Ch 11 Pathoma Exocrine Pancreas, Gallbladder, and Liver Pathology

Question 1

Annular pancreas is a developmental __ in which the pancreas forms a __ around the __. There is a risk of __.

Answer 1

malformation; ring; duodenum; duodenal obstruction

Question 2

Acute pancreatitis is __ and __ of the pancreas. Due to __ of pancreatic __ by __. __ activation of __ leads to __ of other pancreatic enzymes. Results in __ of the pancreas and __ of the peripancreatic fat.

Answer 2

inflammation; hemorrhage; autodigestion; parenchyma; pancreatic enzymes; premature; trypsin; activation; liquefactive hemorrhagic necrosis; fat necrosis (when fat acids are released they undergo saponification leading to fat necrosis)

Question 3

Acute pancreatitis is most commonly due to __ and __. Other causes include these 7.

Answer 3

alcohol (causes contraction of sphincter of Oddi); gallstones (blocks the ampulla; 1) trauma (MVA in child); 2) hypercalcemia (activator of enzymes); 3) hyperlipidemia; 4) drugs; 5) scorpion stings; 6) mumps; 7) rupture of a posterior duodenal ulcer (pancreas sits posterior to duodenum)

Question 4

Name 5 clinical features of acute pancreatitis

Answer 4

1) epigastric abdominal pain that radiates to back; 2) N/V; 3) periumbilical and flank hemorrhage (necrosis spreads into the periumb soft tissue and retroperitoneum); 4) elevated serum lipase and amylase (lipase is more specific for pancreatic damage); 5) hypocalcemia (consumed during saponification in fat necrosis)

Question 5

Name 4 complications of acute pancreatitis

Answer 5

1) shock (due to peripancreatic hemorrhage and fluid sequestration); 2) pancreatic pseudocyst; 3) pancreatic abscess; 4) DIC and ARDS (enzymes get in to blood and chew up anti-coag factors and the capillary alveolar interface)

Question 6

A pancreatic pseudocyst is a complication of __, which is formed by __ surrounding __ and __. It presents as a __ with __ elevated __. __ is associated with release of enzymes into the abdominal cavity and __.

Answer 6

acute pancreatitis; fibrous tissue; liquefactive necrosis; pancreatic enzymes; abdominal mass; persistently (w/necrosis alone you’d expect enzymes to dec eventually); serum amylase; rupture; hemorrhage

Question 7

Pancreatic abscesses are a complication of __, and are often due to __. Presents with these 3 symptoms.

Answer 7

acute pancreatitis; E coli; 1) abdominal pain; 2) high fever; 3) persistently elevated amylase

Question 8

Chronic pancreatitis is __ of pancreatic __, most often 2ndary to __. Most commonly due to __ (adults) and __ (children), however many cases are __.

Answer 8

fibrosis; parenchyma; recurrent acute pancreatitis; alcohol; cystic fibrosis; idiopathic

Question 9

Name 4 clinical features of chronic pancreatitis. Patients have an increased risk for __.

Answer 9

1) epigastric and abdominal pain that radiates to back; 2) pancreatic insufficiency (results in malabsorption); 3) dystrophic calcification of pancreatic parenchyma on imaging; 4) 2ndary DM; pancreatic carcinoma

Question 10

Pancreatic insufficiency is a clinical feature of chronic pancreatitis. It results in __ with __ and __ deficiencies. __ and __ are/are not useful serologic markers of chronic pancreatitis.

Answer 10

malabsorption; steatorrhea; fat soluble vitamin; amylase; lipase; are not (malabsorption b/c you need pancreatic enzymes to aid in digestion and absorption)

Question 11

Dystrophic calcification of pancreatic parenchyma is a clinical feature of ___. Contrast studies reveal a __ pattern due to __ of pancreatic ducts.

Answer 11

imaging; chain of lakes; dilatation

Question 12

Pancreatic carcinoma is __ arising from the pancreatic __. Most commonly seen in __ (avg age is __ yrs). Major risk factors are __ and __. Serum tumor marker is __. Surgical resection involves __ removal of the __ and __ of pancreas, __ and __ (__ procedure). 1-year survival is __%.

Answer 12

adenocarcinoma; ducts; elderly; 70; smoking; chronic pancreatitis; CA 19-9; en bloc; head and neck; proximal duodenum; gallbladder; Whipple; LT 10%

Question 13

Name 5 clinical features of pancreatic carcinoma (these usually occur late in the disease)

Answer 13

1) epigastric abdominal pain and weight loss; 2) obstructive jaundice w/pale stools and palpable gallbladder (assoc with head of pancreas); 3) 2ndary DM (assoc w/body or tail); 4) pancreatitis; 5) migratory thrombophlebitis (Trousseau syndrome)

Question 14

Pancreatic carcinoma can cause obstructive __ with __ and palpable __. Associated w/tumors that arise in the __ of the pancreas (most common location). 2ndary DM is associated w/tumors that arise in the __.

Answer 14

jaundice (block flow of bile so conjugated bilirubin leaks out); pale stools (stool gets its color from a derivative of bilirubin); gallbladder; head; body or tail

Question 15

Migratory thrombophlebitis (__ syndrome) is a clinical feature of pancreatic carcinoma. It presents w/these 3 symptoms

Answer 15

Trousseau; 1) swelling; 2) erythema; 3) tenderness in extremities

Question 16

Name 4 pathologies of the exocrine pancreas

Answer 16

1) annular pancreas; 2) acute pancreatitis; 3) chronic pancreatitis; 4) pancreatic carcinoma

Question 17

Biliary atresia is failure to __ or early __ of __. Leads to __ within the first __ of life. Presents with __ (due to __ bilirubin) and progresses to __.

Answer 17

form; destruction; extra hepatic biliary tree (failure to form lumen of a tube = atresia); biliary obstruction; 2 months; jaundice; conjugated; cirrhosis (back pressure causes damage)

Question 18

Cholelithiasis (aka __) is solid, round __ in the __. Due to precipitation of __ or __ in __. Arises due to one of these 3 causes.

Answer 18

gallstones; stones; gallbladder; cholesterol; bilirubin; bile; 1) supersaturation of cholesterol or bilirubin; 2) decreased phospholipids (e.g. lecithin) or bile acids (they both help to solubilize the cholesterol); 3) stasis (inc chance of infxn, which can then deconjugate bilirubin and lead to formation of stone)

Question 19

Which are the most common type of stones (90%), especially in the West?

Answer 19

cholesterol stones

Question 20

Cholesterol stones (__ color) are usually radiolucent/radiopaque (10% are __ due to associated __). Risk factors include these 6.

Answer 20

yellow; radiolucent; radiopaque; calcium; 1) age (40s); 2) estrogen (female, obesity, multiple pregnancies, oral contraceptives); 3) clofibrate; 4) Native American ethnicity; 5) Crohn disease; 6) cirrhosis

Question 21

Cholestyramine is a __ lowering agent that works by binding __ and preventing __ at ileum. The decrease in __ increases risk for __ stone. Clofibrate is a __ lowering agent, that ends up increasing the activity of __ enzyme, and decreases conversion of __ to __, which both lead to formation of stones.

Answer 21

lipid; bile acids; recycling; bile acids; cholesterol; lipid; HMG-CoA reductase; cholesterol; bile acids

Question 22

Estrogen increases risk of cholesterol gallstone formation for these 2 reasons.

Answer 22

1) increases activity of HMG-CoA reductase; 2) increases lipoprotein receptors on hepatocytes which increases cholesterol uptake (both supersaturate cholesterol)

Question 23

Crohn’s disease increases risk for cholesterol gall stones by causing damage to the __, which is where bile acids are __. Decreased bile acids leads to less solubilization of __, increasing risk for stone. Cirrhosis can lead to cholesterol stones by decreased production of __ by hepatocytes

Answer 23

terminal ileum; recycled; cholesterol; bile salts

Question 24

Bilirubin stones (pigmented) are usually radiolucent/radiopaque. Risk factors include these 2.

Answer 24

radiopaque; 1) extravascular hemolysis (increased bilirubin in bile); 2) biliary tract infxns (when present in the biliary tract, bacteria can deconjugate the bilirubin and increase risk of precipitated bilirubin)

Question 25

Name 3 causes of biliary tract infection that can lead to bilirubin stones.

Answer 25

1) e coli; 2) ascaris lumbricoides; 3) clonorchis sinensis

Question 26

Ascaris lumbricoides is a common __ that infects 25% of world's population, especially in areas with poor sanitation (\_\_ transmission). Infects \_\_, increasing risk for gallstones. Clonorchis sinensis is endemic in these 3 countries (aka \_\_). Infects the \_\_, increasing risk for these 3 things.

Answer 26

roundworm; fecal-oral; biliary tract; China, Korea, and Vietnam; Chinese liver fluke; biliary tract; 1) gallstones; 2) cholangitis; 3) cholangiocarcinoma

Question 27

Gallstones are usually a/symptomatic. Complications include these 6

Answer 27

1) biliary colic; 2/3) acute and chronic cholecystitis; 4) ascending cholangitis; 5) gallstone ileus; 6) gallbladder cancer

Question 28

Biliary colic is waxing and waning \_\_. Due to gallbladder __ against a __ lodged in the \_\_. Symptoms are relieved if the \_\_. __ obstruction may result in __ or \_\_.

Answer 28

RUQ pain; contracting; stone; cystic duct; stone passes; common bile duct; acute pancreatitis; obstructive jaundice

Question 29

Acute cholecystitis is acute inflammation of the \_\_. Impacted __ in the __ results in dilatation with \_\_, \_\_, and \_\_.

Answer 29

gallbladder wall; stone; cystic duct; pressure ischemia (pressure builds up and bv's are squeezed); bacterial overgrowth (E coli); inflammation

Question 30

Acute cholecystitis presents with these 5 symptoms. Risk of __ is left untreated.

Answer 30

1) RUQ pain often radiating to right scapula; 2) fever w/elevated WBC count; 3/4) N/V; 5) inc serum alkaline phosphatase (epithelium that lines gallbladder and biliary tract contains alk phos which leaks when cells are damaged); rupture

Question 31

Chronic cholecystitis is chronic inflammation of the \_\_. Due to __ from longstanding \_\_, with or without superimposed bouts of \_\_. Characterized by __ of gallbladder __ into the __ (\_\_ sinus).

Answer 31

gallbladder; chemical irritation; cholelithiasis; acute cholecystitis; herniation; mucosa; muscular wall; Rokitansky-Aschoff (pictured)

Question 32

Chronic cholecystitis presents with \_\_, especially after \_\_. __ is a late complication, which is a \_\_, __ gallbladder due to \_\_, \_\_, and \_\_. There is an increased risk for \_\_. Treatment is \_\_, especially if __ gallbladder is present.

Answer 32

vague RUQ pain; eating; porcelain; shrunken, hard; chronic inflammation; fibrosis; dystrophic calcification; carcinoma; cholecystectomy; porcelain (pictured)

Question 33

Ascending cholangitis is a __ of the \_\_. Usually due to ascending __ with \_\_. Presents with these 3 symptoms. Increased incidence with \_\_.

Answer 33

bacterial infxn; bile ducts; infxn; enteric gram-negative bacteria; 1) sepsis (high fever and chills); 2) jaundice; 3) abdominal pain; choledocholithiasis (stone in biliary ducts)

Question 34

Gallstone ileus is when gallstone enters and __ the \_\_. Due to __ with __ formation btwn the __ and \_\_.

Answer 34

obstructs; small bowel; cholecystitis; fistula; gallbladder and small bowel

Question 35

Gallbladder carcinoma is __ arising from the __ that lines the \_\_. __ are a major risk factor, especially when complicated by \_\_. Classically presents as __ in a \_\_. Excellent/poor prognosis.

Answer 35

adenocarcinoma; glandular epithelium; wall; gallstones; porcelain gallbladder; cholecystitis (normally this is a disease of 40s/50s so in elderly think cancer); elderly woman; poor

Question 36

Name 8 pathologies of the gallbladder and biliary tract

Answer 36

1) biliary atresia; 2) cholelithiasis; 3) biliary colic; 4) acute cholecystitis; 5) chronic cholecystitis; 6) ascending cholangitis; 7) gallstone ileus; 8) gallbladder carcinoma

Question 37

Jaundice is __ discoloration of the \_\_. Earliest sign is \_\_. Due to increased serum \_\_, usually GT \_\_. Arises with disturbances in \_\_.

Answer 37

yellow; skin; scleral icterus (yellow discoloration of the sclera); bilirubin; 2.5 mg/dL; bilirubin metabolism

Question 38

Bilirubin metabolism: RBC are consumed by __ of the \_\_. __ (from heme) is converted to \_\_, which is carried by __ to the liver. __ in hepatocytes conjugates \_\_. Conjugated bilirubin is transferred to __ to form \_\_, which is stored in the __ and released into the __ to aid in \_\_.

Answer 38

macrophages; reticuloendothelial system (spleen is most prominent location); protoporphyrin; unconjugated bilirubin (UCB); albumin; uridine glucuronyl transferase (UGT); bilirubin; bile canaliculi; bile; gallbladder; small bowel; digestion

Question 39

Intestinal flora convert conjugated bilirubin to \_\_, which is oxidized to __ (makes __ \_\_ color) and __ (partially reabsorbed into blood and filtered by kidney making __ \_\_ color)

Answer 39

urobilinogen; stercobilin; stool brown; urobilin; urine yellow

Question 40

Name 7 causes of jaundice

Answer 40

1) extravascular hemolysis or ineffective erythropoiesis; 2) physiologic jaundice of newborn; 3) gilbert syndrome; 4) crigler-najjar syndrome; 5) dubin-johnson and rotor syndrome; 6) biliary tract obstruction; 7) viral hepatitis

Question 41

Extravascular hemolysis or \_\_\_, cause high levels of __ and overwhelm the __ ability of the liver. Clinically you would see __ urine due to increased \_\_. Also you would have an increased risk for \_\_.

Answer 41

ineffective erythropoiesis (death of RBCs in marrow due to inability to form RBCs); UCB; conjugating; dark; urine urobilinogen (UCB is not water soluble and thus is absent from urine); bilirubin gallstones (high UCB will eventually cause high CB)

Question 42

Physiologic jaundice of the newborn is caused because the newborn __ has transiently low __ activity. On lab findings you see high \_\_. Clinically, __ can deposit in the __ (\_\_\_) leading to __ deficits and \_\_. Treatment is __ (which works by making __ \_\_)

Answer 42

liver; UGT; UCB; UCB (is fat soluble!!); basal ganglia; kernicterus; neurological; death; phototherapy; UCB; water soluble (phototherapy does not conjugate UCB to CB!)

Question 43

Gilbert syndrome is caused by \_\_. PoI. Labs show increased \_\_. Patients get jaundice during __ (e.g. severe infxn), otherwise not clinically significant

Answer 43

mildly low UGT activity (enzyme that performs bilirubin conjugation); autosomal recessive; UCB; stress

Question 44

Crigler-Najjar syndrome is caused by \_\_. Labs show increased \_\_. Clinically it causes \_\_. Usually \_\_.

Answer 44

absence of UGT; UCB; kernicterus; fatal

Question 45

Dubin-Johnson syndrome is a deficiency of __ protein. PoI. Labs show increased \_\_. Clinically the liver is \_\_. Rotor syndrome is similar but lacks \_\_.

Answer 45

bilirubin canalicular transport; autosomal recessive; CB; dark (otherwise not clinically significant); liver discoloration

Question 46

Biliary tract obstruction (aka \_\_) is associated with these 5 things.

Answer 46

1) gallstones; 2) pancreatic carcinoma; 3) cholangiocarcinoma; 4) parasites; 5) liver fluke (clonorchis sinensis)

Question 47

On labs, biliary tract obstructions shows H/N/L CB, urine urobilinogen, alkaline phosphatase. Clinical features include these 5

Answer 47

high CB; low urine urobilinogen; high alk phos; 1) dark urine (due to bilirubinemia); 2) pale stool; 3) pruritis due to inc plasma bile acids; 4) hypercholesterolemia with xanthomas; 5) steatorrhea w/malabsorption of fat-soluble vitamins (things in bile = bile acids, bilirubin, and cholesterol - all leak in to blood)

Question 48

Viral hepatitis causes inflammation, which disrupts __ and \_\_. Lab findings show increased __ and __ (which one is due to which?). Pts have __ urine due to inc \_\_. Urine urobilinogen is H/N/L.

Answer 48

hepatocytes; small bile ductules; CB (bile ductule damage); UCB (hepatocyte damage); dark; urine bilirubin (CB, since UCB is not water soluble); normal or decreased

Question 49

Which causes of jaundice cause increased UCB (5)? Which cause inc CB (3)?

Answer 49

1) extravascular hemolysis/ineffective erythropoiesis; 2) newborn; 3) gilbert; 4) crigler-N; 5) viral hep 1) dubin-johnson/rotor; 2) biliary tract obstruct; 3) viral hep

Question 50

Which cause of jaundice causes inc alk phos? Which cause dark urine? Which are autosomal recessive? Which cause kernicterus? Which causes dark liver? Hypercholesterolemia w/xanthomas?

Answer 50

biliary tract obstruction (probably potentially viral hep); extravascular hemolysis/ineffective EPO, biliary tract obstruct, viral hep; gilbert and dubin-johnson; kernicterus: crigler-najjar and newborn; dark = dubin-johnson; biliary tract obstruct

Question 51

Viral hepatitis is __ of the liver \_\_, usually due to \_\_. Other causes include __ and \_\_. Hepatitis virus causes \_\_, which may progress to \_\_.

Answer 51

inflammation; parenchyma; hepatitis virus; EBV and CMV; acute hepatitis; chronic hepatitis;

Question 52

Acute hepatitis presents with these 6 symptoms

Answer 52

1) jaundice due to mixed CB and UCB; 2) dark urine due to CB; 3) fever; 4) malaise; 5) nausea; 6) elevated liver enzymes (ALT more than AST)

Question 53

Inflammation of acute viral hepatitis involves __ of the liver and __ and is characterized by __ of \_\_. Some cases may be asymptomatic with \_\_. Symptoms last \_\_.

Answer 53

lobules; portal tracts; apoptosis; hepatocytes; elevated liver enzymes; less than 6 months

Question 54

Chronic hepatitis is characterized by symptoms that last \_\_. Inflammation predominantly involves \_\_. Risk of progression to \_\_.

Answer 54

GT 6mo; portal tract; cirrhosis

Question 55

Hepatitis A (HAV) and hepatitis E (HEV) are transmitted \_\_. HAV is commonly acquired by \_\_, and HEV is commonly acquired from __ or \_\_. They both cause __ and do not cause \_\_.

Answer 55

fecal-orally; travelers; contaminated water; undercooked seafood; acute hepatitis; chronic state

Question 56

In HAV and HEV, __ marks active infection. __ is protective, and its presence indicates __ or \_\_. HEV infxn in __ is associated with fulminant hepatitis (liver failure with \_\_)

Answer 56

anti-virus IgM; anti-virus IgG; prior infection; immunization (immunization is available for HAV); pregnant women; massive liver necrosis

Question 57

Hepatitis B (HBV) is transmitted __ (name 4 ex's).

Answer 57

parenterally; 1) childbirth; 2) unprotected intercourse; 3) IV drug abuse; 4) needle stick

Question 58

HBV results in \_\_. __ disease occurs in \_\_% of cases. In acute HBV what is the first serologic marker to rise

Answer 58

acute hepatitis; chronic; 20; HBsAG

Question 59

HBsAG, HBeAG and HBV DNA, HBcAB, HBsAB: which are positive in acute HBV?

Answer 59

HBsAg + (first serologic marker to rise); HBeAG and HBV DNA +; HBcAB IgM; HBsAB neg

Question 60

HBsAG, HBeAG and HBV DNA, HBcAB, HBsAB: which are positive in window HBV (aka btwn acute and either chronic or resolved)?

Answer 60

first two neg; HBcAB IgM; HBsAB neg

Question 61

HBsAG, HBeAG and HBV DNA, HBcAB, HBsAB: which are positive in resolved HBV?

Answer 61

first two neg; HBcAG: IgG; HBsAB IgG (protective)

Question 62

HBsAG, HBeAG and HBV DNA, HBcAB, HBsAB: which are positive in chronic HBV?

Answer 62

HBsAG + (presence gt 6 mo defines chronic state); HBeAG and HBV DNA: +/- (presence of HBeAG or HBV DNA indicated infectivity); HBcAB IgG; HBsAB neg

Question 63

HBsAG, HBeAG and HBV DNA, HBcAB, HBsAB: which are positive in immunized HBV?

Answer 63

first three neg; IgG (protective)

Question 64

What is the difference in serologic markers for HBV in a pt who was infected and resolved vs a pt who was immunized?

Answer 64

Resolved would have IgG for HBcAB and HBsAB; immunized would only have IgG for HBsAB

Question 65

HCV is transmitted \_\_. Risk from __ is almost nonexistent due to __ of blood supply. HCV results in \_\_. __ disease occurs in __ cases.

Answer 65

parenterally (IVDA, unprotected intercourse); transfusion; screening

Question 66

In HCV, __ test confirms infxn. Decreased __ levels indicates recovery. __ indicates chronic disease.

Answer 66

HCV-RNA; RNA; persistence

Question 67

HDV is dependent on __ for infxn. __ upon existing __ is more/less severe than __ (infxn with __ and __ at same time).

Answer 67

HBV; superinfection; HBV; more; confection; HBV and HDV

Question 68

Cirrhosis is __ characterized by disruption of the normal __ by __ and \_\_. Fibrosis is mediated by __ from \_\_, which lie above/beneath the __ cells that line the \_\_.

Answer 68

end-stage liver damage; hepatic parenchyma; bands of fibrosis; regenerative nodules of hepatocytes; TGF-B' stellate cells; beneath; endothelial; sinusoids

Question 69

Name 3 broad categories of clinical features of cirrhosis

Answer 69

1) portal hypertension; 2) decreased detoxification; 3) decreased protein synthesis

Question 70

Portal hypertension from cirrhosis leads to these 4 things.

Answer 70

1) ascites (fluid in peritoneal cavity); 2) congestive splenomegaly/hypersplenism (spleen starts consuming rbcs and plts); 3) portosystemic shunts (esophageal varices, hemorrhoids, caput medusae); 4) hepatorenal syndrome (rapidly developing renal failure 2ndary to cirrhosis)

Question 71

Decreased detoxification abilities of the liver due to cirrhosis results in these 3 things

Answer 71

1) mental status changes, asterixis, eventual coma (due to inc serum ammonia) - metabolic, hence reversible; 2) hyperestrinism (liver removes estrogen) causing gynecomastia, spider angiomata, palmar erythema; 3) jaundice (dec ability to conjugate bilirubin)

Question 72

Decreased protein synthesis abilities of the liver due to cirrhosis results in these 2 things.

Answer 72

1) hypoalbuminemia with edema; 2) coagulopathy due to dec synthesis of clotting factors (degree of deficiency followed by PT)

Question 73

In the liver, vitamin __ is activated by \_\_, which then activates coagulation factors (name them) by \_\_. __ blocks \_\_.

Answer 73

K; epoxide reductase (produced by liver); 2, 7, 9, 10, C + S; gamma carboxylation; warfarin blocks epoxide reductase

Question 74

What is the most common cause of liver disease in the West?

Answer 74

alcohol-related liver disease

Question 75

Alcohol related liver disease is damage to hepatic __ due to \_\_. __ liver is the accumulation of __ in hepatocytes, which results in a heavy, __ liver. Resolves with \_\_. __ is a complication of long-term, chronic alcohol-induced liver damage. Occurs in \_\_% of alcoholics.

Answer 75

parenchyma; consumption of alcohol; fatty (pic in front); fat; greasy; abstinence; cirrhosis; 10-20

Question 76

Alcoholic hepatitis results from __ injury to hepatocytes (different than fatty liver). Generally seen with \_\_. __ (metabolite of alcohol) mediates damage. Characterized by __ of hepatocytes w/formation of __ (damaged \_\_), __ and \_\_.

Answer 76

chemical; binge drinking; acetaldehyde; swelling; Mallory bodies (pictured); cytokeratin filaments; necrosis; acute inflammation

Question 77

Alcoholic hepatitis presents with __ and elevated \_\_. __ is greater than __ because __ is produced in the \_\_, and alcohol is a __ poison. Alcoholic hepatitis may result in \_\_.

Answer 77

painful hepatomegaly; liver enzymes; AST gt ALT: AST; mitochondria; mitochondrial; death

Question 78

Nonalcoholic fatty liver disease is \_\_, \_\_, and /or __ that develop without exposure to __ (or other known \_\_). Associated with \_\_. Diagnosis of \_\_. ALT gt/lt AST

Answer 78

fatty change; hepatitis; cirrhosis; alcohol; insult; obestiy; exclusion; ALT gt AST

Question 79

Hemochromatosis is excess body __ leading to deposition in __ (\_\_) and organ __ (\_\_). Tissue damage is mediated by generation of __ (via the __ rxn).

Answer 79

iron; tissues; hemosiderosis; damage; hemochromatosis; free radicals (fenton rxn)

Question 80

Hemochromatosis is due to PoI defect in __ absorption (primary) or __ (secondary). Primary hemochromatosis is due to mutations in the __ gene, usually __ (\_\_ is replaced by __ at amino acid \_\_)

Answer 80

autosomal recessive; iron; chronic transfusions; HFE; C282Y; cysteine; tyrosine; 282

Question 81

Hemochromatosis presents in early/late adulthood. Name the classic triad. Other findings include these 3.

Answer 81

late adulthood; 1) cirrhosis; 2) secondary DM; 3) bronze skin; 1) dilated cardiomyopathy; 2) cardiac arrythmias; 3) gonadal dysfunction (due to testicular atrophy)

Question 82

In hemochromatosis, labs show H/N/L ferritin, TIBC, serum iron, and % saturation. Pts have an increased risk of \_\_. Treatment is \_\_.

Answer 82

high ferritin; low TIBC; high serum iron; high % sat; hepatocellular carcinoma (free radicals can damage DNA); phlebotomy (remove RBCs which all contain iron)

Question 83

In hemochromatosis liver bx reveals accumulation of __ in \_\_. Similar thing is seen with \_\_, a __ that is a by-product from the turnover of ___ (wear and tear). To differentiate the two, __ stain distinguishes them, with iron turning \_\_.

Answer 83

brown pigment; hepatocytes; lipofuscin; brown pigment; peroxidized lipids (commonly present in hepatocytes); Prussian blue (pictured); blue

Question 84

Wilson disease is a PoI defect (\_\_ gene) in \_\_-mediated hepatocyte \_\_. Results in a lack of __ into __ and lack of __ incorporation into \_\_.

Answer 84

AR; ATP7B; ATP; copper transport; copper transport; bile; copper; ceruloplasmin (carrier of copper in blood)

Question 85

In Wilson disease, __ builds up in \_\_, leaks into \_\_, and deposits in \_\_. \_\_-mediated production of __ leads to tissue damage. Pts have an increased risk of \_\_. Treatment is \_\_, which works by \_\_.

Answer 85

copper; hepatocytes; serum; tissues; copper; hydroxyl free radicals; hepatocellular carcinoma; D-penicillamine; chelating copper

Question 86

Wilson disease presents in __ with these 3 symptoms (be specific)

Answer 86

childhood; 1) cirrhosis; 2) neurologic manifestations (behavioral changes, dementia, chorea, and Parkisonian symptoms due to deposition of copper in basal ganglia); 3) Kayser-Fleisher rings in the cornea

Question 87

Primary biliary cirrhosis is __ \_\_ destruction of \_\_. Classically arises in women/men (avg age __ yrs). Associated with other \_\_. Etiology is \_\_. __ is present. Presents with features of \_\_. __ is a late complication.

Answer 87

autoimmune granulomatous; intrahepatic bile ducts; women; 40yrs; autoimmune diseases; unknown; anti-mitochondrial antibody; obstructive jaundice; cirrhosis

Question 88

Primary sclerosing cholangitis is __ and __ of __ and \_\_. Periductal __ with a __ appearance. Uninvolved regions are dilated resulting in a __ appearance on contrast imaging.

Answer 88

inflammation; fibrosis; intrahepatic; extrahepatic bile ducts; fibrosis; onion skin; beaded;

Question 89

In primary sclerosing cholangitis, the etiology is \_\_. It is associated with \_\_, and __ is often positive. Presents with \_\_. __ is a late complication. Increased risk for \_\_.

Answer 89

unknown; ulcerative colitis; p-ANCA; obstructive jaundice; cirrhosis; cholangiocarcinoma (cancer of bile ducts)

Question 90

Reye syndrome is fulminant __ and __ in __ with __ illness who take \_\_. Likely related to __ of hepatocytes. Presents with these 3 symptoms and may progress to __ and \_\_. What is the one exception where we give children \_\_?

Answer 90

liver failure; encephalopathy; children; viral; aspirin; mitochondrial damage; 1) hypoglycemia; 2) elevated liver enzymes; 3) N/V; coma; death; aspirin; Kawasaki syndrome

Question 91

Hepatic adenoma is a __ tumor of \_\_. Associated with __ use. Regresses upon cessation of \_\_. Risk of __ and \_\_, especially during \_\_. Tumors are __ and grow with exposure to \_\_.

Answer 91

benign; hepatocytes; oral contraceptive; drug (oral contraceptive); rupture; intraperitoneal bleeding; pregnancy (when there are high levels of estrogen); sub capsular (can rupture through capsule and cause intraperitoneal bleeding); estrogen

Question 92

Hepatocellular carcinoma is a __ tumor of \_\_. Name 3 risk factors. Tumors are often detected early/late because symptoms are masked by \_\_. Good/poor prognosis. Serum tumor marker is \_\_.

Answer 92

malignant; hepatocytes; 1) chronic hepatitis (HBV or HCV); 2) cirrhosis; 3) aflatoxins derived from aspergillus (induce p53 mutations); late; cirrhosis; poor; alpha-fetoprotein (AFP)

Question 93

Name 6 causes of cirrhosis

Answer 93

1) alcohol; 2) nonalcoholic fatty liver disease; 3) hemochromatosis; 4) Wilson disease; 5) A1AT deficiency; 6) chronic hepatitis

Question 94

Hepatocellular carcinoma causes an increased risk for __ syndrome, which causes liver __ secondary to \_\_. Presents with painless/ful __ and \_\_.

Answer 94

Budd-Chiari; infarction; hepatic vein obstruction; painful; hepatomegaly; ascites

Question 95

Metastasis to liver is more/less common than primary liver tumors. MC sources include these 4. Results in single/multiple __ in the liver. Clinically may be detected as __ with a __ of the liver.

Answer 95

more; colon, pancreas, lung, and breast carcinomas; multiple nodules; hepatomegaly; nodular free-edge (can palpate the nodules on physical exam)

Question 96

Of liver diseases, which ones is due to a mutation in HFE gene, usually C282Y? Which one due to a mutation in ATP7B gene? Causes an onion skin appearance on bx? Antimitochondrial antibody is present? Is caused by aflatoxins from aspergillus? Subcapsular tumor?

Answer 96

primary hemochromatosis; Wilson disease; primary sclerosing cholangitis; primary biliary cirrhosis; aflatoxins = HCC; hepatic adenoma

Question 97

Of liver diseases, which one is AST gt ALT? Which is a diagnosis of exclusion? Has AFP as a marker? Stains blue with prussian blue stain? Is associated with oral contraceptive use? Is treated with D-penicillamine? Tx with phlebotomy?

Answer 97

alcohol related liver disease; nonalcoholic fatty liver disease; HCC; hemochromatosis; hepatic adenoma; Wilson disease; hemochromatosis