3 Hepatobiliary Function

Question 1

What are the main functions of the liver

Answer 1

Bile production
Metabolism of FA, Carbs, proteins
Bilirubin production
Detoxification

Question 2

What is cirrhosis

Answer 2

Chronic liver disease in which normal liver cells are damaged and replaced by scar tissue (fibrosis)

Question 3

What is the most commmon cause of cirrhosis

Answer 3

Excessive alcohol intake

Question 4

Steatohepatitis is also known as

Answer 4

Fatty liver disease

Question 5

What is the most common cause of portal hypertension

Answer 5

Cirrhosis

Question 6

Where are primary bile acids synthesized

Answer 6

Hepatocytes

Question 7

Secondary bile acids are made where

Answer 7

SI

Question 8

Where are bile salts conjugated

Answer 8

Liver

Question 9

What are the two primary bile acids

Answer 9

Cholic acid and chenodeoxycholic acid

Question 10

What are the two secondary bile acids

Answer 10

Deoxycholic acid from cholic acid

Lithocholic acid from chenodeoxycholic acid

Question 11

What is the most prevalent bile acids

Answer 11

Cholic > chenodeoxycholic > deoxycholic > lithocholic

Question 12

What is the function of bile salts

Answer 12

Solubilizes lipids that are naturally insoluble

Question 13

What is the primary hormonal regulator of bile secretion

Answer 13

Gallbladder contraction directly and relaxation of sphincter of oddi

Question 14

What is the effect of the vagus on bile secretion

Answer 14

Increases bile flow, induces contraction of gallbladder

Question 15

What transporters assist with bile salt uptake into hepatocytes

Answer 15

NTCP and OATPs

Question 16

What are the bile salt transporters that move bile into canaliculi

Answer 16

BSEP and MRP2

Question 17

What moves bile salts into enterocytes

Answer 17

ASBT

Question 18

What moves bile salts out of enterocytes

Answer 18

OST α/β

Question 19

What enzyme makes bile salts

Answer 19

Cholesterol 7α hydroxylase

Question 20

Interruption of the enterohepatic circulation does what to bile acid synthesis

Answer 20

Can increase it by 10 fold

Question 21

What makes conjugated bilirubin

Answer 21

UDP glucuronyl transferase (responsible for infant jaundice)

Question 22

What are the hereditary defects in bilirubin metabolism

Answer 22

Gilbert’s syndrome
Dubin-Johnson syndrome
Rotor syndrome
Crigler-Najjar syndrome (Type 1 and 2)

Question 23

What is hemolytic anemia

Answer 23

Type of anemia which causes increased unconjugated bilirubin

Question 24

What are the 2 main causes of neonatal jaundice

Answer 24

Elevated bilirubin production due to breakdown of fetal erythrocytes

Low activity of UDP gluuronyl transferase

Question 25

What is Gilbert’s syndrome

Answer 25

- increased levels of uncojugated bilirubin in the blood - usually recognized during adolescence - episodes are brought on by physiological stress

Question 26

What causes Gilbert’s syndrome

Answer 26

Mutation in gene that codes fro UDP glucuronyltransferase

Question 27

What causes Crigler-Najjar syndrome

Answer 27

Mutations in the gene that codes for UDP glucuronyltransferase

Question 28

CN1 or 2 is more severe

Answer 28

1

Question 29

What is a major symptom of CN1

Answer 29

Kernicterus (form of brain damage cause by the accumulation of unconjugated bilirubin)

Question 30

What are the treatments for CN1

Answer 30

- phototherapy - heme oxygenase inhibitors - oral calcium phosphate and carbonate - liver transplantation - phenobarbitol (no response in CN1)

Question 31

What are the clinical symptoms of kernicterus

Answer 31

- cerebral palsy - sensory nerual hearing loss - gaze abnormalities

Question 32

What distinguishes CN2

Answer 32

Less than 20% UPD glucuronyltransferase function Less likely to develop kernicterus

Question 33

What is found in Dubin-Johnson syndrome

Answer 33

- increased bilirubin w/o liver enzyme elevation - mutations in MRP2 (unable to secrete conjugated bilirubin) - liver has black pigmentation

Question 34

What is Rotor syndrome

Answer 34

Buildup of conjugated and unconjugated bilirubin but the majority is conjugated

Question 35

What mutation is responsible for Rotor syndrome

Answer 35

OATP1B1 and OATP1B3

Question 36

How does phototherapy work

Answer 36

Changes trans-bilirubin to cis-bilirubin

Question 37

What causes gallstones

Answer 37

- too much absorption of water from bile - too much absorption of bile acids from bile - too much cholesterol in bile - inflammation of epithelium

Question 38

Modification of drugs by liver to their active form

Answer 38

First pass effect

Question 39

What are the liver enzymes

Answer 39

ALT and AST and alkaline phosphatase

Question 40

What does elevated aminotransferases suggest

Answer 40

Hepatocyte injury

Question 41

What does elevated alkaline phosphatase suggest

Answer 41

Cholestasis

Question 42

Bilirubin can be used to measure

Answer 42

The liver’s ability to detoxify metabolites and transport organic anions

Question 43

What is albumin used to measure

Answer 43

Severe impairment in liver function

Question 44

What is prothrombin time used to measure

Answer 44

Reflects the degree of hepatic synthetic dysfunction PT increases as the liver is unable to synthesize clotting factors