24. Biliary/Liver 2

Question 1

Signs of pancreatic cancer?

Answer 1

Painless jaundice and a palpable gallbladder - 75% in head of pancreas from exocrine tissue

Rest are MEN1 arising from endocrine tissue

FLAWS
Exocrine - steatorrhoea
Endocrine - diabetes
Hepatomegaly
Trossaeu sign (migratory thrombophelbitis)

Question 2

Investigations for pancreatic cancer?

Answer 2

Bloods - CA19-9
USS
High res CT if number 1 differential - see double duct sign
Gold standard is biopsy by ERCP or US

Question 3

Management of pancreatic cancer?

Answer 3

ERCP for stenting

Whipple’s resection with adjuvant chemo – side effect would be dumping syndrome (rapid gastric emptying) or PUD

Question 4

What is a cholangiocarcinoma?

Answer 4

Cancer arising from bile ducts either intrahepatic or extrahepatic.

Question 5

Signs and symptoms of a cholangiocarcinoma?

Answer 5

Virchow's node
Sister mary joseph nodule
FLAWS
RUQ pain 
Bile obstruction - jaundice, palpable GB, pruritus, pale stool and dark urine

Question 6

Investigations for cholangiocarcinoma?

Answer 6

Bloods - LFTs (obstructive), clotting, CA19-9 and CEA
Imaging - USS and MRCP
Radiography - PTC
ERCP with biopsy is gold standard

Question 7

Management for cholangiocarcinoma?

Answer 7

Depends on where cancer is 
- Removal of bile duct 
- Partial hepatectomy 
- Whipple's proceduce (distal BD tumours)
Adjuvant chemo or radiotherapy

Question 8

What is a hepatocellular carcinoma?

Answer 8

Primary malignancy of hepatocytes

Question 9

Signs and symptoms of HCC?

Answer 9

Presents late (like rest)
FLAWS
Chronic liver failure symp -
Hepatosplenomegaly
Pruritus
Jaundice
Decompensation -
Acute deterioration of liver failure
Hepatic encephalopathy

Question 10

How to investigate HCC?

Answer 10

Screening for RFs e.g. haemochromatosis, alc hep etc.
Bloods - aFP tumour marker
Imaging - USS

Question 11

How to manage HCC?

Answer 11

Early
- Surgical resection (non-cirrhotic)
- Transplant (cirrhotic)
Unsuitable 
- Percutaneous ethanol injections
- Multikinase inhibitor with sorafenib

Question 12

What is primary biliary cirrhosis?

Answer 12

Autoimmune condition associated with granuloma formation in the bile ducts. Inflammatory disorder causing destruction of interlobular bile ducts.

More common in women.

Question 13

Signs and symptoms of PBC?

Answer 13

Pruiritus and jaundice (obstruction)
RUQ pain
Xanthelasma and xanthomata
Clubbing
Hepatosplenomegaly

Question 14

Investigations for PBC?

Answer 14

Bloods - AMA M2 antibodies (raised serum IgM too)

Question 15

How to treat PBC?

Answer 15

Treat symptoms

Vitamin A, D, E and K supplements
Ursodeoxycholic acid
Cholestyramine for pruritis
Transplant

Question 16

What is primary sclerosing cholangitis?

Answer 16

Biliary inflammation of unknown aetilogy, inflammation of intra or extrahepatic ducts

Question 17

Signs and symptoms of PSC?

Answer 17

Pruritus, jaundice, fatigue, RUQ pain

Question 18

Investigations for PSC?

Answer 18

Bloods - pANCA positive
ERCP/MRCP - bead on a string appearance
Biopsy - onion skin fibrosis

Question 19

How to manage PSC?

Answer 19

Screen for cholangiorcinoma
Follow up LFTs and tumour markers every 6 months
Proceed to ERCP and biopsies of any suspicious lesions

Question 20

What is wilson’s disorder?

Answer 20

An autosomal recessive disorder where there is excessive copper deposition in tissues.
Increased copper absorption from SI and decreased hepatic excretion.

Question 21

Signs and symptoms of wilson’s?

Answer 21

Neuro - parkinsonism, behavioural changes, psychiatric or speech distubances
Eyes - kayser-fleischer rings
Liver - hepatitis and cirrhosis

Question 22

Investigations wilson’s

Answer 22

Slit lamp eye examination
Bloods LFTs, reduced serum caeruloplasmin and raised free copper
Urinalysis - increased 24hr copper excretion
Genetic testing/liver biopsy

Question 23

How to manage wilson’s?

Answer 23

Copper chelation with penicillamine

Other chelation - trientine hydrochloride, tertrathiomolybdate

Question 24

What is haemochromatosis?

Answer 24

Abnormal iron deposition in certain organs causing oxidative damage
Primary - due to mutation on HFE gene, autosomal recessive
Secondary to frequent blood transfusions, iron supplementation or diseases of erythropoiesis

Question 25

Signs and symptoms of haemochromatosis?

Answer 25

Liver, pancreas, skin, pituitary, heart and joint affected.

Classic triad - cirrhosis, diabetes, bronze pigmentation

Lethargy
Impotence
Arthralgia
Deranged LFTs

Question 26

Investigations for haemochromatosis?

Answer 26

Serum ferritin raised
Transferring >45%
LFTs raised AST and ALT
FBC normal
Genetic testing
Liver biopsy 
Investigate through presenting complaint

Question 27

How to manage haemochromatosis?

Answer 27

Guided by - serum transferrin, ferritin, clinical symptoms
Avoid iron and vit c supplements and alcohol intake
Stage 0 - monitor every 3 years
1 - monitor every year
2-4 - venesection/iron chelation therapy
End stage - liver transplant