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85 Cards in this Set

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What is Jaundice?
1) Refers to yelow discoloration of skin, sclerae, and tissues caused by hyperbilirubinemia
2) Most often associated with hepatocellular disease, biliary obstruction, or hemolytic anemia
What is physiologic jaundice of the newborn?
1) Commonly noted during the first week of life but is not usually clinically important
2) Characterized chemically by unconjugated hyperbilirubinemia
3) Results from both increased bilirubin production and a relative deficiency of glucuronyl transferase in the immature liver; these phenomena are exaggerated in premature infants
4) Must be distinguished from neonatal cholestasis, which is due to a wide variety of causes, including extrahepatic biliary atresia, alpha-1-antitrypsin deficiency, CMV infection, and many other conditions
What is Gilbert syndrome?
1) Congenital hyperbilirubinemia
2) Extremely common, occurring in almost 5% of the population
3) Familial disorder characterized by a modest elevation of serum unconjugated bilirubin
4) Liver is otherwise unimpaired, and there are no clinical consequences
5) Caused by a combination of decreased bilirubin uptake by liver cells and reduced activity of glucronyl transferase
What is Crigler-Najjar synrome?
1) Congenital hyperbilirubinemia
2) Severe familial disorder characterized by unconjugated hyperbilirubinemia caused by a deficiency of glucoronyl transferase
3) One form leads to early death from kernicterus, damage to the basal ganglia and other parts of the central nervous system caused by unconjugated bilirubin
4) Less severe form, responds to phenobarbital therapy, which decreases the serum concentration of unconjugated bilirubin
What is Dubin-Johnson syndrome?
1) Autosomal recessive form of conjugated hyperbilirubinemia characterized by defective bilirubin transport
2) Characterized by a striking brown-to-black discoloration of the liver caused by the depostition of granules of very dark pigment, the chemical nature of which is unclear
What is Rotor syndome?
Similar to Dubin-Johnson syndrome, but abnormal pigment is not present
What is hepatocellular jaundice?
1) Conjugated and unconjugated
2) Urine bilirubin is increased
3) Urin urobilinogen is normal to decreased
4) Intrahepatic cholestasis may result in retention of conjugated bilirubin; hepatocellular damage may result in impaired conjugation of bilirubin; enzyme activities of ALT and AST increased; increased alkaline phosphatase indicates intrahepatic obstruction
What is obsturctive jaundice?
1) Conjugated hyperbilirubinemia
2) Urine bilirubin is increased
3) Urine urobilinogen decreased
4) Alkaline phosphatase and cholesterol increased
5) ALT and AST variable
6) With complete obstruction, stools pale and clay-colored and urine urobilinogen undetectable
What is hemolytic jaundice?
1) Unconjugated hyperbilirubinemia
2) Urine bilirubin is absent (acholuria)
3) Urine urobilinogen is increased
4) Degree of urine urobilinogen increase directly related to increased hemoglobin catabolism
What is acute viral hepatitis?
1) Characterized by jaundice and extremely high elevations fo serum aspartate and alanine aminotransferase
2) May be caused by a variety of viral agents, including EBV or CMV, but usually results form hepatic infection by any of the following viruses, all of which are RNA viruses, with the exception of hepatitis B virus, which is a DNA virus.
What is HAV?
1) Spread by fecal-oral transmission; parenteral infection does not occur
2) Has an incubation period of 15-45 days
3) Does not cause a chronic carrier state or lead to chronic hepatitis; complete recovery almost always occurs
4) Has NO relation to hepatocellular carcinoma
What is HBV?
1) Consists of a central core containing the viral DNA genome
2) DNA polymerase
3) Hepatitis B core antigen (HBcAg)
4) Hepatitis B e antigen (HBeAg)
5) Outer lipoprotein coat containing the hepatitis B surface antigen (HBsAg).
6) The complete virion is known as the Dane particle
7) Transmitted ia parenteral, sexual, and vertical routes
8) Incubation period averaging 60-90 days
9) Major association with hepatocelluar carcinoma
10) Infection is associated with a "ground-glass" appearance of hepatocytes.
11) Can result in a carrier state or in chronic liver disease
What is HBsAg?
1) Appears in serum some weeks before the onset of clinical findings, then decreases and generally persists for a total of 3-4 months
2) Persistence as detectable serum antigen for more than 6 months denotes the carrier state
3) Elicits an antibody response (anti-HBsAg); antibody appears a few weeks after the disappearance of the antigen and indicates recovery as well as immunity to future infection
What is Anti-HBcAg?
1) Appears aabout 4 weeks after the appearance of HBsAg, is present during the acute illness, and can remain elevated for several years.
2) Marker (along with anti-HBeAg) of hepatitis infection during the "window period" between the disappearance of HBsAg and the appearance of anti-HBsAg
What is HBeAg?
1) Appears shortly after HBsAg and disappears before HBsAg
2) Closely correlated with viral infectivity
What is HBV DNA?
Can also be detected in serum and is an index of infectivity
What is HCV?
1) Transmitted parenterally and is a frequent cause od transfusion-mediated hepatitis
2) Often leads to a carrier state and chronic hepatitis
3) Frequent association of hepatocellular carcinoma
What is HDV?
1) Very small, spherical virus consisting of a single RNA strand and the associated delta protein antigen (HDAg), surrounded by a proteinaceous coat of HBsAg
2) Replicatively defective, requiring simultaneous infection wiht HBV for viral replication
3) Transmitted via sexual or parenteral routes
4) Found in especially high incidence in IVDU
5) Usually causes illness more severe than HBV infection alone
What is HEV?
1) Causes an enterically transmitted form of viral hepatitis similar to HAV infection that occurs in water-borne epidemic form in underdeveloped countries
2) Has an as yet unknown association with chronic hepatitis or hepatocellular carcinoma
What is HGV?
1) Affects 1-2% of helthy blood donors, but its pathologic significance is questionable
2) Does NOT lead to chronic hepatitis
3) Has no known relationship to hepatocellular carcinoma
What is the etiology of chronic hepatitis?
May result from any of the viral hepatitides except HAV or HEV infection and also from liver damage induced by nonviral agents
What is Autoimmune hepatitis?
1) Morphologically indistinguishable from other forms of chronic hepatitis
2) Secondary to various immunologic abnormalities
3) clinically marked by hypergammaglobulinemia and anti-smooth muscle antibodies
What is neonatal hepatitis?
1) Unknown etiology
2) Characterized by the presence of multinucleated giant cells
3) May demonstrate bile pigment and hemodiserin within parenchymal cells
4) May result in jaundice during the first few weeks of life
What is EBV?
Causes infectious mononucleosis, which often has a hepatitic component
What is CMV?
1) May involve the liver in infants and immunocompromised persons
2) Infected liver cells demonstrate characteristic nuclear inclusions surrounded by a halo
What is HSV-1?
May involve the liver in infants and immunocompromised persons
What is Yellow fever?
1) Characteristically demonstrates a severe hepatitic component characterized by midzonal hepatic necrosis
2) The dying hepatocytes often condense into eosinophilic contracted forms referred to as Councilman bodies.
3) Similar inclusions are observed in all of the viral hepatitides.
What is leptospirosis?
1) Also known as Weil disease or icterohemorrhagic fever
2) Caused by Leptospira species
3) Severe infection characterized by jaundice, renal failure, and hemorrhagic phenomena
What is echinococcus granulosus infestation?
1) Caused by ingestion of tapeworm eggs from the excreta of dogs and sheep
2) Results in hydatid disease of the liver, in which large parasitic cysts invade the liver
What is schistosomiasis?
1) Caused by infestation with Schistosoma mansoni or S. japonicum.
2) The adult worm lodge in teh portal vein and its branches.
3) The eggs are highly antigenic and stimulate granuloma formation, with resultant tissue destruction, scarring, and portal hypertension
What is microvesicular fatty liver?
Group of serious disorders associated with the presence of small fat facuoles in parenchymal liver cells, which differ from the large fat-containing vacuoles characteristic of fatty change.
What is Reye syndrome?
1) An acute disorder of young children characterized by encephalopathy, coma, and microvesicular of fatty change
2) Associated with aspirin administration to children with acute viral infections
What is tetracycline toxicity?
Results in an unpredictable hypersensitivity-like reaction with microvesicular fatty change
What is alcoholic liver disease?
1) Refers to the constellation of hepatic changes associated with excessive alcohol consumption
2) Most common form of liver disease in the US
3) Varies from fatty change to alcoholic hepatitis and cirrhosis
4) May be asymptomatic or may be associated with mild-to-severe hepatic inflammation, cirrhosis, or encephalopathy
What is fatty change (steatosis) in alcoholic liver disease?
1) Most frequent morphologic abnormality caused by alcohol
2) Reversible
What is alcoholic hepatitis?
1) Characterized by fatty change, focal liver cell necrosis, infiltrates of neutrophils, and the presence of intracytoplasmic eosinophilic hyaline inclusions derived from cytokeratin intermediate filaments
2) Often associated with irreversible fibrosis that characteristically surrounds central veins and has been referred to as perivenular fibrosis, sclerosing hyaline necrosis, or central hyaline sclerosis
3) This fibrosis can lead to central vein obstruction and fibrosis surrounding individual liver cells and can result in cirrhosis
What are the general considerations of cirrhosis?
1) Descriptive term for chronic liver disease characterized by generalized disorganization of hepatic architecture with scarring and nodule formation
2) Liver cell damage, regenerative activity, and generalized fibrosis resulting in a nodular pattern are also characteristic.
3) Classification can be morphologic, on the basis of nodule size (micronodular, macronodular, and mixed macromicronodular forms)
4) Associated, in all forms, with an increased incidence of hepatocellular carcinoma
What are the etiologic agents of cirrhosis?
1) Prolonged alcohol intake, drugs, and chemical agents
2) Viral hepatitis, biliary obstruction, and hemochromatosis
3) Wilson disease and other inborn errors of metabolism
4) Heart failure with long-standing chronic passive congestion of the liver
What is alcoholic (laennec, nutritional) cirrhosis?
1) Prototype for all forms of cirrhosis
2) Most frequently occurring form of cirrhosis
3) Associated with alcoholism; micronodular pattern evolving in late stages to typical hobnail liver with large, irregular nodules
What is postnecrotic (macronodular, posthepatic) cirrhosis?
1) Large, irregular nodules containing intact hepatic lobules
2) Often sequela of chronic active hepatitis, most commonly HBV and HCV
3) Can be caused by noninfectious hepatotoxic agents
4) Sometimes can result from the progression of micronotular alcoholic cirrhosis
5) Can be of uncertain etiology
6) Leads to hepatocellular carcinoma more often than other forms of cirrhosis
What is primary biliary cirrhosis?
1) Probable autoimmune origin; increased incidence of other autoimmune disorders in these patients
2) Antimitochondrial antibodies
3) Obstructive jaundice, itching, and hypercholesterolemia
4) Most common in middle-aged women
What is secondary biliary cirrhosis?
1) End result of long-standing extrahepatic biliary obstruction
2) Leads to dilation and increased pressure within intrahepatic bile ducts and cholangioles, further resulting in ductal injury, ductal and periductal inflammation, and resolution by fibrous tissue formation
3) Often complicated by ascending cholangitis and bacterial inflammation of the intrahepatic bile ducts
4) Marked histologically by evidence of bile stasis adn by bile lakes, accumulations of bile within hepatic parenchyma
What is Hereditary (primary) Hemochromatosis?
1) Familial defect in control of iron absorption
2) Massive accumulation of hemosiderin in hepatic and pancreatic parenchymal cells, myocardium, and other sites
3) Classic triad of cirrhosis, diabetes mellitus, and increased skin pigmentation
4) Marked increase in serum iron and modest reduction in TIBC, transferrin
What is secondary hemochromatosis?
1) Caused by chronic iron overload of diverse etiology
2) Most often accociated wiht a combination of ineffective erythropoiesis and multiple transfusions, such as thalassemia major
What is wilson disease?
1) Accumulation of copper liver, kidney, brain, and cornea (Kayser-Fleisher rings)
2) Cirrhosis can be micronodular or macronodular
3) Decreased serum ceruloplasmin
4) Autosomal recessive
What is cirrhosis related to inborn errors of metabolism?
1) Galactosemia
2) Glycogen storage disease
3) Alpha-1-antitrypsin deficiency
What are the findings associated with hepatocellular damage and liver failure in alcoholic cirrhosis?
1) Jaundice: most often mixed conjugated and unconjugated
2) Hypoalbuminemia: caused by decreased albumin synthesis in damaged hepatocytes
3) Coagulation factor deficiencies: caused by decreased synthesis; all coagulation factors, with the exception of von Willebrand factor, are synthesized in the liver
4) Hyperestrinism: manifest as palmar erythema, spider nevi of the face, upper arms, and chest; loss of body and public hair; testicular atrophy; and gynecomastia
What are the consequences of intrahepatic scarring with increased porta venous pressure in alcoholic cirrhosis?
1) Esophageal varices, often leading to upper gastrointestinal hemorrhage
2) Rectal hemorrhoids
3) Periumbilical venous collaterals (caput medusae)
4) Splenomegaly
What are the changes due to both liver cell damage adn portal hypertension in alcoholic cirrhosis?
1) Peripheral edema, ascites, or hydrothorax
2) Encephalopathy
What is peripheral edema, ascites, or hydrothorax associated with alcoholic cirrhosis?
1) Increased portal venous pressure, which leads to increased production of hepatic lymph
2) Decreased plasma oncotic pressure secondary to hypoalbuminemia
3) Retention of sodium and water as a result of decreased hepatic degradation of aldosterone, activation of the renin-angiotensin system, or both
What is encephalopathy (portal-systemic encephalopathy) in alcoholic cirrhosis?
1) Facilitated by shunting from the portal to the systemic circulation
2) Direct delivery of neurotoxic substances such as ammonia and other enteric degradation products directly into the systemic circulation.
3) Neurologic manifestations varying form slight confusion to deep coma along with asterixis are characteristic features
What are the morphologic abnormalities of alcoholic cirrhosis?
1) Liver may be enlarged or small and shrunken
2) Pattern is most often micronodular
3) Hepatic architecture is obscured by fibrous bands surrounding nodules of distorted liver cell plates.
4) Fibrous bands contain proliferating bile ducts and inflamatory cells, most often lymphocytes and plasma cells
5) In late stages, the nodules tend to become larger and irregular; this pattern results in a scarred, shrunken liver termed the hobnail liver
What is primary sclerosing cholangitis?
1) Characterized by inflammation, fibrosis, and stenosis of intrahepatic and extrahepatic bile ducts, eventuating into biliary cirrhosis
2) Rare except in association with inflammatory bowel disease, especially UC
3) Associated with an increased incidence of cholangiocarcinoma
What is portal hypertension?
1) Characterized by the development of venous collaterals wiht varices in the submucosal veins of the esophagus, the hemorrhoidal plexus, and other sites
2) Often classified by the site of portal venous obstruction
What is prehepatic hypertension?
Caused by portal and splenic vein obstruction, most often by thrombosis
What is intrahepatic hypertansion?
Caused by intrahepatic vascular obstruction, most often by cirrhosis or metastatic tumor, and more rarely by exotic entities such as schistosomiasis
What is posthepatic hypertenstion
1) Caused by venous congestion in the distal hepatic venous circulation
2) Most often as a result of constriction pericarditis, tricuspid insufficiency, congestion heart failure, or hepatic vein occlusion (Budd-Chiari syndrome)
What is infarction of the liver?
Unusual because the liver has a double blood supply (mesenteric and hepatic).
What is Budd-Chiari syndrome?
1) Caused by thrombotic occlusion of the major hepatic veins, resulting in abdominal pain, jaundice, hepatomegaly, ascites, and eventual liver failure
2) Most oftn associated with polycythemia vera, hepatocellular carcinoma, and other abdominal neoplasms; may also occur as a complication of pregnancy
How does CHF relate to the liver?
1) Long-standing chronic right-sided heart failure
2) The cut surface of the liver can assume an appearnace referred to as the nutmeg liver, with dark red congestion centrilobular areas alternating with pale portal areas
3) Eventually centrilobular fibrosis occurs, resulting in cardiac cirrhosis
4) Similar changes may follow long-standing constrictive pericarditis or tricuspid insufficiency
What is a hemangioma of the liver?
Most common benign tumor of the liver
What is an adenoma of the liver?
1) Benign tumor of the liver
2) Incidence is apparently related to use of oral contraceptives
3) Whan subscapular in location, may rupture, resulting in severe intraperitoneal hemorrhage
List the benign hepatic tumors.
Hemangioma
Adenoma
List the malignant hepatic tumors.
1) Metastatic
2) Hepatocellular carcinoma
3) Cholangiocarcinoma (bile duct carcinoma)
4) Hemangiosarcoma (angiosarcoma)
How common are metastatic tumors of the liver?
Account for the majority of hepatic malignancies.
What is hepatocellular carcinoma?
1) Most common primary malignancy of the liver
2) Almost always develop is association with pre-existing cirrhosis of any kind, especially when associated with HBV
3) Associated with aflatoxin B1 contamination of nuts and grains (specific point mutations of p53)
4) Frequently is marked by increased serum concentration of alpha-feto-protein (AFP)
5) Propensity for invasion of vascular channels with hematogenous dissemination
What is cholangiocarcinoma (bile duct carcinoma)?
1) Less common than hepatocellular carcinoma
2) Occurs most frequently in the Far East, where it is associated with Clonorchis sinensis (liver fluke) infestation
3) Originates from intrahepatic biliary epithelium
4) Like hepatocellular carcinoma, has a propensity for early invasion of vascular channels
5) Unlike hepatocellular carcinoma is not associated with HBV infection or cirrhosis
6) Sometimes occurs a a late complication of thorium dioxide administration
What is hemangiosarcoma (angiosarcoma)?
1) Rare malignant vascular tumor
2) Associated with toxic exposure to polyvinyl chloride, Thorotrast, and arsenic
What is acute cholecystitis?
1) Acute inflammation of the gallbladder, most often pyogenic
2) Manifest clinically by nausea, vomiting, fever, and leukocytosis associated with right upper quadrant and epigastric pain
What is chronic cholecystitis?
1) Demonstrates thickening of the gallbladder wall as a result of extensive fibrosis
2) Frequently complicated by gallstaones
What are general characteristics of Cholelithiasis (gallstones)?
1) Has a higher incidence in women
2) Often associated with obesity and multiple pregnancies.
What are cholesterol stones?
Often solitary and too large to enter the cystic duct or the common bile duct.
What are pigment stones?
1) Result from precipitation of excess insoluble unconjugated bilirubin
2) Are often associated with hemolytic anemia
3) Can be associated with bacterial infection
What are mixed stones of the gallbladder?
1) Account for most stones (75-80%)
2) Mixture of cholesterol and calcium salts
What are the clinical manifestations of cholelithiasis?
1) Often silent and asymptomatic
2) Fatty food intolerance is characteristic
List the complications of cholelithiasis?
1) Biliary colic
2) Common bile duct obstruction leading to obstructive jaundice
3) Ascending cholangitis
4) Cholecystitis
5) Acute pancreatitis
6) Gallstone ileus
7) Mucocele
8) Malignancy
What is cholesterolosis?
1) Strawberry gallbladder
2) Characterized by yellow cholesterol-containing flecks in the mucosal surface
3) Not associated with inflammatory changes
4) Has no special association with cholelithiasis
What are the tumors of the gallbladder?
1) Benign tumors of the gallbladder are rare
2) The most common primary tumor of the gallbladder is adenocarcinoma, which is often associated with gallstones
What is carconoma of the extrahepatic biliary ducts and the ampulla of Vater?
1) Less common than carcinoma of the gallbladder
2) Almost always adenocarcinoma
3) Typically presents with progressive, relentless obstructive jaundice
4) Often is characterized clinically by the combination of jaundice and a palpably enlarged gallbladder.
5) Tumors that obstruct the common bile duct resut in an enlarged, distended gallbladder; obstructing stones to not (Courvoisier law)
What is acute pancreatitis?
1) Caused by activation of pancreatic enzymes, resulting in autodigestion of the organ, with hemorrhagic fat necrosis adn deposition of calcium soaps, adn sometimes pseudocysts
2) Predisposed by gallstones and excessive alcohol intake
3) Manifest clinically as severe abdominal pain and prostration closely mimicking an acute surgical abdomen
4) Associated with increased serum amylase
5) Characterized by hypocalcemia caused by loss of circulating calcium into precipitated calcium-fatty acid soaps
6) Characterized by hypocalcemia caused by loss of circulating calcium into precipitated calcium-fatty acid soaps
7) Can be superimposed on chronic pancreatitis
What is chronic pancreatitis?
1) Progressive parenchymal fibrosis
2) Almost always associated with alcoholism
3) Calcification can be visualized by x-ray
4) May have pseudocysts
5) Variable findings
What are the variable findings of chronic pancreatitis?
1) Abdominal and back pain
2) Progressive disability
3) Steatorrhea, which is a manifestation of pancreatic insufficiency with lipase deficiency.
4) Malabsorption may be accompanied by a deficiency of fat-soluble vitamins and can lead to night blindness, osteomalacia, etc.
What is carcinoma of the pancreas?
1) Common tumor; incidence is increasing
2) More common in smokers
3) Almost always adenocarcinoma
4) Often silent before widespread dissemination
5) Usually results in death wihtin 1 year
Where does carcinoma of the pancreas arise?
1) Most often arises in the head of the pancreas, causing obstructive jaundice
2) Somewhat less often in the body or tail
3) Carcinoma involving the pancreatic tail can cause islet destruction and secondary diabetes mellitus
How does carcinoma of the pancreas manifest?
1) Abdominal pain radiating through to the back
2) Weight loss and anorexia
3) Sometimes by migratory thrombophlebitis (Trousseau sign)
4) Frequently by common bile duct obstruction resulting in obstructive jaundice
5) Often accompanied by a distended, palpable gallbladder