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GIR Test 3 > B5-017 Hepatic and Gallbladder Physiology > Flashcards

B5-017 Hepatic and Gallbladder Physiology Flashcards

1
Q

large capillary betwen plates of hepatocytes

A

liver sinusoids

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2
Q

drains filtered blood into hepatic vein and IVC

A

central vein

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3
Q

polarized parenchymal cells

A

hepatocytes

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4
Q

bile duct epithelial cells

A

cholangiocytes

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5
Q

liver resident macrophages

A

Kupffer cells

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6
Q

fenestrated endothelial cells

A

sinusoidal endothelial cells

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7
Q
  • stores fat soluble vitamins
  • produces collagen
A

stellate cells

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8
Q

what types of metabolism occur in zone 1?

3

A
  • fatty acid oxidation
  • gluconeogenesis
  • bile acid uptake
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9
Q

what types of metabolism occur in zone 2?

A
  • fatty acid synthesis
  • glycolysis
  • bile acid synthesis
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10
Q

prevents reflux of duodenal contents into the pancreatic and bile ducts

A

spincter of Oddi

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11
Q

three organic solutes important to bile formation

A
  • cholesterol
  • bile salts
  • phospholipids
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12
Q
  • glycosylated protein forming gel lubrication barrier
  • found in bile
A

mucin

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13
Q

proteins found in bile

4

A
  • IgA, IgM, IgG
  • mucin
  • albumin
  • apolipoproteins
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14
Q

precursor of sex hormones, arenal hormones, vitamin D, and bile acids

A

cholesterol

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15
Q

hepatic cholesterol sources of input

A

diet
de novo synthesis

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16
Q

hepatic cholesterol output

A

biliary secretion and bile acid synthesis

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17
Q

rate limiting enzyme in cholesterol formation

A

HMG CoA reductase

statins inhibit

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18
Q

major CYP pathway of cholesterol -> bile acid synthesis

A

CYP7A1

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19
Q

primary bile acids

2

A

CDCA
CA

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20
Q

secondary bile acids

2

A

DCA
LCA

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21
Q

how much bile acid is eliminated through feces?

%

A

5%

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22
Q

how much cholesterol is eliminated through feces?

%

A

50%

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23
Q

secretion via I cells is stimulated by dietary fats and amino acids

A

CCK

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24
Q
  • stimulates gallbladder contraction
  • relaxes spinchter of Oddi
A

CCK

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25
Q
  • inhibits gastric secretion to promote digestion
  • induces satiety
A

CCK

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26
Q

stimulates HMG CoA reductase and LDLR to increase de novo cholesterol synthesis and uptake of lipoprotein

during low cholesterol

A

SREBP2

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27
Q

stimulates ABCG5/G8 and CYP7A1 to increase cholesterol catabolism and secretion

during high cholesterol

A

LXR

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28
Q

induces FGF19 to inhibit CYP7A1 in hepatocytes

A

bile-acid activated FXR

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29
Q

bile activated FXR induces […] to increase bile acid secretion

A

BSEP

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30
Q

bile activated FXR induces […] to promote bile acid transport

A

OST/aB

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31
Q

bind negatively charge bile acids to prevent bile acid reabsorption

A

bile acid sequestrants

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32
Q

defects in micelle synthesis will cause

2

A

hypercholesterolemia
artherosclerosis

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33
Q

defects in the hepatic transport of mixed micelles will cause

A

cholestasis

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34
Q

defects in intestinal absorption with mixed micelles cause

A

chronic diarrhea

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35
Q

enzyme that converts unconjugated bilirubin to conjugated bilirubin

A

UGT

36
Q

most common cause of neonatal cholestasis

A

biliary atresia

37
Q
  • elevated conjugated bilirubin
  • jaundice
  • first 8 weeks of life
  • dark urine
  • pale stool
A

biliary atresia

38
Q
  • male dominant
  • average age of dx: 30-40
  • associated with IBD
  • p-ANCA
A

primary sclerosing cholangitis

39
Q
  • female dominant
  • average age of diagnosis: 35-70
  • associated with frequent UTIs
  • AMA antibody
A

primary biliary cholangitis

40
Q
  • pruritis in 3rd trimester -> jaundice
  • elevation of AST, ALT, bile acid
A

intrahepatic cholestatis of pregnancy

41
Q
  • progressive liver disease leading to liver failure
  • genetic defects in bile secretion
  • pruritis, jaundice, FTT, cirrhosis, portal HTN, hepatomegaly
A

progressive familial intrahepatic cholestasis

42
Q
  • defect in ATP8B1
  • extrahepatic manifestations (diarrhea, pancreatitis, etc.)
  • no gallstones
A

PFIC1 (byler disease)

43
Q
  • BSEP (ABC11) mutation
  • gallstones
  • high incidence of HCC and cholangiocarcinoma
A

PFIC2 (Byler syndrome)

44
Q
  • MDR3 (ABCB4) mutation
  • gallstones
  • elevated GGT
  • decreased biliary phospholipids
A

PFIC3

45
Q
  • Mrp2 mutation
  • elevated conjugated bili
  • normal total urine coproporphyrin with elevated isomer I fraction
  • black liver
A

Dubin-johnson syndrome

46
Q
  • OATP1B1/3 mutation
  • elevated conjugated bilirubin
  • elevated total urine coproporphyrin with Isomer I fraction
  • visualized on oral cholecystography
A

rotor syndrome

47
Q
  • defect in UGT1A1
  • extremely high unconjugated bilirubin
A

Crigler-Najjar Syndrome

48
Q
  • defect in UGT1A1
  • elevated unconjugated bilirubin
  • episodic jaundice under stress
A

Gilbert syndrome

49
Q

most common reason for pediatric liver transplantation

A

biliary atresia

50
Q
  • newborn
  • persistant jaundice after 2 weeks of life
  • darkening urine
  • acholic stools
  • hepatosplenomegaly
A

biliary atresia

51
Q
  • asymptomatic/mild jaundice with stress, fasting, illness
  • mildy decreased UDP glucuronosyltrasnferase conjugation
  • relatively common, benign condition
A

Gilbert

52
Q
  • absent UDP-glucuronosyltrasnferase
  • presents early in life, but may not have neurologic symptoms until later in life
  • jaudice, kernicterus
  • increased unconjugated bilirubin
A

Crigler-Najjar

type 1, type 2 less severe

53
Q
  • conjugated hyperbilirubinemia due to defective liver excretion
  • grossly black Dark liver due to impaired extretion of epinephrine metabolites
A

Dubin-Johnson syndrome

54
Q

AMA is sensitive and specific for

A

PBC

55
Q

occurs 3rd trimester and patients present with itching

A

intrahepatic cholestasis

56
Q
  • decreased biliary bile acid secretion causes intrahepatic accumulation of bile acids
  • causes basolateral bile efflux and elevated serum bile acid
A

intrahepatic cholestasis

57
Q

what test should be ordered if intrahepatic cholestasis is suspected?

A

fasting serum bile acids

58
Q

RUQ ultrasound and MRCP can be used to rule out

A

extrahepatic biliary obstruction

59
Q
  • infant
  • jaundice
  • dark urine
  • clay-colored stool
  • elevated conjugated/direct bilirubin
A

congenital extrahepatic biliary atresia

60
Q

causes gallbladder contraction and relaxation of ampulla of Vater leading to bile secretion

A

CCK

61
Q

standard treatment for patients with acute cholecystitis

A

IV fluid, antibiotics, observe

62
Q

if a patient with acute cholecystitis improves within 48 hrs,…

A

schedule elective cholecystectomy

63
Q

procedure used to diagnose and treat problems in liver, gallbladder, bile ducts, pancreas

A

ERCP

64
Q

used to dissolve small, non-calcified cholesterol gallstones in poor surgical candidates

A

UDCA

65
Q

caused by Mrp2 mutation

A

Dubin-Johnson

66
Q

loss of UGT1A1 causes

A

Gilbert’s syndrome

67
Q

Gilbert’s syndrome is associated with elevated […] bilirubin

A

indirect

68
Q

Dubin-Johnson causes elevated […] bilirubin

A

direct

69
Q

caused by ATP8B1 mutation

A

PFIC1 (byler disease)

70
Q
  • elevated transaminases
  • normal GGT
  • elevated serum bile acid
  • elevated indirect bilirubin
A

PFIC1

71
Q

gold standard for diagnosis and treatment of common bile duct obstruction

A

ERCP

72
Q

if cholangitis is suspected, what should be performed?

A

ERCP

73
Q

charcot’s triad has high specificity for

A

acute cholangitis

74
Q

decreases pruritis

A

UDCA

75
Q

acute cholangitis most commonly arises from

A

choledocholithiasis

also can be caused by tumor or biliary stricture

76
Q

epigastric pain
elevated lipase/amylase

A

pancreatitis

77
Q

takes up bile acids at the apical side of the enterocyte

A

ASBT

78
Q

takes up cholesterol at the apical side of the enterocyte

A

NPC1L1

then cholesterol effluxes out basolateral side and enters lymphatics

79
Q

effluxes cholesterol into lumen at apical side of enterocyte

A

ABCG5/G8

80
Q

takes up bile acids into hepatocyte

A

NTCP

81
Q

effluxes bile acids out basolateral side of enterocyte into portal circulation

A

OSTa/B

82
Q

transports bilirubin into cannaliculi

A

MRP2

83
Q

transports bile acids into cannaliculi

A

BSEP

84
Q

transports phospholipids into cannaliculi

A

MDR3

85
Q

transports cholesterol into cannaliculi

A

ABCG5/G8

GIR Test 3 (16 decks)

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