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
Biliary colic is (relieved/provoked) by food intake. How might the patient describe nature/location of pain?
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)
26
T/F: Biliary colic is thought to be the result of hypoxia.
False - tonic spasm around a transiently obstructed cystic duct
27
Patient presenting with biliary colic. What clinical signs would make you entertain diagnosis of acute cholecystitis?
Fever (or signs of inflammation) and pain lasting over 6h
28
What's Choledocholithiasis?
Common bile duct stone
29
T/F: All common bile duct stones came from gallbladder.
False - may be primary (originating in CBD - usually brown pigment stones)
30
What's cholangitis?
Bacterial infection of bile ducts
31
Charcot's triad, for diagnosis of (X):
X = cholangitis 1. Fever 2. Jaundice 3. RUQ
32
Reynold's pentad adds which additional symptoms to Charcot's triad? This is indicative of (X).
Hypotension and altered mental status X = severe, life-threatening cholangitis
33
Common Rx for acute cholangitis includes:
1. Broad-spectrum antibiotics | 2. Biliary decompression (endoscopic preferred over surg)
34
T/F: Gallstones are one of the most common causes of acute pancreatitis.
True - obstruction of pancreatic duct when stone passes through ampulla
35
Diagnostic imaging study of choice for gallstone detection:
Ultrasound
36
List the 3 ultrasonographic criteria for gallstone diagnosis.
1. Echogenic foci 2. Cast acoustic shadow 3. Gravitational dependency
37
Cholescintigraphy or "HIDA scan" is considered a first-line test for diagnosing (X), though ultrasound is usually preferred.
X = acute cholecystitis
38
What would you expect to see on cholescintigraphy (HIDA scan) in case of obstructed cystic duct?
No visualization of gallbladder (cannot take up tracer)
39
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.
Non-invasive (safe); X = ERCP (but without therapeutic capability) Intermediate (good screening tool)
40
(X) imaging method is likely the best choice for patients with high probability of CBD stone. Why?
X = ERCP (Endoscopic Retrograde Cholangiopancreatography) Therapeutic capabilities (sphincterotomy, stone extraction, stenting, etc.)
41
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?
Amylase/lipase; Imaging not required to establish diagnosis
42
Autoimmune hepatitis: target is (X) and LFT pattern is (hepatic/cholestatic).
X = hepatocyte | Hepatic
43
Autoimmune hepatitis: which autoantibodies are you likely to find and with which frequency?
1. ANA (70%) 2. Anti-SM (80%) 3. LKM Ab (under 10%)
44
Autoimmune hepatitis: which Igs (increased/decreased)?
IgG increased
45
Primary biliary cirrhosis (PBC): target is (X) and LFT pattern is (hepatic/cholestatic).
X = small interlobular bile duct Cholestatic
46
Primary sclerosing cholangitis (PSC): target is (X) and LFT pattern is (hepatic/cholestatic).
X = medium and large bile duct Cholestatic
47
Primary biliary cirrhosis (PBC): which autoantibodies are you likely to find and with which frequency?
AMA (95%!!)
48
Primary sclerosing cholangitis (PSC): which autoantibodies are you likely to find and with which frequency?
pANCA (70%)
49
Primary biliary cirrhosis (PBC): which Igs (increased/decreased)?
IgM increased
50
Primary sclerosing cholangitis (PSC): which Igs (increased/decreased)?
No specific Ig change
51
Which test used to diagnose autoimmune hepatitis?
Liver biopsy (peri-portal inflammation with piecemeal necrosis, plasma cells, fibrosis)
52
Which test used to diagnose Primary biliary cirrhosis (PBC)?
Liver biopsy
53
Which test used to diagnose Primary sclerosing cholangitis (PSC)?
Liver biopsy and/or cholangiography
54
Treatment for autoimmune hepatitis:
Steroids, azathioprine
55
T/F: Most patients with autoimmune hepatitis present acutely.
False - 70% with chronic disease, 30% acute
56
T/F: Autoimmune hepatitis has association with HLA.
True - DR3/4
57
Three main clinical symptoms of PBC.
1. Pruritis 2. Fatigue 3. RUQ discomfort
58
PBC is common (in up to 80%) of which other autoimmune disease?
Sicca (primary Sjogren's)
59
Periportal inflammation with granulomas in bile duct is seen in which autoimmune disease?
PBC
60
PBC treatment is (X) which has which effects?
X = ursodiol 1. Improves biliary secretion 2. Replaces hydrophobic bile salts 3. Decreases biliary HLA expression
61
T/F: Liver transplant for PBC patients has high rate of survival/success.
True - 5y survival in over 80%
62
T/F: Liver transplant for PBC patients prevents recurrence of disease.
False - 40% have recurrence at 10y
63
What's the best prognostic test in advanced PBC?
BR levels (8 mg/dL associated with 18 mo survival)
64
(AIH/PBC/PSC) associated with increased risk of cholangiocarcinoma.
PSC
65
Most, 70%, of patients with (AIH/PBC/PSC) have concurrent IBD, more commonly (UC/Crohn's).
PSC; UC
66
T/F: There's no effective therapy for PSC currently.
True - but liver transplant successful with low chance recurrence