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73 Cards in this Set

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Jaundice during 1st week of life;not clinically important;charact. by uncongugated hyperbilirubinemia;due to inc. bilirubin prod. & def. glucuronyl transferase in immature liver
Physiologic jaundice of the newborn
-distinguished from neonatal cholestasis(due to extrahepatic biliary atresia, alpha1-antitrypsin def.,CMV infxn, & many other conditions)
Commonly occuring congenital hyperbilirubinemia charact. by inc. serum unconjugated bilirubin w/ no clinical consequence due to dec. uptake of bilirubin by liver cells & dec. glucuronyl transferase activity
Gilbert Synd.
Severe congenital hyperbilirubinemia charact. by inc. unconjugated bilirubin due to deficiency of glucuronyl transferase;leads to early death due to Kernicterus(damage to basal ganglia & CNS)
Crigler-Najjar Synd.
-responds to Phenobarbital w/c dec. serum conc. of unconjugated bilirubin
An autosomal recessive form of unconjugated hyperbilirubinimia due to defective bilirubin transport & charact. by brown-black discoloration of the liver due to deposition of dark pigment granules
Dubin-Johnson Synd.
Similar to Dubin-Johnson Synd but w/o the abnormal pigment present
Rotor Synd.
Type of juandice charact. by inc. serum conjugated or unconjugated bilirubin, inc. urine bilirubun,normal-dec. urine urobilinogen,inc. ALT,AST,ALP(indicates intrahepatic obstruction)
-hepatocellular damage results in impaired conjugation;intrahepatic cholestasis results in retention of conjugated bilirubin
Type of jaundice charact. by inc. serum conjugated bilirubin,inc. urine bilirubin,dec. urine urobilinogen,inc. ALP,variable ALT & AST
-w/ complete obstruction,stools are pale & clay colored,urine urobilinogen is undetectable
Type of jaundice charact. by inc. serum unconjugated bilirubin,absent urine bilirubin(acholuria),inc. urine urobilinogen
-degree of urobilinogen inc. directly related to inc. hemoglobin catabolism
Liver dse. charact. by jaundice,inc. serum aspartate(AST)& alanine aminotransferase(ALT)due to RNA viral agents(EBV,CMV)except for HBV(DNA virus)
Acute Viral Hepatitis
-presents w/ fever,anorexia,nausea, vomitting,jaundice,dark urine,pale feces,elevated transaminase levels
Type of hepatitis spread via fecal-oral route,has no carrier state,does not lead to chronic liver dse.,incubation period(15-45dys),no relation to hepatocellular carcinoma,affects mostly children
-presence of IgM antibody(ongoing infxn,most important for dx.,not found in acute stage),IgG(past infxn)
Type of hepatitis virus containing a DNA genome;spread via parenteral, sexual,vertical route;incub.(60-90dy); assoc. w/ hepatocellular carcinoma; results in a carrier state or chronic liver dse.;charact. by "ground-glass" appearance of hepatocytes
HBV(hepadna family)
-consist of HBcAg,HBeAg(indicator of infectivity),HBsAg(if present >6mos.~carrier state,elicits anti-HBsAg,persist for 3-4mos)
Serum marker in HBV infection w/c is present during the window phase
-also anti-HBcAg(appears 4wks after appearance of HBsAg,present during acute illness,remain inc. for years)
Hepatitis virus transmitted parentally(common cause of transfusion mediated hepatitis);leads to a carrier state & chronic hepatitis;assoc. w/hepatocellular carcinoma
-formally known as non-A,non-B hepatitis
Hepatitis virus surrounded by a proteinaceous coat of HBsAg; replicatively defective(requires simultaneous infxn. w/ HBV;transmitted sexually or parentally;found in high incidence w/ intravenous drug users
HDV(delta agent)
Hepatitis virus transmitted fecal-oral route(similar to HAV);occurs in water borne epidemic form in underdevelop countries
Hepatitis in w/c symptoms persist for >6mos.;results from any viral hepatitides(except HAV,VEV)infxn,liver damagedue from non-viral agents or from non immunologic abnormalities(marked by hypergammaglobulinemia & anti-smooth muscle antibodies)
Chronic Hepatitis
Inflammatory liver disorder of unknown etiology charact. by multinucleated giant cells;demonstrates bile pigment & hemosiderin w/ in the parenchyma; results in juandice during 1st wk of life
Neonatal Hepatitis
Virus w/c causes "infectious mononucleosis" often causing hepatitis & involving other organ systems
Epstein-Barr virus(EBV)
Virus affecting the liver in infants & immunocompromised people demonstrating the charact. "owl's eye appearance"
Liver infxn. charact. by a midzonal hepatic necrosis & peresence of "councilman bodies"(dying hepatocytes condense into contracted eosinophilic forms)
Yellow Fever
Inflammatory liver disorder caused by leptospira species charact. by jaundice,renal failure, & hemorrhagic phenomena
-also known as Weil dse or icterohemorrhagic fever
Inflammatory liver disorder caused by ingestion of tapeworm eggs from the feces of dogs & sheep resulting in hydatid dse. of the liver in w/c parasitic cyst invade the liver
Echinococcus Granulosus
Inflammatory liver dse. caused by Schistosoma mansoni or S.japonicum in w/c the adult worms lodge in the portal vein & branches~eggs are anitgenic, stimulating granuloma formation resulting in tissue destruction, scarring, & portal HPN
A liver disorder assoc. w/ presence of small fat vacoules in parenchymal liver cells
Microvesicular fatty liver
-differs from large fat containing vacoules charact. of fatty change
An acute microvesicular fatty liver disorder found in young children also charact. by encephalopathy & coma; assoc. w/ aspirin administration in children w/ acute viral infections
Reye Synd.
Most common form of liver dse. in the U.S w/c vary from fatty liver(steatosis,most frequent,reversible) to alcoholic hepatitis & cirrhosis;may be asymptomatic,assoc. w/ mild-severe hepatic infl.,cirrhosis, or encephalopathy
Alcoholic Liver Dse
-assoc. w/ excessive alcohol consumption
Variant of alcoholic liver dse. charact. by fatty change,focal liver cell necrosis,infiltrates of neutrophils & Mallory bodies(intracytoplasmic eosinophilic hyaline inclusions) derived from cytokeratin intermediate filaments
Alcoholic Hepatitis
-assoc. w/ irreversible fibrosis w/c surround the central veins(perivenular fibrosis,sclerosing hyaline necrosis, central hyaline necrosis)leading to central vein obstruction & fibrosis surrounding the liver cells causing cirrhosis
Chronic liver dse. charact. by generalized disorganization of hepatic architecture w/ scarring & nodular form. due to cell damage,regenerative activity,& fibrosis;assoc. w/ inc. incidence of hepatocellular carcinoma
-classified on basis of nodular size(micronodular,macronodular,mixed)
Most common form of cirrhosis assoc. w/ alcoholism w/ micronodular pattern evolving in late stages to typical hobnail liver w/ large, irregular nodules
Type of cirrhosis due to a familial defect in control of iron absorption~accumulation of hemosiderin in hepatic & pancreatic parenchymal cells,myocardium etc.;micronodular
Hemochromatosis(Hereditary primary)
-triad of cirrhosis,diabetes mellitus, & inc. skin pigmentation
Cirrhosis due to accumulation of copper in the liver,kidney,brain & cornea;can be micronodular or macronodular;dec. serum ceruplasmin
Wilson Dse.
Cirrhosis assoc. w/ galactosemia, glycogen storage dse., or alpha1-antitrypsin def.
Inborn errors of metabolism
Findings in alcoholic cirrhosis assoc. w/ hepatocellular damage & liver failure
Jaundice(mixed),hypoalbuminemia(dec. albumin synthesis),coagulation factor def.(dec. synthesis of coagulation factors except von Willebrand factor, hyperestrinism(palmar erythema;spider nevi of face,upper arms,chest;loss of body hair;testicular atrophy & gynecomastia
Causes of peripheral edema,ascites, or hydrothorax w/c are changes due to liver cell damage & portal HPN in alcoholic cirrhosis
Inc. portal venous pressure(leads to inc. production of hepatic lymph),dec. plasma oncotic pressure(due to hypoalbuminemia),retention of Na & H20(due to dec. hepatic degradation of aldosterone,activation of renin-angiotensin system, or both)
Consequence of intrahepatic scarring w/ inc. portal venous pressure in alcoholic cirrhosis
Esophageal varices(leads to upper GI bleeding),rectal hemmorhoids,caput medusae(periumbilical collaterals), splenomegaly
Pathology of encephalopathy(portal-systemic encephalopathy)due to cell damage & portal HPN in alcoholic cirrhosis
Shunting of portal bld. to systemic circulation~delivery of unfiltered neurotoxins(ammonia,enteric degradation products)directly into systemic circulation~confusion,deep coma, asterixis(flapping tremor of hands)
Morphologic abnormalities found in alcoholic liver dse.
Enlarge-small-shrunken liver; miconodular;fibrous bands surrounding nodules of distorted liver cell plates; presence of proliferating bile ducts & inflammatory cells(lymphocytes,plasma cells)in fibrous bands;hobnail liver(form. of large,irregular nodules resulting in scarred,shrunken liver in the late stages)
Cirrhosis charact. by large,irregular nodules due to broad fibrous bands dividing the liver w/c often contain intact hepatic lobules;sequela of chronic active hepatitis(HBV&HCV are most common)
-may result from alcoholic cirrhosis; leads to hepatocellular carcinoma;may be caused by noninfxn. hepatotoxic agents
Cirrhosis autoimmune in origin assoc. w/ inc. incidence of other autoimmune disorders & charact. anti-mitochondial antibodies;common in middle-age women; inc. parenchymal copper concentration; charact. by severe obstructive jaundice,itching,hypercholesterolemia(leads to cutaneous xanthoma form.)
Primary Billiary Cirrhosis
Cirrhosis due to extrahepatic biliary obstruction~dilation,inc. pressure w/in intrahepatic bile ducts & cholangioles~ ductal injury,ductal & periductal infl.~fibrous tissue form.;complicated by ascending cholangitis & bact. infl. of intrahepatic bile ducts
Secondary Biliary Cirrhosis
-marked histologically by bile stasis, bile lakes, & accumulation of bile w/in the hepatic parenchyma
Cirrhosis charact. by inflammation, fibrosis,stenosis of intrahepatic & extrahepatic bile ducts resulting in biliary cirrhosis;rare except in assoc. w/ inflammatory bowel dse(ulcerative colitis);assoc w/ inc. incidence of cholangiocarcinoma
Primary Sclerosing Cholangitis
Cirrhosis charact. by triad of cirrhosis(micronodular,pigmented cirrhosis), diabetes mellitus, & inc. skin pigment.(hemosiderin & melanin deposition)also known as bronze diabetes;inc. serum iron w/ dec. TIBC~inc. transferrin iron saturation,inc. serum ferritin
Hereditary Hemochromatosis
-familial defect of iron absorption by the intestinal mucosa;one of the most common autosomal recessive disorder
Test done to detect hereditary hemochromatosis in its early stages so it may be treated successfully before end organ damage
Screening for inc. transferrin iron saturation or for mutation in the Hfe gene on chromosome 6
-flapping tremor & neurologic sign, caused by nitrogenous metabolites w/c cannot be detoxified by the damaged liver
Biliary tract dse., commonly seen in assoc. w/ ulcerative colitis
Primary Sclerosing Cholangitis
-histologically characterized by onion-skin fibrosis of bile ducts
Councilman bodies
Viral Hepatitis
-Apoptotic hepatocytes
Fatal thrombotic occlusion of the hepatic veins assoc. w/ polycythemia vera & other hypercoagulable states
Budd-Chiari Synd
Grossly ditended abdomen, esophageal varices, caput medusae, & hemorrhoides
-caused by portal hypertention & active portocaval anastomoses
Hepatolenticular degeneration causing extrapyramidal motor signs
Wilson Dse.
-Copper deposits in the liver, cornea of the eye(Kayerser-Fleischer Rings), & putamen & lenticular nuclei of the basal ganglia
Increase in AFP(alpha-fetoprotein)
Hepatocellular Carcinoma
-also present in yolk sac tumors
Macronodular pattern on the liver surface
Posthepatitic(postnecrotic, macronodular)cirrhosis
Mallory bodies, neutrophilic infiltrate,& hepatocyte swelling & necrosis
Alcoholic hepatitis
-Mallory bodies=accumulation of cytokeratin intermediate filaments in hepatocytes
Micronodules on the liver surface in early stages
Alcoholic cirrhosis
Most common liver dse. in the U.S.
Alcoholic liver dse.
Middle-aged female w/ pruritus, jaundice, xanthomas, & antimitochondrial antibodies
Primary biliary cirrhosis
-histologically characterized by granulomatous destruction of intrahepatic bile ducts
Most common primary malignancy of the liver
Most common primary malignancy of the liver
Obese multiparous woman w/ fat intolerance
Obstructive jaundice w/ palpably enlarged gallbladder
Carcinoma of the head of the Pancrease or carcinoma of the extrahepatic biliary ducts & ampulla of vater
-Courvoisier Law=obstructive jaundice w/ palpable gallbladder indicates probable tumor; jaundice w/ nonpalpable gallbladder indicates probable gallstones
Palmar erythema, spider angiomatas, & gynecomastia
-all caused by hyperestrinism
Soft, yellow, greasy liver
Fatty liver
-hepatic steatosis
Sudden right upper quadrant or epigastric pain w/ nausea, vomitting, & leukocytosis
Acute Calculous Cholecystitis
-one of the most common indications for abdominal surgery
Triad of cirrhosis, skin pigmentation, and diabetes mellitus("bronze diabetes")
Idiopathic Hemochromatosis
-primary defect in iron absorption leading to a net accumulation
Malignant tumor assoc. w/ Clonorchis sinensis(liver fluke)infest.
;originates from intrahepatic biliary epith;propensity for invasion of vascular channels;not assoc. w/ HBV infect.;late complication of thorium dioxide administration
Cholangiocarcinoma(bile duct carcinoma)
Malignant tumor assoc. w/ toxic exposure to polyvinyl(vinyl)chloride, Thorotrast,& arsenic
Complications of cholelithiasis
Biliary colic,common bile duct obstruction,ascending cholangitis, cholecystitis,acute pancreatitis, gallstone ileus,mucocele,malignancy
Disease of the gallbladder characterized by yellow cholesterol-containing flecks in the mucosal surface;non-inflammatory;no special assoc. w/ cholelithiasis
Cholesterolosis(strawberry gallbladder)
Most common primary tumor of the gallbladder often assoc. w/ gallstones
Tumor presenting w/ progressive obstructive jaundice & a palpable enlarged gallbladder;often adenocarcinoma
Carcinoma of extrahepatic biliary ducts & ampulla of vater
-tumors obstructing the common bile duct~enlarge distended gallbladder; obstructing stones do not(Courvoisier law)
Vascular disorder of the liver charact. by venous collaterals w/ varies in the submucosal veins of the esophagus,the hemorrhoidal plexus,& other sites
Portal HPN
-classified by site of portal venous obstruction:prehepatic(portal & splenic obstruct. often by thrombosis), intrahepatic(intrahepatic vascular obstruct. due to cirrhosis or metastatic tumor),posthepatic(congestion of distal hepatic venous circl. due to constrictive pericarditis,tricuspid insuff.,CHF, hepatic vein occlusion(Budd-Chiari synd)
Hepatic vascular disorder due to occlusion of the major hepatic veins~abdominal pain,jaundice, hepatomegally,ascites~liver failure
Budd-Chiari Synd.
-assoc. w/ polycythemia vera, hepatocellular carcinoma,abdominal neoplasms;complication of pregnancy
Benign tumors of the liver
Hemangioma(most common),Adenoma(assoc. w/ oral contraceptives,may rupture ~intraperitoneal hemmorrhage when subscapular in location)
Malignant tumors of the liver
Metastatic tumor(common), Hepatocellular carcinoma(most common), Cholangiocarcinoma(bile duct carcinoma),Hemangiosarcoma(angiosarcoma)
Malignant tumor assoc. w/ pre-existing cirrhosis of any kind,HBV infxn., aflatoxin B1 contamination of nuts & grains causing mutations in p53 gene; propensity for invasion of vascular channels w/ hematogenous dissemination
Hepatocellular Carcinoma