liver disorders and related conditions

Question 1

what is Cholestasis?

Answer 1

Impairment of bile formation and/or bile flow

Question 2

what are the symptoms of cholestasis? (5)

Answer 2

1) Fatigue
2) Pruritus
3) Dark urine, pale stools
4) Jaundice
5) Signs of fat soluble vitamin deficiencies

Question 3

summarise the treatment options for cholestatic pruritus

Answer 3

1) Colestyramine (Anion-exchange) is the drug of choice for treating cholestatic pruritus
2) Ursodeoxycholic acid
3) Rifampicin [unl] can be used as an alternative treatment for pruritus
4) sertraline [unl] may be used but its use is limited due to significant side-effects.

Question 4

Explain how colestyramine relieves symptoms in choleostatic pruritius?

Answer 4

Relieves pruritus by forming an insoluble complex in the intestine with bile acids- the reduction of serum bile acid levels reduces excess deposition in the dermal tissue with a resultant decrease in pruritus.

Question 5

who should Rifampicin be used in caution with?

Answer 5

patients with pre-existing liver disease because of possible hepatotoxicity

Question 6

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

Answer 6

1) liver disorder that occurs in pregnant women

2) Usually occurs in late pregnancy and is associated with adverse fetal outcomes.

Question 7

which drug is effective in the management of Intrahepatic cholestasis in pregnancy?

Answer 7

1) Ursodeoxycholic acid
2) No evidence that ursodeoxycholic acid used in late pregnancy affects birth weight in the infant or the risk of preterm delivery.

Question 8

1) What is gallstone disease?

2) How do gallstones (cholelithiases) form?

Answer 8

1) The presence stones in the gallbladder or bile duct, and the symptoms and complications they may cause
2) Occur when hard mineral or fatty deposits form in the gallbladder

Question 9

outline the non-drug treatments for gallstones

Answer 9

1) Asymptomatic gallbladder stones do not need to be treated unless symptoms develop.
2) The definitive treatment- surgical removal by laparoscopic cholecystectomy.

Question 10

Summarise the drug treatments that can be used to manage gallstones

Answer 10

1) Paracetamol or NSAID for mild-to-moderate pain.
2) IM diclofenac sodium can be given for severe pain or, if not suitable, an intramuscular opioid (such as morphine or pethidine)
3) Ursodeoxycholic acid used for the management of gallstone disease (no evidence)

Question 11

what are Inborn errors of primary bile acid ?

Answer 11

When 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 12

what are the 3 treatments for Inborn errors of primary bile acid ?

Answer 12

1) Cholic acid- for treatment when there is a deficiency of two specific liver enzymes. It acts by replacing some of the missing bile acids, thus relieving symptoms
2) Chenodeoxycholic acid -Deficiency of one specific enzyme in the bile acid synthesis pathway
3) Ursodeoxycholic acid [unl] - little evidence

Question 13

what is Primary biliary cholangitis (primary biliary cirrhosis) and what is the treatment?

Answer 13

1) Chronic cholestatic disease which develops due to progressive destruction of small and intermediate bile ducts within the liver, subsequently evolving to fibrosis and cirrhosis.
2) Ursodeoxycholic acid - slows down disease progression