Biliary disorders

Question 1

what is cholestasis and how does it present

Answer 1

impairment of bile formation and/or bile flow

may clinically present with fatigue, pruritus, dark urine, pale stools and

in its most overt form, jaundice and signs of fat soluble vitamin deficiencies.

Question 2

what is the drug of choice for treating cholestatic pruritis

Answer 2

colestyramine

Question 3

how does colestyramine relieve pruritis

Answer 3

by forming an insoluble complex in the intestine with bile acids and other compounds

the reduction of serum bile acid levels reduces excess deposition in the dermal tissue with a resultant decrease in pruritus.

Question 4

this drug has a small and variable impact on cholestatic pruritis

Answer 4

ursodeoxycholic acid

Question 5

this antibiotic (unlicensed indication) can be used as alt treatment for pruritis

but when should it be used with caution

Answer 5

rifampicin

caution in pt with pre existing liver disease bc of possible hepatotoxicity

Question 6

where previous therapy has proved ineffective or was not tolerated, these 2 drugs (unlicensed) can be used to treat cholestatic pruritis. But their use is limited due to significant SE

hint: ssri, opioid receptor ant

Answer 6

sertraline
naltrexone

Question 7

which drug is effective for treatment of pruritus associated with intrahepatic cholestasis in pregnancy.

Answer 7

ursodeoxycholic acid

There is no evidence that ursodeoxycholic acid used in late pregnancy affects birth weight in the infant or the risk of preterm delivery. There is limited data about the effect of fetal exposure during the first trimester.

Question 8

when does intrahepatic cholestasis usually occur and what is it associated with in pregnancy

Answer 8

late pregnancy and is associated with adverse fetal outcomes

Question 9

cholestryamine - how should it be taken

Answer 9

Separation of admin!!
Take other drugs at least 1 hour before or 4-6 hours after colestyramine

Question 10

true or false - colestryamine is not absorbed from the git

Answer 10

true

Question 11

colestyramine can impair the absorption of what?

Answer 11

Interference with absorption of fat soluble vitamins (supplements of vit A,D, E, K, and folic acid may be required when treatment is prolonged)

Question 12

how do gallstones occur

Answer 12

hard mineral or fatty deposits form in the gallbladder

Question 13

Gallstone disease is a general term that describes….

Answer 13

presence of one or more stones in the gallbladder or in the bile duct, and the symptoms and complications that they may cause.

Question 14

are gallstones symptomatic

what happens if left untreated

Answer 14

The majority of patients with gallstones remain asymptomatic.

When the stones irritate the gallbladder or block part of the biliary system, the patient can experience symptoms such as pain, or infection and inflammation that if left untreated, can lead to severe complications such as biliary colic, acute cholecystitis, cholangitis, pancreatitis, and obstructive jaundice.

Question 15

definitive treatment of symptomatic gallstones (and all bile duct stones)

Answer 15

surgical removal by laparoscopic cholecystectomy.

Question 16

drug treatment for pain - gallstones

Answer 16

Paracetamol or a NSAID is recommended for intermittent mild-to-moderate pain.

Intramuscular diclofenac sodium can be given for severe pain or, if not suitable, an intramuscular opioid (such as morphine or pethidine hydrochloride).

Question 17

what are: inborn errors of primary bile acid synthesis

Answer 17

• Group of diseases in which the liver does not produce enough primary bile acids due to enzyme deficiencies
• These acids are the main components of the bile, and include cholic acid and chenodeoxycholic acid

Question 18

treatment for inborn errors of primary bile acid synthesis - 3 drugs

Answer 18

cholic acid
chenodeoxycholic acid
ursodeoxycholic acid (unlicensed indication)

Question 19

what is colic acid licensed for (inborn errors of primary bile acid synthesis) & how does it work

Answer 19

licensed for the treatment of inborn errors in primary bile acid synthesis due to an inborn deficiency of two specific liver enzymes. It acts by replacing some of the missing bile acids, therefore relieving the symptoms of the disease.

Question 20

what is chenodeoxycholic acid licensed for (Inborn errors of primary bile acid synthesis)

Answer 20

Licensed for the treatment of inborn errors of primary bile acid synthesis due to a deficiency of one specific enzyme in the bile acid synthesis pathway when presenting as cerebrotendinous xanthomatosis.

Question 21

discuss use of ursodeoxycholic acid for treatment of inborn errors of primary bile acid synthesis

Answer 21

Ursodeoxycholic acid [unlicensed indication] has been used to treat inborn errors of primary bile acid synthesis, but there is an absence of evidence to recommend its use.