SM_153: Pathology of the Gallbladder and Biliary Tract

Question 1

Describe intrahepatic flow of bile

Answer 1

Intrahepatic flow of bile

1. Bile canaliculi
2. Bile ductules
3. Septal ducts
4. Left and right hepatic ducts

Question 2

Descibe extrahepatic flow of bile

Answer 2

Extrahepatic flow of bile

1. Left and right hepatic ducts
2. Common hepatic duct and cystic duct
3. Common bile duct

Question 3

Bile is synthesized in the ____, stored in the ____, and secreted into the ____

Answer 3

Bile is synthesized in the liver, stored in the gallbladder, and secreted into the duodenum

• Lipid digestion and absorption
• Absorption / recycling of lipid soluble materials
• Process lipid-soluble waste for excretion: bilirubin, cholesterol, xenobiotics, heavy metals
• Most water, also inorganic electrolyes and organic solutes

Question 4

Describe pathway of bilirubin

Answer 4

Bilirubin

1. Breakdown of heme into bilirubin
2. Extrahepatic bilirubin bound to albumin
3. Hepatocellular uptake
4. Glucuronidation (water soluble) -> excreted into bile
5. Gut bacteria deconjugate the bilirubin -> colorless urobilinogens -> feces (some reabsorption and excretion into urine)

Question 5

Cholestasis is ____

Answer 5

Cholestasis is when bile cannot flow from liver to duodenum

• Intrahepatic
• Extrahepatic

Question 6

Cholestasis results in ____, ____, ____, and ____

Answer 6

Cholestasis results in

• Retention of pruritogens (bile acids) -> pruritis
• High serum bilirubin and serum lipids -> jaundice xanthomas
• Reduced / no bile acid entry into the intestine -> malabsorption of fat and fat soluble vitamins (ADEK)
• Hepatic accumulation of copper, bile acids, other compounds -> liver damage

Question 7

These are ____

Answer 7

These are bile plugs

Question 8

Two autoimmune disorders of bile ducts are ____ and ____

Answer 8

Two autoimmune disorders of bile ducts are primary biliary cholangitis (primary biliary cirrhosis) and primary sclerosing cholangitis

• Primary biliary cholagnitis is intrahepatic
• Primary sclerosing cholangitis is extraheptic

Question 9

Primary biliary cholangitis is ____

Answer 9

Primary biliary cholangitis is autoimmune destruction of small intraheptic bile ducts

• Inflammation and fibrosis
• Decreases bile flow (obstructive)
• Anti-mitochondrial autoantibody in 90-95%
• Symptoms: pruritis, jaundice, and xanthomas

Question 10

Anti-mitochondrial autoantibody indicates ____

Answer 10

Anti-mitochondrial autoantibody indicates primary biliary cholangitis

• Elevated alkaline phosphatase, gamma glutamyltranspeptidase, bilirubin, immunoglobulins (IgM)

Question 11

Pruritis, jaundice, and xanthomas is ___

Answer 11

Pruritis, jaundice, and xanthomas is primary biliary cholangitis

Question 12

Describe stages of primary biliary cholangitis on histology

Answer 12

Primary biliary cholangitis stages on histology

1. Florid duct lesions: lymphoplasmacytic inflammation with or without granulomas
2. Ductular proliferations
3. Fibrosis / scarring
4. Cirrhosis

Question 13

This is ___

Answer 13

This is primary biliary cholangitis

(bile plugs)

Question 14

Primary sclerosing cholangitis involves ____ that leads to ____

Answer 14

Primary sclerosing cholangitis involves chronic cholestatic liver disease that leads to progressive inflammation, fibrosis, and destruction of the intra- and extrahepatic bile ducts

• Massively increased risk of cholangiocarcinoma
• Most have coexisting ulcerative cholitis

Question 15

____ is chronic progressive inflammatory destruction of intrahepatic and extrahepatic biliary duct

Answer 15

Primary sclerosing cholangitis is a chronic progressive inflammatory destruction of intrahepatic and extrahepatic biliary duct

• Typical scenario: young men with diarrhea / bloody stools / jaundice, fatigue / itching, Alk-P > 1000, elevated bilirubin, and AST/ALT near normal

Question 16

Primary sclerosis cholangitis histology involves ____ and ____

Answer 16

Primary sclerosis cholangitis histology involves sclerosis causing ductule destruction and concentric onion-skin like fibrosis

Question 17

This is ____

Answer 17

This is primary sclerosing cholangitis

Question 18

Middle age woman with jaundice and itching likely has ____

Answer 18

Middle age woman with jaundice and itching likely has primary biliary cholangitis

Question 19

Young man with jaundice and diarrhea and a history of ulcerative cholitis likely has ____

Answer 19

Young man with jaundice and diarrhea and a history of ulcerative cholitis likely has primary sclerosing cholangitis

Question 20

Hepatic duct reduction in diameter can result from ____, ____, ____, ____, and ____

Answer 20

Hepatic duct reduction in diameter can result from congenital biliary atresia, tumor, stricture, stone, and pancreatic disease

Question 21

Extrahepatic bile ducts include ____, ____, ____, and ____

Answer 21

Extrahepatic bile ducts include right and left hepatic ducts, hepatic dut, cystic duct, and common bile duct

Question 22

Most gallstones are ____ so ____ or ____ is required

Answer 22

Most gallstones are NOT radiopaque so contrast material or ultrasound is required

Question 23

Cholesterol stones are ____

Answer 23

Cholesterol stones are predominantly cholesterol crystals

Question 24

Pigmented stones are ____

Answer 24

Pigmented stones are predominantly calcium salts of unconjugated bilirubin

Question 25

Describe pathogenesis of cholesterol stones

Answer 25

Cholesterol stones pathogenesis * Free cholesterol is insoluble in water * Free cholesterol is converted to bile acids and eliminated through bile * Cholesterol converted to bile acids via 7-a-hydroxylase

Question 26

Bile acids are \_\_\_\_

Answer 26

Bile acids are detergents * Water-soluble sterols derived from hepatic catabolism of cholesterol * Display both hydrophobic and hydrophilic groups (amphophilic) * Solubilize lipids in water via micelles formation

Question 27

Describe pathogenesis of cholesterol stones

Answer 27

Cholesterol stones pathogenesis 1. Bile supersaturated with cholesterol -\> formation of solid cholesterol monohydrate crystals (nucleation) 2. Hypomotility of the gallbladder -\> accelerated nucleation 3. Hypersecretion of mucus in the gallbladder traps the nucleated crystals -\> aggregation

Question 28

Risk factors for cholesterol stones are \_\_\_, \_\_\_, \_\_\_, \_\_\_, and \_\_\_

Answer 28

Risk factors for cholesterol stones are fat, female, fair skin, fertile, and 40+ in age * Fertile: premenopausal -\> increased estrogen -\> increased cholesterol and decreased gallbladder contraction

Question 29

Black pigment stones are \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 29

Black pigment stones are unconjugated bilirubin + calcium, no cholesterol, hard consistency, mostly radiopaque * Chronic hemolysis -\> increase in unconjugated bilirubin -\> precipitation of Ca with bilirubin (black pigmented stone)

Question 30

Someone with cholangiohepatitis may also have ____ stones

Answer 30

Someone with cholangiohepatitis may also have brown pigmented stones * Calcium salts of unconjugated bilirubin + cholesterol fraction + calcium salts of palmitate * Radiolucent * Form in bile duct * Soft, friable consistency

Question 31

These are \_\_\_

Answer 31

These are cholesterol gallstones

Question 32

Gallstones in gallbladder can cause \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 32

Gallstones in gallbladder can cause cholecystitis, choldeocolithiasis, and gallstone ileus

Question 33

Acute cholecystitis is \_\_\_\_

Answer 33

Acute cholecystitis is acute inflammation of the gallbladder * Calculous cholecystisis (90%): gallstone obstructing the cystic duct * Acalculous cholecystitis (10%): assocated with critical conditions

Question 34

Fever, leukocytosis, and right upper quadrant pain indicates \_\_\_\_

Answer 34

Fever, leukocytosis, and right upper quadrant pain indicates acute cholecystitis

Question 35

Chronic cholecystitis is \_\_\_\_

Answer 35

Chronic cholecystitis is repeated mild to severe bouts of acute cholecystitis * Associated with gallstones in 90% * Many without apparent antecedent attacks

Question 36

Recurrent attacks some with steady or colicky RUQ pain with nausea / vomiting or intolerance to fatty food indicates \_\_\_\_

Answer 36

Recurrent attacks some with steady or colicky RUQ pain with nausea / vomiting or intolerance to fatty food indicates chronic cholecystitis

Question 37

This is \_\_\_\_

Answer 37

This is chronic cholecystitis

Question 38

Gallbladder carcinoma involves the risk factor of \_\_\_

Answer 38

Gallbladder carcinoma involves the risk factor of gallstone * Porcelain gallbladder is a calcified gallbladder * 6th and 7th decades * Female:male is 4:1 * Common in Caucasians