Pathology(gallbladder,liver,exocrine pancreas)*

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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
-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: Obstructive
-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: Hemolytic
-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: HAV
-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 hepatocy: 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: HBcAg
-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: HCV
-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: HEV
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": Cytomegalovirus(CMV)
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: Leptospirosis
-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: Schistosomiasis
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: Cirrhosis
-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: Alcoholic(Laennec,nutritional)
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): Postnecrotic(macronodular,posthepatic)cirrhosis
-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,hyperchol: 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 intrahepa: 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.: 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
Asterixis: Hepatic(portosystemic)encephalopathy
-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: Cirrhosis
-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: Cholelithiasis(gallstones)
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: Cirrhosis
-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: Hemangiosarcoma(angiosarcoma)
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: Adenocarcinoma
-benign,rare
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

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