Liver Injury

Question 1

What is alcoholic liver disease?

Answer 1

Build-up of fat in the liver leading to inflammation and eventually cirrhosis

Question 2

How would you diagnose alcoholic liver disease? (3)

Answer 2

Raised AST: ALT > 2:1
Raised GGT
Biopsy: Mallory body + neutrophil infiltrate

Question 3

How would you treat alcoholic liver disease? (3)

Answer 3

Quit alcohol
Treat malnutrition from alcohol: thiamine (vit B1)
Corticosteroids (suppress immune system)

Question 4

What is non-alcoholic fatty liver disease (NAFLD)?

Answer 4

Fat deposits in the liver (not due to alcohol/ viral causes) leading to hepatocyte destruction.

Question 5

How would you diagnose NAFLD? (2)

Answer 5

Raised ALT

Biopsy: Mallory-denk bodies

Question 6

How would you treat NAFLD?

Answer 6

Lifestyle - lose weight, healthy diet, manage diabetes

Question 7

An autoimmune hepatitis patient would typically present as? (3)

Answer 7

Female
Other autoimmune condition
Liver problems

Question 8

How would you diagnose type 1 (most common) autoimmune hepatitis? (2)

Answer 8

Antinuclear antibodies (ANAs)
Anti-smooth muscle antibodies (ASMA)

Question 9

How would you diagnose type 2 autoimmune hepatitis? (2)

Answer 9

ALKM-1

ALC-1

Question 10

How would you treat autoimmune hepatitis? (2)

Answer 10

Immunosuppressants: prednisolone, azathioprine

Liver transplant

Question 11

What is alpha-1-antitrypsin (A1AT) deficiency?

Answer 11

Autosomal recessive disorder where alpha-1-antitrypsin doesn’t inhibit elastase so too much elastin is broken down

Question 12

What does alpha-1-antitrypsin deficiency lead to? (2)

Answer 12

Emphysema

Cirrhosis

Question 13

What is the clinical presentation of a pt w/ alpha-1-antitrypsin deficiency? (4)

Answer 13

Young person
Cirrhosis
Dyspnoea (difficulty breathing)
No history of smoking

Question 14

How would you diagnose alpha-1-antitrypsin deficiency? (3)

Answer 14

Chest X-ray/ CT: hyperinflated lung/ damaged tissue
Bloods: A1AT levels
Liver biopsy: PAS stain +ve

Question 15

How would you treat alpha-1-antitrypsin deficiency? (4)

Answer 15

IV infusion of normal A1AT protein
Inhalers
Supplemental oxygen
Standard treatments for cirrhosis (lactulose)

Question 16

What id primary biliary cholangitis (PBC)?

Answer 16

Autoimmune destruction intrahepatic biliary ducts leading to cholestasis

Question 17

PBC is common in which pt? (2)

Answer 17

Females

Other autoimmune condition

Question 18

What are the clinical presentations of PBC? (6)

Answer 18

Pruritus (itch)
Jaundice
Xanthelasma (cholesterol deposits under skin)
Hyperpigmentation
Xerophthalmia (dry eyes = vit A def)
Corneal arcus (cholesterol deposits around cornea)

Question 19

How would you diagnose PBC? (2)

Answer 19

Antimitochondrial antibodies (AMA)
Raised serum cholesterol

Question 20

How would you treat PBC? (4)

Answer 20

Ursodeoxycholic acid (UDCA)
Colestryamine for pruritus
KADE supplementation
Bisphosphonates for osteoporosis

Question 21

What is primary sclerosing cholangitis (PSC)? (2)

Answer 21

Inflammation, fibrosis and strictures of intra and extrahepatic parts of the biliary tree

Aka onion-skin fibrosis

Question 22

Why is PSC important?

Answer 22

Pre-malignant disease, can cause colorectal cancer

Question 23

What are the clinical presentations of PSC? (5)

Answer 23

Pruritus (itch)
Jaundice
Dark urine
Clay coloured stool
Hepatosplenomegaly

Question 24

How would you diagnose PSC? (2)

Answer 24

Raised p-ANCAs (antinuclear cytoplasmic antibodies)

MRCP: biliary tracts are beaded or have strictures (‘pruned-tree’)

Question 25

How would you treat PSC? (3)

Answer 25

Colestryamine for pruritus Ursodeoxycholic acid may improve LFT Liver transplant

Question 26

What is hemochromatosis?

Answer 26

Excess iron everywhere

Question 27

What causes hemochromatosis? (2)

Answer 27

Autosomal recessive HFE gene disorder (causing excess iron absorption in SI) Frequent blood transfusions

Question 28

What are the clinical presentations of hemochromatosis? (3 + 1)

Answer 28

- Cirrhosis - Diabetes mellitus - Skin hyperpigmentation (always tanned look) Bronze diabetes Arthralgia from pseudogout

Question 29

How would you diagnose hemochromatosis? (2)

Answer 29

- Blood: increased serum iron, ferritin, decreased transferrin - Decreased total iron-binding capacity

Question 30

How would you treat hemochromatosis? (3)

Answer 30

- Bloodletting - Deferoxamine (iron chelator) - Reduce iron in diet

Question 31

What is Wilson disease?

Answer 31

Excess copper build-up in the liver and CNS

Question 32

What causes Wilson disease?

Answer 32

Autosomal recessive disorder (ATPB7 in chromosome 13)

Question 33

What are the clinical presentations of Wilson disease? (4)

Answer 33

- Cirrhosis - Neurological Sx - Psychiatric Sx - Kayser-Fleischer ring (visible copper deposits in cornea)

Question 34

How would you diagnose Wilson disease? (3)

Answer 34

- Bloods: raised serum copper; low serum ceruloplasmin - Urine: copper - Hepatosplenomegaly

Question 35

How would you treat Wilson disease? (2)

Answer 35

- Penicillamine (copper chelator) | - Reduce copper diet (shellfish)