04c: Biliary Tree, Autoimmune Flashcards

1
Q

Bile composed mainly of (water/lipid). The major components are:

A

Water

  1. Bile acids
  2. Phospholipids (lecithins)
  3. Cholesterol
  4. Bile pigments (BR)
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2
Q

T/F: Cholesterol accounts for over 50% of the organic components of bile.

A

False - present only in small amounts (4%)

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3
Q

T/F: Bile acids account for over 50% of the organic components of bile.

A

True

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4
Q

Cholic and chenodeoxycholic acid are examples of (X), synthesized from (Y).

A
X = Primary bile acids
Y = cholesterol
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5
Q

Deoxycholic and lithocholic acid are examples of (X), synthesized from (Y).

A
X = Secondary bile acids
Y = primary bile acids
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6
Q

Bile acids are made more (hydrophobic/hydrophilic) by (X) process in the liver, which converts them to (Y).

A

Hydrophilic
X = conjugation (with taurine or glycine)
Y = bile salts

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7
Q

What’s the purpose of bile acid conjugation in the liver?

A

Permits their accumulation within intestine to facilitate fat digestion/absorption

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8
Q

Bile salts are more (hydrophilic/hydrophobic) than bile acids, with (higher/lower) pKa and (increased/decreased) resistance to pancreatic enzyme hydrolysis.

A

Hydrophilic;
Lower (remain ionized in intestine at physiological pH);
Increased

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9
Q

T/F: Bile acids returning to liver is negative feedback for additional secretion of bile.

A

False - potent stimulus of additional bile secretion

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10
Q

T/F: Bile acids returning to liver is negative feedback for additional synthesis of bile acids.

A

True

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11
Q

(Liver/gallbladder) concentrates bile by actively (reabsorbing/secreting) (X).

A

Gallbladder
Reabsorbing
X = Na, Cl, HCO3
(Water follows)

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12
Q

Major stimulus for gallbladder contraction and (X) sphincter relaxation.

A

X = sphincter of Oddi

CCK

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13
Q

(Active/passive) bile acid reabsorption occurs specifically at (X) whereas (active/passive) reabsorption occurs throughout (small/large) intestine.

A

Active (Na-bile ATPase/cotransporter);
X = terminal ileum
Passive;
Small (and some in large) intestine

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14
Q

Two basic types of gallstones: (star the more common type in Western societies)

A
  1. Cholesterol* (75%)

2. Pigment

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15
Q

Basic underlying problem in cholesterol gallstone formation is:

A

Failure of bile constituents to maintain cholesterol in solution

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16
Q

RFs for cholesterol gallstone formation.

A
  1. MAINLY: 4F’s (forty, fat, F, fertile)
  2. Increasing age
  3. Rapid weight loss
  4. Ileal disease
  5. Genetics
  6. Certain drugs
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17
Q

FHx of cholesterol gallstone formation indicates potential mutation in gene encoding (X), leading to extremely (high/low) (Y).

A

X = hepatocanalicular phosphatidylcholine transporter (ABCB4)
Low;
Y = biliary phosphatidylcholine

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18
Q

List some drugs that increase risk of cholesterol gallstone formation.

A
  1. Fibrates
  2. Estrogen
  3. Oral contraceptives
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19
Q

Pigment gallstones are (black/brown).

A

Both can occur (two different subtypes)

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20
Q

Major component in pigment gallstones:

A

Ca bilirubinate

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21
Q

Pathogenesis of pigment gallstones is believed to involve (conjugation/deconjugation) and (X) of (Y).

A

Deconjugation;
X = precipitation
Y = BR

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22
Q

RFs associated with black pigment gallstones.

A
  1. Chronic hemolysis (SCD, thalassemia)
  2. Cirrhosis
  3. CF
  4. Long-term total parenteral nutrition
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23
Q

RFs associated with brown pigment gallstones.

A
  1. Biliary stasis/infection
  2. Choledochal cysts
  3. Periampullary diverticulum
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24
Q

T/F: Most (2/3) of patients with gallstones are asymptomatic.

A

True

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25
Q

Biliary colic is (relieved/provoked) by food intake. How might the patient describe nature/location of pain?

A

Provoked;

Episodic and severe with sudden onset, steep rise, and steady plateau that lasts for hours; usually located in epigastrium or RUQ (radiates to upper back)

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26
Q

T/F: Biliary colic is thought to be the result of hypoxia.

A

False - tonic spasm around a transiently obstructed cystic duct

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27
Q

Patient presenting with biliary colic. What clinical signs would make you entertain diagnosis of acute cholecystitis?

A

Fever (or signs of inflammation) and pain lasting over 6h

28
Q

What’s Choledocholithiasis?

A

Common bile duct stone

29
Q

T/F: All common bile duct stones came from gallbladder.

A

False - may be primary (originating in CBD - usually brown pigment stones)

30
Q

What’s cholangitis?

A

Bacterial infection of bile ducts

31
Q

Charcot’s triad, for diagnosis of (X):

A

X = cholangitis

  1. Fever
  2. Jaundice
  3. RUQ
32
Q

Reynold’s pentad adds which additional symptoms to Charcot’s triad? This is indicative of (X).

A

Hypotension and altered mental status

X = severe, life-threatening cholangitis

33
Q

Common Rx for acute cholangitis includes:

A
  1. Broad-spectrum antibiotics

2. Biliary decompression (endoscopic preferred over surg)

34
Q

T/F: Gallstones are one of the most common causes of acute pancreatitis.

A

True - obstruction of pancreatic duct when stone passes through ampulla

35
Q

Diagnostic imaging study of choice for gallstone detection:

A

Ultrasound

36
Q

List the 3 ultrasonographic criteria for gallstone diagnosis.

A
  1. Echogenic foci
  2. Cast acoustic shadow
  3. Gravitational dependency
37
Q

Cholescintigraphy or “HIDA scan” is considered a first-line test for diagnosing (X), though ultrasound is usually preferred.

A

X = acute cholecystitis

38
Q

What would you expect to see on cholescintigraphy (HIDA scan) in case of obstructed cystic duct?

A

No visualization of gallbladder (cannot take up tracer)

39
Q

MRCP (Magnetic Resonance Cholangiopancreatography) is (invasive/non-invasive) imaging method that’s comparable to (X) method. Its optimal use is in patients with (low/intermediate/high) risk of gallstones.

A

Non-invasive (safe);
X = ERCP (but without therapeutic capability)

Intermediate (good screening tool)

40
Q

(X) imaging method is likely the best choice for patients with high probability of CBD stone. Why?

A

X = ERCP (Endoscopic Retrograde Cholangiopancreatography)

Therapeutic capabilities (sphincterotomy, stone extraction, stenting, etc.)

41
Q

Patient with CBD gallstone suddenly develops worsened epigastric pain (radiating to back) with N/V. Which tests/imaging would you order to confirm diagnosis of acute gallstone pancreatitis?

A

Amylase/lipase;

Imaging not required to establish diagnosis

42
Q

Autoimmune hepatitis: target is (X) and LFT pattern is (hepatic/cholestatic).

A

X = hepatocyte

Hepatic

43
Q

Autoimmune hepatitis: which autoantibodies are you likely to find and with which frequency?

A
  1. ANA (70%)
  2. Anti-SM (80%)
  3. LKM Ab (under 10%)
44
Q

Autoimmune hepatitis: which Igs (increased/decreased)?

A

IgG increased

45
Q

Primary biliary cirrhosis (PBC): target is (X) and LFT pattern is (hepatic/cholestatic).

A

X = small interlobular bile duct

Cholestatic

46
Q

Primary sclerosing cholangitis (PSC): target is (X) and LFT pattern is (hepatic/cholestatic).

A

X = medium and large bile duct

Cholestatic

47
Q

Primary biliary cirrhosis (PBC): which autoantibodies are you likely to find and with which frequency?

A

AMA (95%!!)

48
Q

Primary sclerosing cholangitis (PSC): which autoantibodies are you likely to find and with which frequency?

A

pANCA (70%)

49
Q

Primary biliary cirrhosis (PBC): which Igs (increased/decreased)?

A

IgM increased

50
Q

Primary sclerosing cholangitis (PSC): which Igs (increased/decreased)?

A

No specific Ig change

51
Q

Which test used to diagnose autoimmune hepatitis?

A

Liver biopsy (peri-portal inflammation with piecemeal necrosis, plasma cells, fibrosis)

52
Q

Which test used to diagnose Primary biliary cirrhosis (PBC)?

A

Liver biopsy

53
Q

Which test used to diagnose Primary sclerosing cholangitis (PSC)?

A

Liver biopsy and/or cholangiography

54
Q

Treatment for autoimmune hepatitis:

A

Steroids, azathioprine

55
Q

T/F: Most patients with autoimmune hepatitis present acutely.

A

False - 70% with chronic disease, 30% acute

56
Q

T/F: Autoimmune hepatitis has association with HLA.

A

True - DR3/4

57
Q

Three main clinical symptoms of PBC.

A
  1. Pruritis
  2. Fatigue
  3. RUQ discomfort
58
Q

PBC is common (in up to 80%) of which other autoimmune disease?

A

Sicca (primary Sjogren’s)

59
Q

Periportal inflammation with granulomas in bile duct is seen in which autoimmune disease?

A

PBC

60
Q

PBC treatment is (X) which has which effects?

A

X = ursodiol

  1. Improves biliary secretion
  2. Replaces hydrophobic bile salts
  3. Decreases biliary HLA expression
61
Q

T/F: Liver transplant for PBC patients has high rate of survival/success.

A

True - 5y survival in over 80%

62
Q

T/F: Liver transplant for PBC patients prevents recurrence of disease.

A

False - 40% have recurrence at 10y

63
Q

What’s the best prognostic test in advanced PBC?

A

BR levels (8 mg/dL associated with 18 mo survival)

64
Q

(AIH/PBC/PSC) associated with increased risk of cholangiocarcinoma.

A

PSC

65
Q

Most, 70%, of patients with (AIH/PBC/PSC) have concurrent IBD, more commonly (UC/Crohn’s).

A

PSC; UC

66
Q

T/F: There’s no effective therapy for PSC currently.

A

True - but liver transplant successful with low chance recurrence