conjugate all the bilirubin formed from hemolyzed fetal RBC.
Jaundice:
Yellow discoloration of tissues, best visible on the skin and mucous membranes. Caused by hyperbilirubinemia which may be prehepatic (e.g. Hemolysis of red blood cells), hepatic (e.g. Viral hepatitis) or posthepatic (e.g. Obstruction of bile flow by carcinoma of pancreas).
Kernicterus:
Deposition of bilirubin in the basal ganglia of the brain. Typically found in neonates affected by severe erythroblastosis fetalis and hyperbilirubinemia exceeding 20 mg/dl. Associated with brain damage.
Neonatal hepatitis:
Inflammation of the liver in neonates. Etiology is unknown. Histologically, presents in the form of giant cell hepatitis.
Portal hypertension:
Increased pressure in the portal venous system, usually caused by cirrhosis or some other liver disease.
Portal vein:
Conduit for the venous blood from the intestines and spleen to the liver.
Primary biliary cirrhosis:
An autoimmune disease characterized by destruction of intrahepatic bile ducts and obstructive jaundice. Typically affects middle aged women. Often associated with other autoimmune diseases (e.g. Thyroiditis, rheumatoid arthritis). Antimitochondrial antibodies are present in the serum of almost all patients. Disease progresses to cirrhosis.
Primary sclerosing cholangitis:
A chronic inflammatory disease of extrahepatic bile ducts. The cause of PSC is not known but 40% of patients have ulcerative colitis. It causes obstructive jaundice and biliary cirrhosis.
Reye syndrome:
Acute disease of children characterized by hepatic failure and encephalopathy. Histologically, the liver shows microvesicular steatosis. It is related to aspirin intake for fibrile disease but the exact pathogenesis is not known.
Schistosomiasis:
Chronic disease caused by the deposited ova of Schistosoma species. S. mansoni and S. japonica cause liver fibrosis and portal hypertension.
Secondary biliary cirrhosis:
Cirrhosis caused by extrahepatic obstruction of bile flow.
Splenomegaly:
Enlargement of the liver typically a feature of portal hypertension but may be caused by other diseases (e.g. Leukemia, hereditary spherocytosis).
Steatosis:
Fatty change of the liver.
Wilson disease:
Autosomal recessive disorder of copper metabolism. Characterized by cirrhosis, cerebral symptoms and eye lesions (Kayser-Fleischer ring).