Diseases Of The Gallbladder And Bile Ducts

Question 1

Defective transporter in progressive familial intrahepatic cholestasis type 2 and benign recurrent intrahepatic cholestasis type 1

Answer 1

F1C1

Question 2

An amino phospholipid transferase (flippase) essential for maintaining the lipid assymetry of the canalicular membrane

Answer 2

F1C1

Question 3

Hemitransporters ABCG5/G8 transport the __ and ___.

Answer 3

Cholesterol

Phytosterol

Question 4

Defective transporter in progressive familial intrahepatic cholestasis type 3

Answer 4

MDR3 (ABCB4)

Question 5

Defective ABCG5/G8 will lead to

Answer 5

Sitosterolemia

Question 6

Ablation of all other ATP-dependent transporter functions is a result from a defective___ transporter.

Answer 6

F1C1

Question 7

Mutation in Dubin-Jonhnson syndrome

Answer 7

MRP2 (ABCC2)

Question 8

Inherited form of conjugated hyperbilirubinemia

Answer 8

Dubin-Jonhnson syndrome

Question 9

Defective membrane regulator in cystic fibrosis

Answer 9

CFTR, ABCC7

Located on bile duct epithelial cells

Question 10

Impaired cholangiocellular pH regulation during ductular bile formation and chronic cholestatic liver disease is associated with

Answer 10

Cystic fibrosis

Resulting in biliary cirrhosis

Question 11

Normal bile acid pool size

Answer 11

2-4 grams

Question 12

How many times per day the bile acid pool circulates?

Answer 12

5-10 times

Question 13

Fecal loss of the bile acid is in the range of __

Answer 13

0.2-0.4 g/day

Question 14

Bile salts are actively absorbed at the__

Answer 14

Distal ileum

Question 15

Secondary bile acids:

Answer 15

Deoxycholate
Lithocholate
Ursodeoxycholic acid

Question 16

Most frequently absorbed secondary bile acid

Answer 16

Deoxycholic acid

Question 17

Maximum rate of bile acid synthesis

Answer 17

5 grams per day

Question 18

Release by the intestine to suppress synthesis of bile acids from cholesterol by inhibiting the rate-limiting enzyme P450 7a1 (CYP P7A1)

Answer 18

FGF 19

Question 19

Upregulator of ABCG5/G8

Answer 19

LXR - liver X receptor (an oxysterol sensor)

Question 20

Upregulator of bile salt export pump (ABC B11)

Answer 20

FXR- Farnesoid X receptor (a bile acid sensor)

Question 21

Normal capacity of the gallbladder

Answer 21

30 ml

Question 22

Major factor that controls the evacuation of bile from the gallbladder

Answer 22

CCK

Question 23

Action of CCK: (3)

Answer 23

1. Powerful contraction of the gallbladder
2. Decrease resistance of the sphincter of Oddi
3. Enhanced flow of biliary contents into the duodenum

Question 24

The most important mechanism in bile stone formation

Answer 24

Increased biliary secretion of cholesterol

Question 25

Rate-limiting enzyme of hepatic cholesterol synthesis

Answer 25

HMG-CoA reductase

Question 26

Variables that predict survival in PSC:

Answer 26

1. age
2. serum bilirubin
3. histologic stage
4. splenomegaly

Question 27

Dose of UDCA in sclerosing cholangitis

Answer 27

20 mg/kg

Question 28

Beaded appearance on cholangiography

Answer 28

PSC

Question 29

Disease entities associated with PSC:

Answer 29

IBD

Ig G4-associated cholangitis

Question 30

treatment for IgG4 associated cholangitis

Answer 30

Glucocorticoids

Question 31

Signs and symptoms of PSC :

Answer 31

Biliary obstruction
RUQ pain
Jaundice, pruritus
Acute cholangitis

Question 32

biliary pain, obstructive jaundice, melena

Answer 32

Hemobilia

Question 33

Most common cause of obstructive jaundice

Answer 33

Pancreatic head mass

Question 34

Bile leak or abcess formation in the early postoperative period, biliary obstruction or cholangitis as long as 2 years or more following the inciting trauma

Answer 34

strictures

Question 35

Laboratory picture after relief of obstruction :

Answer 35

aminotransferase returns to normal rapidly
serum bilirubin 1-2 weeks to normalize
alkaline phosphatase level usually falls slowly

Question 36

Acute cholecystitis presenting with back pain or pain to the left of the abdominal line, prolonged vomiting with paralytic ileus and pleural effusion on the left side

Answer 36

Pancreatitis

Question 37

Cholecystitis with S. bilirubin 5-15 mg/dL

Answer 37

CBD stones

Question 38

Cholecystitis with serum bilirubin of 20mg/dL

Answer 38

neoplastic obstruction

Question 39

medical therapy for acute cholecystitis :

Answer 39

NPO
Nasogastric suction
Correct electrolytes and volume depletion
Meperidine or NSAID
IV antibiotic therapy