2 Biliary Disease Flashcards

1
Q

What is Hartmann’s pouch?

A

Infundibulum of gallbladder; sometimes described as abnormal pouching at the gallbladder neck

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2
Q

Bile is secreted by the ______ and concentrated in the _______

A

Liver

Gallbladder

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3
Q

What is bile made of?

A
Water
Bile salts
Lecithin/traces of phospholipids
Bilirubin
Cholesterol
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4
Q

What is the function of bile?

A

Digestion and absorption of fats

Vehicle for excretion of bilirubin, excess cholesterol, and metabolic byproducts

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5
Q

Inflammation of the gallbladder

A

Cholecystitis

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6
Q

Stones in the gallbladder

A

Cholelithiasis

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7
Q

Inflammation of the bile ducts

A

Cholangitis

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8
Q

Stones in the common bile duct

A

Choledocholithiasis

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9
Q

Disruption of bile flow

A

Cholestasis

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10
Q

In the US, gallstones are seen in approx ___ of men and ____ of women

A

6%

9%

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11
Q

Risk factors for cholelithiasis

A
The Four F’s (female, forty, fluffy, fertile):
• Females
• Age ≥40
• Obesity
• Pregnancy
• Estrogen (OCPs, HRT)
Other RFs:
• Rapid weight loss (esp low cal diets or post bariatric surgery)
• Family Hx or genetics
• Ethnicity (Native Americans
• Diabetes
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12
Q

80% of cholelithiasis involves stones made of …

A

Cholesterol

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13
Q

20% of cholelithiasis in the US involves ______ stones

A

Pigment stones

Either black or brown pigment stones

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14
Q

Clinical manifestation of cholelithiasis for the majority of patients

A

Asymptomatic, incidental gallstones

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15
Q

What differentiates uncomplicated gallstone disease from cholelithiasis?

A

SYMPTOMATIC biliary colic in the absence of gallstone-related complications

(Compared to ASYMPTOMATIC cholelithiasis - the stones are there but don’t hurt)

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16
Q

What is considered complicated gallstone disease?

A

Gallstone related complications:
• Acute cholecystitis
• Choledocholithiasis with or w/o acute cholangitis
• Gallstone pancreatitis

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17
Q

What is the initial diagnostic test of choice for cholelithiasis?

A

Ultrasound

95% sensitivity for detection of gallstones
Inexpensive and non-invasive

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18
Q

Abdominal plain films are only positive in ______ of patients with cholesterol stones

A

~10-15%

B/c it takes a lot of cholesterol content to make them radiopaque

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19
Q

Why aren’t CT’s preferred imaging for cholelithiasis?

A

Less sensitive than US (only 55-80%)

Increased expense

Radiation exposure

Used more if trying to eval for other conditions

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20
Q

What do you do for someone with incidental gallstones?

A

Majority of patients do not require treatment

Manage expectantly - reassurance, pt ed for warning signs

Refer for cholecystectomy if Sx develop, unless increased risk of gallbladder cancer or hemolytic disorders (ie sickle cell)

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21
Q

Biliary colic in the absence of gallstone-related complications

A

Uncomplicated gallstone disease

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22
Q

How does biliary colic occur?

A

NO inflammation of the gallbladder

Gallbladder contracts, forcing stone against the outlet or cystic duct opening

—> increased intra-gallbladder pressure

—> Increase in pressure results in pain

—> as GB relaxes, obstruction is relieved and pain slowly subsides

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23
Q

What will your patient report to you that will make you suspect biliary colic?

A

Constant, intense RUQ pain radiating to R shoulder blade

Pain lasts at least 30 min, plateaus within 1 hour, usually lasts <5-6 hours

Postprandial, esp if fatty/greasy meal

Associated N/V and diaphoresis

Not exacerbated by movement

Not relieved by squatting, BM, or flatus

Nocturnal pain common

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24
Q

Physical exam findings for biliary colic

A

Normal vitals (no fever, tachycardia)

Not ill appearing, no evidence of jaundice, anicteric

Abdominal exam:
• Often benign, possible RUQ TTP
• No peritoneal signs
• (-) Murphy’s sign (b/c no inflammation)

All labs normal

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25
Q

What will US show for biliary colic?

A

Gallstones and/or gallbladder sludge

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26
Q

DDx for biliary colic

A

PUD

Acute cholecystitis

Choledocholithiasis

Functional gallbladder disorder

Sphincter of Oddi dysfunction

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27
Q

How to manage uncomplicated gallstone disease

A

Patient with biliary colic but normal PE, normal labs who has US (+) for gallstones or sludge —> cholecystectomy recommended

Why? Prevent recurrent sx and complications

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28
Q

Biliary-type pain in the absence of gallstones, sludge, microlithiasis, or microcrystal disease

A

Functional Gallbladder Disorder

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29
Q

Functional Gallbladder Disorder occurs as a result of…

A

Gallbladder dysmotility - something is impairing GB’s ability to eject bile

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30
Q

What do labs/imaging look like in functional GB disorder?

A

Normal labs (CBC, LFTs, amylase/lipase)

Normal imaging (no stones on US, normal EGD)

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31
Q

How is functional gallbladder disorder diagnosed?

A

Diagnosis of exclusion in a patient with typical biliary-type pain

1st exclude other causes for patient’s pain (PUD, dyspepsia, ischemic heart disease)

If no other causes identified —> Cholecystokinin (CCK)-stimulated cholescintigraphy (AKA: HIDA scan)

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32
Q

HIDA scan is the test of choice for…

A

Functional gallbladder disorder, after you’ve ruled out other causes of biliary type pain

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33
Q

How does a HIDA scan w/ CCK work?

A

CCK given to stimulate GB to contract

GB ejection fraction calculated

A GBEF <35-40% is considered low and supportive of diagnosis

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34
Q

What is the Rome IV criteria for functional gallbladder disorder?

A

Required:
• Biliary Pain
• Absence of gallstones or other structural pathology

Supports Dx but not required:
• Low EF on scintigraphy (HIDA scan)
• Normal liver enzymes, conjugated bilirubin, and amylase/lipase

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35
Q

How does the Rome IV criteria define biliary pain?

A

Pain in the epigastrium and/or RUQ
Builds up to a steady level and lasts at least 30 min
Occurs at variable intervals (not daily)
Is severe enough to interrupt ADL or lead to ER visit
Not significantly relieved by BM, postural changes, or acid suppression

Other criteria supportive of biliary pain but not required:
Pain with N/V
Pain that radiates to the back or right subscapular region
Pain that awakens patient from sleep

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36
Q

How do you manage functional gallbladder disorder?

A

Education and reassurance

Referral

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37
Q

When is a cholecystectomy recommended for patients with functional gallbladder disorder?

A

Typical biliary-type pain and a GBEF <40%

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38
Q

Acute inflammation of the gallbladder together with cystic duct obstruction

A

Acute (calculous) cholecystitis

Predominantly a complication of gallstone disease

~5-10% are acalculous

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39
Q

How does acute cholecystitis present?

A

Usually begins as an attack of biliary pain that progressively worsens (most have had previous episodes of biliary pain)

Prolonged (>4-6 hours), steady, severe RUQ/Epigastric pain

May radiate to right shoulder or back

Fever, N/V, anorexia

Often a Hx of fatty food ingestion

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40
Q

PE findings for acute cholecystitis

A

Vitals: FEVER, TACHYcardia
Gen: ILL appearing, might be lying still on exam table
Skin: NO jaundice
Eyes: anicteric
Abd: RUQ TTP, +/- guarding, (+) MURPHY’S SIGN

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41
Q

What will labs show in acute cholecystitis

A

LEUKOCYTOSIS W/ LEFT SHIFT

May see mild elevation in serum AST/ALT

Elevated serum total bilirubin and alk phos are NOT common
• If present, should raise concern for biliary obstruction (cholangitis, choledocholithiasis)

Serum amylas usually normal (if elevated, suggests pancreatitis)

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42
Q

Most common complication of acute cholecystitis

A

Gangrene (up to 20% of cases)

More common in older patients, patients with DM, those that delay seeking treatment

Sepsis-like presentation, can lead to perforation, peritonitis

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43
Q

Complications of acute cholecystitis

A

Gangrene (20%)***

Perforation (10%)*** - usually 2˚ to gangrene

Cholecystoenteric FISTULA

Gallstone ILEUS

Emphysematous cholecystitis, empyema, hydros (from gas-producing infection)

MIRIZZI SYNDROME (1%)

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44
Q

A stone in the cystic duct or Hartmann’s pouch of the gallbladder that compresses the common hepatic duct and causes jaundice

A

Mirizzi Syndrome - uncommon but good to know about

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45
Q

How do you manage Acute Cholecystitis?

A
Admit to hospital
NPO
IV fluids
IV pain control (Ketorolac, Morphine, Meperidine)
IV Abx (empiric)
CHOLECYSTECTOMY*** mainstay of treatment
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46
Q

RUQ Pain
Fever
Leukocytosis
GB Inflammation

What you think?

A

Acute Cholestasis!

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47
Q

Chronic inflammation of the gallbladder, almost always associated with gallstones

A

Chronic cholecystitis

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48
Q

Chronic cholecystitis results from…

A

Repeated acute/subacute cholecystitis or prolonged mechanical irritation of the gallbladder wall by stones

49
Q

How do patients with chronic cholecystitis present?

A

May only have minimal Sx

May be asymptomatic for years, may progress to symptomatic GB disease or acute cholecystitis, or may present with complications

You Just. Don’t. Know.

50
Q

Acute necroinflammatory disease of the gallbladder in the absence of gallstones

A

Acalculous cholecystitis

Associated with high morbidity and mortality rates (much more sick)

51
Q

What is the pathogenesis of acalculous cholecystitis?

A

GB stasis and ischemia

—> Local inflammatory response in the GB wall

—> Secondary bacterial infection is common

—> Perforation in severe cases

More common in hospitalized and critically ill patients (trauma/burn patients, postpartum after prolonged labor, ortho surgery)

52
Q

How do patients with acalculous cholecystitis present?

A

May present similar to acute (calculous) cholecystitis

May have a clinical picture of sepsis-related cholestasis and jaundice

53
Q

What diagnostic approach do you take for acalculous cholecystitis?

A

Clinical suspicion in critically ill patients with sepsis w/o clear source or jaundice

Work up includes imaging (US, CT, HIDA scan), liver lasts, CBC, CMP, pancreatic enzymes, UA

Prompt treatment is imperative

54
Q

How do you treat acalculous cholecystitis?

A

Treat to manage risk of GB gangrene and perforation

Obtain blood cultures and initiate abx (b/c already septic)

Cholecystectomy

If too sick for surgery, possible GB drainage (up to the surgeon)

55
Q

Gallstones in the common bile duct

A

Choledocholithiasis

In patients with cholelithiasis, passage of gallstones in CBD occurs in about 10-15%

56
Q

Incidence of choledocholithiasis increases with …

A

Age

57
Q

In choledocholithiasis, the gallstones can block the flow of _____ and cause _______

A

Bile

Jaundice

58
Q

How does choledocholithiasis present?

A

Biliary-type pain, often more prolonged than seen with typical biliary colic

Reports of JAUNDICE

59
Q

PE findings for choledocholithiasis

A

Vital signs typically WNL

RUQ or epigastric TTP

May appear JAUNDICED

Courvoisier sign** (palpable GB)

60
Q

What is the name for a palpable GB, typically noted on PE in choledocholithiasis?

A

Courvoisier sign

61
Q

Lab findings indicative of choledocholithiasis

A

No leukocytosis on CBC

Elevated liver tests (due to cholestasis)
• ALT and AST elevated early in course
• Cholestatic pattern later - inc bilirubin, alk phos, and gamma-glutamyl transpeptidase (GGT) exceeding elevations in ALT/AST

Normal amylase and lipase (no pancreatic involvement

62
Q

What imaging do you want to order for your poor patient with choledocholithiasis?

A

US - initial imaging of choice
• Cholelithiasis with CBD stone and CBD dilation >6mm

If diagnosis uncertain, consider MRCP or endoscopic US

63
Q

MRCP is great for showing…

A

Biliary tree and pancreatic ducts

Used to confirm diagnosis of common bile duct stone/obstruction

64
Q

How do you manage choledocholithiasis?

A

Removal of the CBD stone

Identify and treat complications
• Acute cholangitis
• Acute pancreatitis

ERCP = therapeutic and diagnostic

Cholecystectomy to follow if appropriate

65
Q

Inflammation of the biliary duct system caused primarily by a bacterial infection in a patient with biliary obstruction

A

Acute cholangitis

66
Q

Biliary obstruction causing cholangitis can be from…

A

Choledocholithiasis***
Benign biliary stricture
S/P ERCP
Malignancy

67
Q

Where do the bacteria come from in acute cholangitis?

A

Typically ascend from the duodenum

68
Q

“Pus under pressure”

A

Acute cholangitis

It’s a surgical emergency - early intervention is imperative

69
Q

How does acute cholangitis present?

A

Charcot’s Triad:
• Fever
• Abdominal Pain
•Jaundice

If Charcot’s Triad + AMS + Hypotension = Reynold’s Pentad (significant morbidity and mortality)

70
Q

Fever + Abdominal Pain + Jaundice

A

Charcot’s Triad

71
Q

Fever + Abdominal Pain + Jaundice + AMS + Hypotension

A

Reynolds Pentad

72
Q

What lab findings are indicative of acute cholangitis?

A

LEUKOCYTOSIS W/ LEFT SHIFT

Elevated CRP (or ESR)

Liver tests show evidence of cholestasis
• Inc bilirubin
• Inc alk phos
• Inc GGT
• May see Inc AST/ALT

Serum amylase or lipase can be 3-4x normal (suggests associated pancreatitis)

(+) blood cultures in 25-40%

73
Q

What imaging do you want for acute cholangitis?

A

Ultrasound

CT

MRCP (or EUS)
• CBD Dilation
•CBD Stone

74
Q

How do you manage acute cholangitis?

A

Admit to hospital

Monitor for and treat sepsis (empiric abx)

Emergent consult with GI and surgery
• Establishing biliary drainage (typically with ERCP)

75
Q

What it the treatment of choice for acute cholangitis?

A

Biliary Drainage

For relief of biliary obstruction imperative (“pus under pressure”)

76
Q

How is biliary drainage performed?

A

ERCP w/ sphincterotomy and stone extraction (and/or stent insertion)

Follow with cholecystectomy if appropriate

77
Q

Biliary Colic or Acute Cholecystitis?

Temporary RUQ pain
No fever
No leukocytosis
No jaundice

A

Biliary Colic

78
Q

Biliary Colic or Acute Cholecystitis?

Persistent RUQ pain
Fever
Leukocytosis
No Jaundice

A

Acute Cholecystitis

79
Q

Choledocholithiasis or Acute Cholangitis?

RUQ/epigastric pain
Jaundice
No fever
No leukocytosis

A

Choledocholithiasis

80
Q

Choledocholithiasis or Acute Cholangitis?

RUQ/epigastric pain
Jaundice
Fever
Leukocytosis

A

Acute cholangitis

81
Q

Autoimmune destruction of the intrahepatic bile ducts which causes cholestasis

A

Primary Biliary Cholangitis (PBC)

May result in cirrhosis and liver failure

82
Q

Clinical presentation of primary biliary cholangitis

A

May be asymptomatic

FATIGUE, intense PRURITIS, +/- Jaundice
RUQ discomfort
Skin hyperpigmentation
Xanthelasmas
Hepatomegaly
83
Q

Primary Biliary Cholangitis is associated with…

A

Other autoimmune conditions

ie Sjogren syndrome and autoimmune thyroid disease

84
Q

Incidence of PBC is higher in…

A

Women

86
Q

Lab findings for Primary Biliary Cholangitis

A

Liver tests = cholestatic pattern
• Elevated Alk Phos, GGT, 5-NT (5’-nucleotidase)
• ALT/AST may be normal or slightly elevated
• Inc serum bilirubin concentration (later in disease)

Antimitochondrial antibodies (AMA) in 95%**

ANA (70% - non specific)

Hyperlipidemia (LDL and HDL may be markedly elevated)

87
Q

Serologic hallmark of PBC

A

Antimitochondrial antibodies (AMA)

Seen in 95% of PBC cases

88
Q

Complications of Primary Biliary Cholangitis

A
Cirrhosis
Hepatocellular carcinoma
Malabsorption (and steatorrhea)
Fat-soluble vitamin deficiences
Metabolic bone disease, incl osteopenia and osteoporosis
89
Q

How do you diagnose Primary Biliary Cholangitis?

A

No extrahepatic biliary obstruction
No comorbidities affecting the liver

At least 2 of the following present:
• Elevated Alk Phos (1.5x ULN)
• (+) AMA
• Histologic evidence of PBC (liver bx if needed)

90
Q

What should you do for patients you suspect have PBC?

A

Liver bx is helpful for prognosing and staging

Refer to GI for:
Medication management
Treatment of complications

91
Q

Sclerosing, inflammatory, and obliterative process involving the intrahepatic and/or extrahepatic biliary tree

A

Primary Sclerosing Cholangitis (PSC)

Chronic/progressive disease of unknown etiology

92
Q

Incidence of PSC is higher in …

A

Men (70%)

Mean age at diagnosis is ~40 years

93
Q

PSC is closely associated with …

A

IBD (UC > CD)

94
Q

Primary Sclerosing Cholangitis ultimately leads to complications of…

A

Cholestasis

End-stage liver disease

95
Q

SSx of Primary Sclerosing Cholangitis

A

Asymptomatic

FATIGUE

PRURITIS

Jaundice

Hepatomegaly

96
Q

Lab tests indicative of PSC

A

Liver tests demonstrate cholestatic pattern

Increased IgM levels, (+) P-ANCA, Autoantibodies (ANA, ASMA)

(-) AMA

97
Q

How do you diagnose PSC?

A

Cholestatic pattern on liver test, esp with underlying IBD

Cholangiography
• MRCP or ERCP (or Percutaneous transhepatic cholangiography)
• Multifocal structuring and dilation of intrahepatic and/or extrahepatic bile ducts

Liver bx
• May support diagnosis but is rarely diagnostic

98
Q

Complications of PSC

A
End-stage liver disease
Fat-soluble vitamin deficiencies
Metabolic bone disease
Cholangitis and cholelithiasis
Hepatobiliary cancer
Colon cancer (in pt with UC)
99
Q

Inherited deficiency in enzyme for glucuronidation of bilirubin in the liver (conjugation)

A

Gilbert Syndrome

100
Q

Unconjugated hyperbilirubinemia in the absence of hemolysis

A

Gilbert Syndrome

Normal CBC, blood smear, and reticulocyte count

Remainder of liver tests normal

101
Q

Clinical presentation of Gilbert Syndrome

A

Typically presents during post-pubertal adolescence

More common in males

Mild INTERMITTENT episodes of jaundice

Otherwise, most are asymptomatic

No specific treatment required

102
Q

What can trigger jaundice in patients with Gilbert Syndrome?

A

Dehydration
Fasting
(Menstruation)

103
Q

Most common cancer arising in the biliary tract

A

Gallbladder Cancer

Uncommon but highly fatal malignancy

104
Q

How is Gallbladder Cancer usually diagnosed?

A

Often found incidentally during workup for cholelithiasis

105
Q

Gender distribution for Gallbladder Cancer

A

W>M

Also, increased incidence with age

106
Q

Risk factors for gallbladder cancer

A
Gallstone Disease***
Porcelain Gallbladder
Gallbladder polyps
PSC
Chronic infection (salmonella, Helicobacter)
Obesity
107
Q

What is porcelain gallbladder?

A

Calcified GB due to chronic inflammation

108
Q

How does GB cancer present?

A

May be asymptomatic

May mimic cholelithiasis or suggestive of malignancy (weight loss)

May see jaundice

109
Q

Cholangiocarcinoma arises from…

A

Epithelial cells of the bile ducts

Can be intrahepatic, perihilar, or distal (extrahepatic) biliary tree

Exclusive of GB and ampulla of vater

110
Q

What is the incidence of cholangiocarcinoma?

A

Rare in the US, with slightly higher incidence in men

111
Q

Cholangiocarcinoma is associated with…

A

PSC and choledochal cysts

112
Q

Presentation of cholangiocarcinoma?

A
Jaundice
Pruritis
Abdominal pain
Anorexia
Weight loss
Palpable gallbladder (Courvoisier sign)

Labs may show cholestasis

Increased CA19-9 (screen those with PSC)

113
Q

Cancer arising distal to the bifurcation of the distal CBD and pancreatic duct

A

Ampullary carcinoma

114
Q

There is an increased incidence of ampullary carcinoma with…

A

Familiar Adenomatous Polyposis (FAP) and Hereditary Non-Polyposis Colon Cancer (HNPCC)

115
Q

How does ampullary carcinoma present?

A
Obstructive Jaundice (most common)
Occult GI bleed with microcytic anemia 
Abdominal pain
116
Q

Name that diagnosis:

Women
Pruritis
(+) AMA titer

A

PBC

117
Q

Name that diagnosis:

Men
IBD
Risk for cholangiocarcinoma

A

PSC

118
Q

Name that diagnosis:

Men
Post-puberty
Elevated unconjugated hyperbilirubinemia

A

Gilbert Syndrome

119
Q

How do you manage PSC?

A

Referral to GI for med management and treatment of complications