Liver Disorders

Question 1

What is cholestasis?

Answer 1

Impaired bile formation or flow

Question 2

Symptoms of cholestasis

Answer 2

Fatigue, pruritus, dark urine, pale stools, jaundice, signs of fat-soluble vitamin deficiencies

Question 3

Treatment of cholestatic pruritus

Answer 3

Cholestyramine

Ursodeoxycholic acid

Rifampicin (unlicensed)

Sertraline (unlicensed)

Naltrexone (unlicensed)

Question 4

How does cholestyramine relieve pruritus?

Answer 4

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 5

Cautions of rifampicin

Answer 5

Patients with pre-existing liver disease

Question 6

Treatment of intrahepatic cholestasis in pregnancy

Answer 6

Ursodeoxycholic acid

Question 7

When is it most common to have intrahepatic cholestasis in pregnancy?

Answer 7

During late pregnancy and associated with adverse fetal outcomes

Question 8

What are gallstones?

Answer 8

Hard mineral or fatty deposits forming stones in the gallbladder bile duct

Question 9

Risk factors of gallstones

Answer 9

Crohn’s disease.
Diabetes mellitus.
Diets higher in triglycerides and refined carbohydrates and low in fibre are associated with gallstones.
Females
Non-alcoholic fatty liver disease

Question 10

Symptoms of gallstones

Answer 10

Majority of patients will be asymptomatic

If gallbladder is blocked or irritated = pain, infection, inflammation

Question 11

Complications of gallstones

Answer 11

Biliary colic, cholecystitis, cholangitis, pancreatitis, obstructive jaundice

Question 12

Advice to patients with gallstones

Answer 12

Avoid food and drink that triggers their symptoms until they have their gallbladder or gallstones removed

Do not need to avoid this food and drink after surgery.

Seek further advice if eating or drinking triggers existing symptoms or causes new symptoms to develop after they have recovered from having their gallbladder or gallstones removed.

Question 13

Treatment of gallstones

Answer 13

Surgical removal if symptoms develop

Mild-moderate pain: paracetamol or NSAID
Severe pain: IM diclofenac or morphine/pethidine
Ursodeoxycholic acid has been used but no evidence behind it

Question 14

What is primary biliary cholangitis?

Answer 14

Chronic cholestatic disease which develops due to progressive destruction of small and intermediate bile ducts within the liver, subsequently evolving to fibrosis and cirrhosis

Question 15

Treatment of primary biliary cholangitis?

Answer 15

Ursodeoxycholic acid (even asymptomatic patients)

Liver transplantation can be considered with advanced disease

Question 16

Role of Ursodeoxycholic acid in the treatment of primary biliary cholangitis

Answer 16

Slows disease progression but the effect on overall survival is uncertain

Question 17

What is inborn errors of primary bile acid synthesis?

Answer 17

Group of diseases in which the liver does not produce enough bile acids due to enzyme deficiencies

Question 18

Which acids would you be deficient in with inborn errors of primary bile acid synthesis?

Answer 18

Main components of bile: cholic acid and chenodeoxycholic acid

Question 19

Treatment of inborn errors of primary bile acid synthesis. Role of these drugs?

Answer 19

Cholic acid; where there is deficiency of two specific liver enzymes
Acts by replacing some of the missing bile acids

Chenodeoxycholic acid; where there is deficiency of one specific enzyme in the bile acid synthesis pathway when presenting as cerebrotendinous xanthomatosis

Urosdeoxycholic acid (Unlicensed)

Question 20

Treatment of inborn errors of primary bile acid synthesis due to inborn deficiency of two specific liver enzymes

Answer 20

Cholic acid