Ch 11 Pathoma Exocrine Pancreas, Gallbladder, and Liver Pathology Flashcards

1
Q

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

A

malformation; ring; duodenum; duodenal obstruction

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2
Q

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.

A

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)

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3
Q

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

A

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)

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4
Q

Name 5 clinical features of acute pancreatitis

A

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)

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5
Q

Name 4 complications of acute pancreatitis

A

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)

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6
Q

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 __.

A

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

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7
Q

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

A

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

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8
Q

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

A

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

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9
Q

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

A

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

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10
Q

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.

A

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

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11
Q

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

A

imaging; chain of lakes; dilatation

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12
Q

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 __%.

A

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

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13
Q

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

A

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)

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14
Q

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 __.

A

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

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15
Q

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

A

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

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16
Q

Name 4 pathologies of the exocrine pancreas

A

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

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17
Q

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

A

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)

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18
Q

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

A

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)

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19
Q

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

A

cholesterol stones

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20
Q

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

A

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

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21
Q

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.

A

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

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22
Q

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

A

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

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23
Q

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

A

terminal ileum; recycled; cholesterol; bile salts

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24
Q

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

A

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)

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25
Q

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

A

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

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26
Q

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.

A

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

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27
Q

Gallstones are usually a/symptomatic. Complications include these 6

A

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

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28
Q

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

A

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

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29
Q

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

A

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

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30
Q

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

A

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

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31
Q

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

A

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

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32
Q

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.

A

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

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33
Q

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

A

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)

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34
Q

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

A

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

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35
Q

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.

A

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

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36
Q

Name 8 pathologies of the gallbladder and biliary tract

A

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

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37
Q

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

A

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

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38
Q

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 __.

A

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

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39
Q

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

A

urobilinogen; stercobilin; stool brown; urobilin; urine yellow

40
Q

Name 7 causes of jaundice

A

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

41
Q

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 __.

A

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)

42
Q

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 __ __)

A

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

43
Q

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

A

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

44
Q

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

A

absence of UGT; UCB; kernicterus; fatal

45
Q

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

A

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

46
Q

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

A

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

47
Q

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

A

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)

48
Q

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.

A

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

49
Q

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

A

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

50
Q

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?

A

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

51
Q

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

A

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

52
Q

Acute hepatitis presents with these 6 symptoms

A

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)

53
Q

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

A

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

54
Q

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

A

GT 6mo; portal tract; cirrhosis

55
Q

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 __.

A

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

56
Q

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 __)

A

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

57
Q

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

A

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

58
Q

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

A

acute hepatitis; chronic; 20; HBsAG

59
Q

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

A

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

60
Q

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

A

first two neg; HBcAB IgM; HBsAB neg

61
Q

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

A

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

62
Q

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

A

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

63
Q

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

A

first three neg; IgG (protective)

64
Q

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

A

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

65
Q

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

A

parenterally (IVDA, unprotected intercourse); transfusion; screening

66
Q

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

A

HCV-RNA; RNA; persistence

67
Q

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

A

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

68
Q

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

A

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

69
Q

Name 3 broad categories of clinical features of cirrhosis

A

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

70
Q

Portal hypertension from cirrhosis leads to these 4 things.

A

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)

71
Q

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

A

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)

72
Q

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

A

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

73
Q

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

A

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

74
Q

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

A

alcohol-related liver disease

75
Q

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.

A

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

76
Q

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 __.

A

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

77
Q

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

A

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

78
Q

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

A

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

79
Q

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

A

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

80
Q

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 __)

A

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

81
Q

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

A

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

82
Q

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

A

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

83
Q

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 __.

A

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

84
Q

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

A

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

85
Q

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 __.

A

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

86
Q

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

A

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

87
Q

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.

A

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

88
Q

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

A

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

89
Q

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

A

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

90
Q

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 __?

A

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

91
Q

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 __.

A

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

92
Q

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 __.

A

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

93
Q

Name 6 causes of cirrhosis

A

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

94
Q

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

A

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

95
Q

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.

A

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

96
Q

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?

A

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

97
Q

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?

A

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