Liver, Biliary, Pancreas

Question 1

Concerning chronic viral hepatitis which of the following is most correct?

1. Chronic hepatitis is defined as histological evidence of acute hepatic injury, repair and regeneration at the same time.
2. Hepatitis B transmission is predominately due to blood or sexual contact in developing nations
3. Younger patients have a greater risk of progressing to chronic infection than older patients
4. Hepatitis E causes a benign sporadic acute hepatitis, with mortality rates of less than 1% across all ages.
5. The majority of patients with Hepatitis C clear the virus spontaneously within 2 months with only 1- 2% of those infected progressing to cirrhosis.

Answer 1

*Younger patients have a greater risk of progressing to chronic infection than older patients

Question 2

Concerning advanced cirrhosis, which of the following is LEAST correct?

1. The presence and degree of splenomegaly does not necessarily reflect the presence/extent of portal hypertension.
2. Hepatopulmonary syndrome may be seen in up to 30% of patient with severe cirrhosis and portal hypertension
3. Liver disease with portal hypertension is a potential cause of pulmonary hypertension
4. Only 2 – 5% of patients with advanced cirrhosis have oesophageal varies
5. About 40% of patients with cirrhosis are asymptomatic

Answer 2

4. *Only 2 – 5% of patients with advanced cirrhosis have oesophageal varies

Question 3

A patient with well-compensated cirrhosis suddenly develops acute hepatic failure. Which of the follow is LEAST correct?

1. It may reflect spontaneous mutation/ endogenous reinfection with a chronic viral agent
2. It may reflect ascending cholangitis particularly if the cirrhosis is related to primary sclerosing cholangitis +/- instrumentation
3. It may reflect vascular thrombosis
4. It may reflect sepsis
5. It may reflect splenic infarct

Answer 3

5. *It may reflect splenic infarct

Question 4

Concerning chronic viral hepatitis which of the following is most correct?

1. Chronic hepatitis is defined as histological evidence of acute hepatic injury, repair and regeneration at the same time.
2. Hepatitis B transmission is predominately due to blood or sexual contact in developing nations
3. Younger patients have a greater risk of progressing to chronic infection than older patients
4. Hepatitis E causes a benign sporadic acute hepatitis, with mortality rates of less than 1% across all ages.
5. The majority of patient with Hepatitis C clear the virus spontaneously within 2 months with only 1- 2% of those infected progressing to cirrhosis.

Answer 4

3. *Younger patients have a greater risk of progressing to chronic infection than older patients

Question 5

Concerning advanced cirrhosis, which of the following is LEAST correct?

1. The presence and degree of splenomegaly does not necessarily reflect the presence/extent of portal hypertension.
2. Hepatopulmonary syndrome may be seen in up to 30% of patient with severe cirrhosis and portal hypertension
3. Liver disease with portal hypertension is a potential causes of pulmonary hypertension
4. Only 2 – 5% of patients with advanced cirrhosis have oesophageal varies
5. About 40% of patients with cirrhosis are asymptomatic

Answer 5

4. *Only 2 – 5% of patients with advanced cirrhosis have oesophageal varies

Question 6

Concerning autoimmune hepatitis which of the following is LEAST correct?

1. In 30 – 40% of cases it may have a fulminant presentation with hepatic failure within 2 months of presentation
2. There are 2 subtypes, 1 more common in middle aged females and a second more common in elderly males
3. Immunotherapy can induce remissions in 70-85% of patients
4. 20% of transplanted patient experience recurrent disease in the transplant
5. Without treatment the disease eventually progresses to liver failure

Answer 6

2. *There are 2 subtypes, 1 more common in middle aged females and a second more common in elderly males

Question 7

A 12 year girl has a slowly progressive movement disorder. MRI shows putamen abnormality without a history of methanol or carbon monoxide toxicity. Wilson’s disease is considered. Which of the following most correct?

1. Wilson’s disease is unlikely as it is X-linked recessive
2. Wilson’s disease is unlikely as it does not present at this age
3. Wilson’s disease is unlikely as it a disease of the liver not CNS
4. Wilson’s disease is unlikely as the movement disorder is due to cerebellar involvement
5. The given history is compatible with Wilson’s disease but other tests would be required for diagnosis.

Answer 7

5. *The given history is compatible with Wilson’s disease but other tests would be required for diagnosis.

Question 8

Concerning neonatal jaundice, which of the following is LEAST recognised as a cause of prolonged hyperbilirubinemia

1. Alpha-1-antitryspin deficiency
2. Biliary atresia
3. Bilateral adrenal haemorrhage
4. Renal failure
5. Crigler-Najjar Syndrome

Answer 8

4. *Renal failure

Question 9

Concerning biliary atresia which of the following is LEAST correct?

1. All forms show at birth an absence or lack of complete cannulation of the common bile duct
2. It has association with heterotaxy
3. The most common or perinatal form is an idiopathic destructive/ fibrosing condition affecting the biliary tree in the first 3 months of life
4. Cirrhosis eventually develops in 20- 40% of patients over 3 – 5 years
5. In some patients the disease involves the proximal ducts away from the porta and surgery may not be possible.

Answer 9

1. *All forms show at birth an absence or lack of complete cannulation of the common bile duct

Question 10

Concerning primary sclerosing cholangitis (PSC), which of the following is LEAST correct?

1. 70% of patients have associated Ulcerative colitis
2. Less than 0.01% of patients with inflammatory bowel disease develop PSC
3. It is most common in males 20 – 50 years old
4. There is a lifetime risk of cholangiocarcinoma of 15% -20%
5. Associated strictures can be a cause of chronic pancreatitis

Answer 10

2. *Less than 0.01% of patients with inflammatory bowel disease develop PSC

Question 11

A patient has evidence of portal vein thrombosis with long established collateral formation but no cirrhosis. Which of the following is LEAST likely to relevant?

1. History of prematurity with 6/52 neonatal ICU admission
2. History of Choledocholithiasis with recurrent pancreatitis
3. Current myelofibrosis (from previous polycythaemia)
4. Previous appendiceal abscess
5. History of Rheumatoid Arthritis

Answer 11

5. *History of Rheumatoid Arthritis

Question 12

Sinusoidal Obstruction syndrome is most likely to occur in which of the following settings:

1. 3 weeks post allogenic haematological stem cell transplantation
2. 1st week of life in a prem infant <1000 g
3. 1st week of life of a term infant
4. In the 1st month postpartum
5. Severe right heart failure

Answer 12

1. *3 weeks post allogenic haematological stem cell transplantation

Question 13

Concerning gall bladder inflammation, which of the following is LEAST correct?

1. 1-10% of cases of acute cholecystitis are acalculous with ischemia thought to play a significant role
2. Acalculous cholecystitis is more common in setting of septic shock and burns
3. Rare but recognised causes of acalculous cholecystitis include vasculitis and typhoid infection
4. Xanthogranulomatous cholecystitis is characterised by a markedly thickened wall, in a shrunken nodular gall bladder.
5. Chronic cholecystitis, unlike acute is more common in females and has a lower (70%) association with cholelithiasis.

Answer 13

5. *Chronic cholecystitis, unlike acute is more common in females and has a lower (70%) association with cholelithiasis.

Question 14

Which of the following is NOT an accepted cause of pancreatitis?

1. Metabolic conditions including hyperparathyroidism and hyperlipidaemia
2. Medications including frusemide
3. Pancreatic ischemia
4. Mumps virus
5. SIADH

Answer 14

5. *SIADH

Question 15

A child has pancreatitis. Which of the following is LEAST correct?

1. This could be hereditary pancreatitis which can behave as an autosomal dominant condition
2. This could be hereditary pancreatitis which can behave as an autosomal recessive condition
3. This could be secondary to cystic fibrosis
4. This could be associated with mumps virus infection
5. Hereditary pancreatitis is not associated elevated risk of pancreatic carcinoma

Answer 15

5. *Hereditary pancreatitis is not associated elevated risk of pancreatic carcinoma

Question 16

Concerning chronic pancreatitis, which of the following is LEAST likely to be a cause?

1. Hereditary causes
2. Pancreatitis duct stricture
3. Marfan’s syndrome.
4. IgG4-realted disease
5. Pancreatic duct/ common duct calcui

Answer 16

3. *Marfan’s syndrome.

Question 17

Concerning cystic neoplasms of the pancreas, which of the following is LEAST correct?

1. Only 5 – 15% of pancreatic cysts are neoplastic
2. Serous neoplasms are usually unilocular and more common in the head of the pancreas
3. Serous pancreatic neoplasms are more common in females (at least 2 – 3 x) and most common in the 60 – 80 year age group.
4. Mucinous neoplasms are far more common in females (~ 95%)
5. IPMN are more common in males than females, more common in the pancreatic head (with 10 – 20% are multifocal)

Answer 17

2. *Serous neoplasms are usually unilocular and more common in the head of the pancreas

Question 18

Concerning pancreatic adenocarcinoma, which of the following is LEAST correct?

1. Smoking is a risk factor for pancreatic adenocarcinoma
2. Diabetes is a risk factor of pancreatic adenocarcinoma
3. Diabetes can be a complication of pancreatic adenocarcinoma
4. The risk of pancreatic adenocarcinoma in hereditary pancreatitis is up 40%
5. There is no association with BRACA mutations

Answer 18

5. *There is no association with BRACA mutations

Question 19

A body builder admits to taking steroids. He has yellow eyes and a normal biliary ultrasound. What is the most likely diagnosis? (March 2015)

a. Cholestasis
b. Fatty liver
c. Steatohepatitis
d. Cirrhosis with fibrosis

Answer 19

• Anabolic steroids have been implicated in acute cholestatic liver injury
• Fatty liver, steatohepatitis and cirrhosis with fibrosis should have abnormalities on ultrasound

ANSWER: Cholestasis (or steatohepatitis)

Question 20

A woman has deranged LFTs. She has been on the oral contraceptive pill for 12 months. Her ultrasound shows a normal biliary tree. (September 2013)

a. Steatosis/fatty liver
b. Steatohepatitis
c. Hepatocellular necrosis

Answer 20

ANSWER: Steatohepatitis given the normal biliary tree and deranged liver function tests

Question 21

Which is a cause of fulminant hepatitis? (August 2014)

a. Autoimmune hepatitis
b. Hepatitis B
c. Carbon tetrachloride

Answer 21

Fulminant hepatitis
- onset of encephalopathy w/in 2 weeks of the onset of jaundice

Viral:

• Hepatitis A, B, D, E (Hepatitis B and E the most common causes of fulminant hepatitis in Asia)
• Other: CMV, EBV, HSV
• HCV is not listed as a cause in Robbins

Toxins:
- Drugs:
• Paracetamol (overdose most common cause of fulminant hepatic failure in Western Countries)
- Synergistic effect with alcohol or barbituates
• Isoniazid
• Tetracyclines
• Carbamazepine
• Valproate
- Other toxins:
• Halogenated hydrocarbons

Metabolic:

• Wilson disease
• A-1 AT deficiency
• Galactosaemia
• Tyrosinaemia
• Reye Syndrome
• NASH

Associated w/ pregnancy:

• Acute fatty liver of pregnancy
• HELLP syndrome

Vascular:

• Budd-Chiari syndrome
• Veno-occlusive disease
• Shock
• Heart failure

Miscellaneous:

• Autoimmune hepatitis
• Malignant infiltration
• Hyperthermia
• Sepsis

ANSWER: All of the options are implicated in the development of fulminant hepatic failure. Hepatitis B is one of the most common causes in Asian nations.

Question 22

Which is not an indication for a TIPSS procedure? (March 2016)

a. Hepatorenal syndrome
b. Hepatopulmonary syndrome
c. Intractable ascites
d. Bleeding gastric varices
e. Fulminant liver failure

Answer 22

ANSWER: Fulminant liver failure is a contra-indication to TIPSS placement

Question 23

Regarding liver transplant medication, what is the most common complication? (August 2016)

a. Veno-occlusive disease

Answer 23

Hepatic veno-occlusive disease – now known as sinusoidal obstruction syndrome
- Arises from the occlusion of the hepatic venules

Clinical presentation:
RUQ pain
Hepatomegaly
Ascites
Abnormal liver function tests

Pathology:

• Toxic injury to the liver sinusoids causes sloughing of the endothelial cells which embolise to the hepatic venules and cause eventual fibrosis
• Results in hepatic congestion (similar to Budd-Chiari) and post-sinusoidal portal hypertension

Causes:
- BM transplantation
- Chemotherapy
• Systemic neoadjuvant chemotherapy in colorectal cancer (especially oxaliplatin)
- Jamaican bush tea

ANSWER: Veno-occlusive disease does not appear to be related to liver transplant medications

Question 24

Fibrosis is not seen in: (September 2013, March 2017)

a. Budd-Chiari
b. Cirrhosis
c. Haemochromatosis
d. Wilson disease

Answer 24

ANSWER: ? some sources state Budd-Chiari although this gives centrilobular fibrosis and scarring.

Question 25

Which is least likely to cause generalized cirrhosis? (March 2017)

a. Hepatitis C
b. Budd-Chiari syndrome
c. Schistosomiasis

Answer 25

• Budd-Chiari: centrilobular fibrosis and scarring
• Schistosomiasis: pipestem fibrosis without distortion of the intervening parenchyma

ANSWER: Schistosomiasis is least likely to give generalized cirrhosis of these options

Question 26

What is the least likely cause of fulminant hepatitis? (March 2015)

a. Hepatitis A
b. Hepatitis B
c. Hepatitis C
d. HSV
e. CMV

Answer 26

• Fulminant hepatitis is defined as severe impairment of synthetic function and progression to encephalopathy within 8 weeks

ANSWER: HCV is the least likely cause of fulminant hepatitis

Question 27

In a hepatitis D needlestick injury, which of the following is the most important? (September 2013)

a. Hepatitis A
b. Hepatitis B
c. Hepatitis C
d. Hepatitis E
e. Hepatitis F

Answer 27

ANSWER: Hepatitis B, as hepatitis D is only virulent with HBV coinfection

Question 28

Emphysema and cirrhosis in a patient are most suggestive of: (September 2013)

a. Alpha-1 anti-trypsin deficiency

Answer 28

Inherited metabolic disorder of the lungs and liver (autosomal recessive, chromosome 14)

• Deficiency of alpha-1 antitrypsin leads to the unopposed action of neutrophil elastase
• More carriers are asymptomatic but some show clinical symptoms

Pathology:

• 90% of AAT is produced by hepatocytes
• No opposition to neutrophil elastase causing unopposed damage to the lower respiratory tract
• Basal predominance due to gravitational blood flow effects

Associations: asthma, pancreatitis, aneurysms

Complications:
o Cirrhosis with increased risk of HCC
o 70-100% increased risk of lung cancer

ANSWER: Alpha-1 anti-trypsin deficiency causes lower zone predominant emphysema and liver cirrhosis

Question 29

Which of the following is false regarding alpha-1 anti-trypsin deficiency? (September 2013)

a. Autosomal dominant with incomplete penetrance
b. Associated with cirrhosis
c. Associated with HCC
d. Associated with lower lobe emphysema

Answer 29

ANSWER: Alpha-1 anti-trypsin deficiency is an autosomal recessive condition

Question 30

Which affects both the liver and the skin? (August 2014)

a. Alpha-1 antitrypsin
b. Wilson disease

Answer 30

Alpha-1 antitrypsin affects the liver (cirrhosis secondary to accumulation of faulty alpha-1 antitrypsin in the hepatocytes) and can cause inflammation of subcutaneous fat in some patients (panniculitis)
o Primarily a disease of the liver and lungs

Wilson disease affects the liver but not the skin

ANSWER: ?Alpha-1 AT due to liver involvement and subcutaneous panniculitis

Question 31

Regarding Wilson disease, which is false? (March 2017)

a. It is due to excess transport of Copper from the liver into the bloodstream
b. Ceruloplasmin is low
c. Deposits in the cerebellum

Answer 31

Wilson disease:

• AR disorder (ATP7B gene, chromosome 13)
• Encodes a transmembrane copper transporting ATPase
• Variable age at presentation – mean 11 years
• Results in impaired copper excretion into the bile & a failure to incorporate copper into ceruloplasmin
• Inhibits ceruloplasmin excretion into the blood

Toxic levels of copper accumulated in many tissues:
- Liver, Brain, Eye

Clinical:

• Latent period (variable duration) followed by an acute spillage of non-ceruloplasmin bound copper into the blood
• Causes severe systemic illness: haemolysis & pathologic changes in the brain, corneas, kidneys, bones, joints & parathyroids
• Marked increase in urinary copper

o Hepatic changes (variable):

• Fatty change: mild to moderate w focal hepatocyte necrosis
• Acute fulminant hepatitis
• Chronic hepatitis: severe inflammation w hepatocyte necrosis mixed with changes of steatohepatitis (Hepatocyte ballooning and Mallory-Denk bodies)
• Eventual cirrhosis

o Toxic brain injury:

• Basal ganglia: putamen most common. Initial swelling, followed by atrophy; May demonstrate cavitation
• Other: Thalami; Midbrain (panda sign), pons and cerebellum
• Cortical and subcortical lesions – mostly frontal lobe

o Eye lesions: Kayser-Fleischer rings

Diagnosis:

• Decreased serum ceruloplasmin
• Increase in hepatic copper content
• Increased urinary excretion of copper
• Serum copper levels are variable depending on the stage of the disease

ANSWER: In Wilson disease, there is impaired transport of copper from the liver into the blood stream due to low ceruloplasmin. Although copper usually deposits in the basal ganglia (putamen most common), it can deposit in the cerebellum and brainstem.

Question 32

Polycystic kidney disease has no association with: (March 2015)

a. Liver fibrosis
b. Von Meyenberg complexes

Answer 32

Associations of ADPKD:
o Cerebral berry aneurysms
o Intracranial dolichoectasia
o Hypertension
o Colonic diverticulosis
o Bicuspid aortic valve
o Mitral valve prolapse
o Aortic dissection
o Multiple biliary hamartomas (Von Meyenberg complex)
o Cysts in other organs:
Liver (most common)
Ovaries
Spleen
Seminal vesicles
Prostate
Pancreas

ANSWER: Liver fibrosis is not associated with ADPKD, but it is associated with ARPKD

Question 33

What association makes PSC more likely than PBC? (March 2015)

a. Crohn’s disease
b. Cholestatic liver function tests
c. Anti-mitochondrial antigen
d. Elevated AFP

Answer 33

Primary sclerosing cholangitis

• Idiopathic inflammatory condition
• Affects the biliary tree resulting in multiple strictures and eventual cirrhosis

Pathology:

• Periaqueductal fibrosis (onion skin lesions)
• Periportal eosinophilic infiltrate
• Paucity of ducts

Epidemiology: Young males; mean age 40; 2:1 M>F

Associations:
Inflammatory bowel disease (70%) - UC > CD
Sjogren syndrome
Retroperitoneal fibrosis
Mediastinal fibrosis
Riedel thyroiditis
Orbital pseudotumour

Complications:

• Cholangiocarcinoma (15%)
• CRC (4x increased risk in IBD patients w PSC than w/o PSC; 10 times increased risk compared to background)
• HCC (related to cirrhosis)

Primary biliary cirrhosis:

• Chronic progressive cholestatic liver disease
• 1-2% of cirrhosis deaths
• 3rd most common indication for transplantation in adults

Epidemiology: Middle aged women with cholestatic liver function

Pathology:

• Destruction of small intrahepatic bile ducts, portal inflammation & progressive scarring
• Aetiology unknown
• Anti-mitochondrial antibody in 85-100%

Associations:

• Cholelithiasis (~40%)
• Other autoimmune diseases e.g. thyroiditis, systemic sclerosis
• ILD (~15%)

Complications:

• HCC (5%) - Additional risk w older patients, males, DM or past HBV infection
• Hepatic osteodystrophy

ANSWER: PSC is associated w Crohns – others are more common w primary biliary cirrhosis

Question 34

What is most associated with PBC? (August 2016)

a. Anti-smooth muscle antibody
b. Anti-mitochondrial antibody

Answer 34

• PBC is associated with anti-mitochondrial antibody (AMA) in 85-100%
• Anti-smooth muscle antibody is typically associated with autoimmune hepatitis

ANSWER: Anti-mitochondrial antibody is associated with primary biliary cirrhosis

Question 35

Which is not associated with primary biliary cirrhosis? (April 2013)

a. Osteodystrophy
b. Gallstones
c. Choledochocoele

Answer 35

Associations of primary biliary cirrhosis:
o Cholelithiasis (40%)
o Other autoimmune diseases (especially thyroiditis, systemic sclerosis)
o Interstitial lung disease
o HCC (greater risk in males with diabetes +/- HBV infection)
o Hepatic osteodystrophy

ANSWER: Choledochocoele is not associated with PBC

Question 36

Which is true regarding primary sclerosing cholangitis?

a. More common in males
b. Is usually an adenocarcinoma which is well to moderately differentiated
c. ? peak age

Answer 36

ANSWER: PSC is more common in males (2:1); the peak age is 40

Question 37

Which is false of primary biliary cirrhosis? (March 2014)

a. Elevated serum transaminases
b. Associated with anti-mitochondrial antibodies
c. Associated with HCC
d. Most commonly affects middle aged females
e. Involves both intra and extra hepatic radicals

Answer 37

• AST and ALT are elevated
• Anti-mitochondrial antibodies in 85-100%
• 5% complicated by HCC
• 9:1 female predominance
• PBC causes destruction of the small and medium sized intrahepatic ducts

ANSWER: PBC does not involve the intrahepatic and extrahepatic ducts, intrahepatic ducts only

Question 38

Which is the least likely feature of primary sclerosing cholangitis? (March 2016)

a. Anti-smooth muscle antibody
b. Is associated with cholangiocarcinoma
c. Mainly affects intrahepatic ductules
d. Results in biliary cirrhosis

Answer 38

PSC antibodies:
o 80% pANCA
o 20-50% ANA or anti-smooth muscle

• 15% risk of cholangiocarcinoma

Distribution:
o 88% involves intra and extrahepatic ducts
o 10% involves intrahepatic ducts only
o 2% involves extrahepatic ducts only

Pathological features (common):
o Periductal fibrosis (onion skin)
o Periportal eosinophilic infiltrate
o Paucity of ducts
o Cirrhosis (end-stage)

ANSWER: Primary sclerosing cholangitis most commonly affects the intra and extrahepatic ducts

Question 39

What is a recognised cause of cholesterol over pigment gallstones? (March 2015)

a. Crohn’s disease
b. Oral contraceptive pile
c. Sickle cell disease

Answer 39

Risk factors for cholesterol gallstones:
o Obesity, sedentary lifestyle and diet
o Rapid weight loss
o Oral contraceptive pill
o TPN
o Ethnicity
o Genetic predisposition
o Older age
o Female gender

Risk factors for pigment stones:
o Black pigment stones:
- Chronic haemolysis
- Liver cirrhosis
- Intestinal malabsorption (e.g. Crohn’s disease)
o Brown pigment stones:
- Bacterial infection
- Parasitic infection (e.g. Clonorchis)
- Biliary stasis

ANSWER: Oral contraceptive pill

Question 40

A patient has intra and extrahepatic biliary cysts. What is the Todani classification?

a. Type I
b. Type II
c. Type III
d. Type IV
e. Type V

Answer 40

Todani classification:

1. Extra-hepatic choledochal cyst
2. Bile duct diverticulum
3. Choledochocoele
4. Multiple communicating intra and extra-hepatic duct cysts
   a. Fusiform dilation of the entire extra-hepatic bile duct with extension of dilation of the intra-hepatic ducts
   b. Multiple cystic dilations involving only the extrahepatic bile duct
5. Caroli disease

ANSWER: Todani type IV

Question 41

A patient has a choledochal cyst which is localized to the duodenal wall. What is this classified as? (March 2017)

1. Type I
2. Type II
3. Type III
4. Type IV
5. Type V

Answer 41

ANSWER: Type III (choledochocoele)

Question 42

Regarding biliary atresia, which is the least correct? (April 2013)

1. The liver has giant cell granulomas on biopsy
2. It is associated with a preduodenal portal vein
3. Associated with situs ambiguous
4. Leads to cirrhosis by six months
5. Liver biopsy shows paucity of intrahepatic ducts

Answer 42

ANSWER: Biopsy of the liver shows an absence of multinucleated giant cells in biliary atresia. 10% is associated with heterotaxy syndrome.

Question 43

Renal transplant anti-rejection medication is most likely to cause (March 2016)

1. AMLs
2. Hepatic adenoma
3. FNH
4. Regenerative nodules

Answer 43

• Regenerative nodular hyperplasia:
  
  • Micro or macronodular transformation of hepatic parenchyma without fibrous septa between nodules
    
    • Larger nodules are called multiacinar (large) regenerative nodules
    • No potential for malignant transformation
    • Diffuse and focal changes have separate causative factors
  • Diffuse nodular regenerative nodules:
    
    • Other diseases and drugs e.g. myeloproliferative disorders and immunosuppression
    • Signs of portal hypertension common
  • Focal large regenerative nodules:
    
    • Caused by vascular derangement in the liver with decreased portovenous or hepatovenous flow
      
      • Common occurrence in patients with Budd-Chiari syndrome and portal vein thrombosis
    • Multiple nodules 0.5cm-5cm in size
    • Arterial enhancement with persistent enhancement throughout PV and delayed phases (no washout)
    • Takes up hepatobiliary agents

ANSWER: Patients with transplants on immunosuppressive medications are more likely to develop diffuse regenerative nodular hyperplasia.

Question 44

Which is false regarding hepatic adenoma? (March 2016)

1. Large lesions are more likely to haemorrhage
2. Subcapsular lesions are more likely to haemorrhage
3. Beta catenin lesions are more likely to be malignant
4. HNF1A mutated lesions occur almost universally in women

Answer 44

• Hepatic adenomas >35mm are more likely to haemorrhage
• Beta catenin lesions:
  
  • Male gender
  • Associated with hormones/anabolic steroids, glycogen storage disorders and FAP
  • Have a higher risk of transformation to HCC
• HNF1A mutated lesions:
  
  • Only in female patients with a history of oral contraceptive use

ANSWER: All options are true

Question 45

What is not associated with HCC? (March 2015)

1. Wilsons disease
2. Alpha-1 AT
3. Hereditary haemochromatosis
4. Glycogen storage type 1
5. Tyrosine

Answer 45

• Risk factors for HCC:
  
  • Infection:
    
    • HBV (10% 5 year cumulative risk)
    • HCV (30% 5 year cumulative risk)
  • Toxic:
    
    • Alcoholism (8% 5 year cumulative risk)
    • Food toxins
  • Biliary cirrhosis (5% 5 year cumulative risk)
  • Congenital biliary atresia
  • Inborn errors of metabolism:
    
    • Haemochromatosis (20% 5 year cumulative risk)
    • Alpha-1 AT
    • Type 1 glycogen storage disease
    • Wilson disease
    • Tyrosinaemia type 1
  • Type 1 storage diseases (with suboptimal treatment) predisposes the development of hepatic adenomas, with a small risk of malignant transformation
    
    • These patients are screened with serial AFP
• ANSWER: All options are inborn errors of metabolism which predispose the development of HCC. The development of HCC however is somewhat controversial in Wilson disease.*
• **Robbins: Wilsons disease probably increases the risk of HCC with much less frequency*

Question 46

Which is true regarding fibrolamellar carcinoma? (March 2016)

1. Peak incidence in 20s-30s
2. Predilection for women
3. Associated with a raised AFP
4. Occurs in cirrhotic livers

Answer 46

• Fibrolamellar hepatocellular carcinoma:
  
  • Epidemiology:
    
    • Young adults 20-40 years
    • No gender predilection
    • No association with cirrhosis, hepatitis viruses or alcohol
  • Pathology:
    
    • Often large tumours at presentation
    • May secrete oestrogen but do not typically secrete AFP
    • Fibrolamellar organization: cords of cells separated by sheet-like fibrous stroma
  • Imaging features:
    
    • Arterially enhancing
    • Central scar in 75%
      
      • May demonstrate delayed enhancement
    • Calcification in 35%
    • Regional nodal metastases in 50%
    • Do not accumulate Tc99m sulfur colloid, which distinguishes from FNH
  • Prognosis:
    
    • Less aggressive than HCC, but often presents at a more advanced stage
    • Resected tumours have a 75% 5 year survival
    • Unresectable tumours have a poor prognosis

ANSWER: The peak incidence of fibrolamellar HCC is 20s-30s

Question 47

Which association is false? (March 2017)

1. Cirrhosis and fibrolamellar hepatocellular carcinoma

Answer 47

ANSWER: Fibrolamellar carcinoma typically occurs in younger patients in non-cirrhotic livers

Question 48

Most associated with cholangiocarcinoma: (March 2015)

1. Arsenic
2. Primary biliary cirrhosis
3. Primary sclerosing cholangitis
4. Porcelain gallbladder
5. Gallstones

Answer 48

• Risk factors for cholangiocarcinoma:
  
  • Primary sclerosing cholangitis (major risk factor for Western countries)
  • Recurrent pyogenic cholangitis (major risk factor for endemic areas)
  • Choledocholithiasis>cholelithiasis
  • Asian liver flukes:
    
    • Opisthorchis viverrine
    • Clonorchis sinensis
  • Caroli’s disease/choledochal cysts
    
    • Lifetime risk 10-15%
  • Toxins:
    
    • Thorotrast
    • Dioxin
    • PVC
    • Heavy alcohol use
  • Viral infections: HIV, HBV, HCV, EBV

ANSWER: Primary sclerosing cholangitis

Question 49

Which is associated with cholangiocarcinoma? (March 2014)

1. Primary biliary cirrhosis
2. Choledochal cyst
3. Bile duct adenoma

Answer 49

ANSWER: Choledochal cyst

Question 50

Which is a risk factor for cholangiocarcinoma? (August 2014)

1. Chronic hepatitis B
2. Female gender
3. Hepatic fibrosis
4. Primary biliary cirrhosis

Answer 50

ANSWER: Chronic hepatitis B. Cholangiocarcinoma has a slight male predilection and is associated with primary sclerosing cholangitis.

Question 51

Gallbladder cancer is least likely: (August 2016)

1. With invasion into the liver
2. With peritoneal metastases
3. Gallbladder wall thickening
4. Portal lymph node metastases
5. Arises from a normal gallbladder

Answer 51

• Gallbladder carcinoma
  
  • Usually asymptomatic until advanced stages
    
    • Important to recognize as an incidental finding where curative treatment is still an option
  • Epidemiology
    
    • Most common primary biliary carcinoma
    • Most common in elderly women
  • Pathology
    
    • 90% are adenocarcinoma, ~10% squamous cell carcinoma
  • Risk factors
    
    • Chronic cholecystitis
    • Gallstones (70-90%)
    • Familial adenomatous polyposis syndrome
    • Inflammatory bowel disease
    • Porcelain gallbladder
    • Gallbladder polyps (>1cm, solitary and sessile)
  • Natural history
    
    • Symptoms dependent on which structures are invaded
      
      • Usually locally advanced at the time of diagnosis
    • Biliary obstruction causes jaundice
    • Lesions in the body or the fundus extend into the liver or adjacent colon or small bowel
      
      • Pain or symptoms of bowel obstruction
  • Imaging features:
    
    • One of three morphologies:
      
      • Intraluminal mass
      • Diffuse mural thickening
      • Mass replacing the gallbladder
        
        • May be the progression of intraluminal mass or diffuse thickening
        • Most common presentation
    • Features of advanced disease
      
      • Intrahepatic biliary dilatation
      • Invasion into adjacent structures
      • Lymphadenopathy
      • Peritoneal carcinomatosis
      • Hepatic and distant metastases

ANSWER: Gallbladder carcinoma is least likely to arise from a normal gallbladder, with a majority of patients having a history of chronic cholecystitis or gallstones

Question 52

Risk factors for angiosarcoma of the liver: (March 2015)

1. PVC

Answer 52

• Risk factors for liver angiosarcoma:
  
  • Haemochromatosis
  • NF1
  • Environmental exposure:
    
    • Thorotrast
    • PVC
    • Arsenic
    • Radiation
• Third most common primary liver tumour but rare (1% of primary liver tumours)
  
  • Poor prognosis – survival beyond one year uncommon
  • Often have lung and spleen metastases at presentation
  • Chemo and radiation therapy resistant

ANSWER: PVC

Question 53

What liver neoplasm gives a bile-stained mass?

1. HCC

Answer 53

• Macroscopic description of HCC:
  
  • Unifocal, multifocal or diffusely infiltrative soft tumour
  • Paler than normal liver tissue
    
    • May be bile stained
  • Snakelike masses of tumour which may involve the portal vein (35-80%), hepatic vein (20%) or inferior vena cava
  • Haemorrhage and necrosis common
  • Usually occurs in a cirrhotic liver, which is often enlarged

ANSWER: HCC will give a bile stained resected mass

Question 54

All of the following are complications of pregnancy except: (March 2014)

1. Hepatic haematoma
2. Thrombocytosis

Answer 54

• HELLP syndrome
  
  • Features:
    
    • Haemolysis
    • Elevated liver enzymes
    • Low platelets
  • Complications:
    
    • DIC
    • Hepatic infarction
    • Hepatic rupture
    • Hepatic haematoma
    • Placental abruption
  • Histology:
    
    • Deposits of fibrin within the liver
    • Haemorrhage
    • Hepatocellular necrosis surrounding the portal triads

ANSWER: Thrombocytosis is not a complication of pregnancy – thrombocytopaenia is part of HELLP Syndrome

Question 55

What is not a risk factor for pre-eclampsia? (August 2014)

1. Anti-phospholipid syndrome
2. Hypertension
3. Renal problems
4. Liver problems
5. Diabetes mellitus

Answer 55

• Preeclampsia
  
  • Characterised by high blood pressure and proteinuria
    
    • Severe disease: haemolysis, thrombocytopaenia, liver dysfunction, renal impairment, peripheral oedema, pulmonary oedema, visual disturbances
    • Eclampsia: above with seizures
  • Pathology:
    
    • Fibrin deposits in the periportal sinusoids associated with haemorrhage into the space of Disse, leading to periportal hepatocellular coagulative necrosis
  • Risk factors:
    
    • Obesity
    • Previous hypertension
    • Older age
    • Diabetes mellitus
    • SLE
      
      • Associated with anti-phospholipid antibody
    • Chronic renal disease
    • Pregnancy related factors:
      
      • First pregnancy
      • Twin pregnancy
      • New paternity
      • Prior IUGR
      • Prior stillbirth

ANSWER: Pre-existing maternal liver disease is not associated with preeclampsia

Question 56

Regarding pre-eclampsia and acute fatty liver of pregnancy, which makes pre-eclampsia most likely?

1. Coagulopathy
2. Neurological impairment
3. Proteinuria
4. Foetal distress
5. Abnormal LFTs

Answer 56

• Acute fatty liver of pregnancy:
  
  • Spectrum of presentation: subclinical hepatocyte dysfunction to hepatic failure, coma and death
    
    • Mortality is 20% secondary to complications: sepsis, renal failure, circulatory collapse, pancreatitis or GI bleeding
  • Mitochondrial dysfunction is usually implicated
    
    • Accumulation of toxic level fetal metabolites which causes maternal hepatotoxicity
  • Microscopically: microvesicular steatosis
    
    • Severe cases: portal inflammation, hepatocyte dropout and lobular disarray
  • Swansea criteria for diagnosis:
    
    • Vomiting
    • Abdominal pain
    • Polyuria/polydipsia
    • Encephalopathy
    • Elevated bilirubin
    • Hypoglycaemia
    • Elevated urea
    • Leukocytosis
    • Ascites or increased liver echogenicity on ultrasound
    • Transaminitis
    • Elevated ammonia
    • Renal impairment
    • Coagulopatht
    • Microvesicular steatosis on biopsy

ANSWER: Proteinuria is associated with pre-eclampsia, but not acute fatty liver of pregnancy

Question 57

What is the most common cause of jaundice in pregnancy? (March 2017)

1. Viral hepatitis
2. Cholestasis

Answer 57

ANSWER: Viral hepatitis is the most common cause of jaundice in pregnancy worldwide

Question 58

What is false regarding pancreatitis? (September 2013)

1. Associated with SPINK-1 mutation
2. Activation of trypsinogen is an important step
3. 10-20% of patients with gallstones develop pancreatitis
4. On gross pathology, the pancreas is yellow, chalky white
5. Can be caused by coxsackie virus

Answer 58

• Acute pancreatitis:
  
  • Reversible parenchymal damage associated with inflammation
  • Inappropriate activation and release of pancreatic enzymes – activation of trypsinogen is a key triggering event
    
    • Proelastase and prophospholipidase are activated and damage blood vessels and adipose tissue
    • Activation of the complement and coagulation pathways
    • Pancreatic inflammation and thrombosis causes further damage to acinar cells which amplify intraparenchymal enzyme activation
  • Underlying mechanisms:
    
    • Pancreatic duct obstruction
      
      • Gallstones/sludge – 30-40%
      • Neoplasms
      • Choledochocoeles
      • Parasites
      • Congenital abnormalities (pancreas divisum)
    • Primary acinar cell injury
      
      • Oxidative stress
      • Hypercalcaemia
    • Defective intracellular transport of proenzymes
    • Alcohol
      
      • Direct toxic effect
    • Metabolic disorders
    • Medications
    • Trauma
    • Ischaemia
    • Infection
      
      • Viruses: mumps, coxsackie B, HBV, CMV, VSV, HSV
      • Bacteria: mycoplasma, legionella
      • Fungi: aspergillus
      • Parasites: toxoplasma, cryptosporidium
    • Cystic fibrosis
    • Hereditary
      
      • SPINK-1: altered proteins fail to inhibit trypsin activity
      • PRSS1: activated trypsin is immune to its own self inhibition mechanisms

ANSWER: Pancreatitis occurs in 30-40% of patients with gallstones, not 10-20%

Question 59

Least likely found in association with IgG4 pancreatitis: (March 2015)

1. Chronic sclerosing sialadenitis
2. Mediastinal fibrosis
3. Thyroid disease

Answer 59

• Extrapancreatic manifestations of autoimmune/IgG4 pancreatitis:
  
  • Inflammatory bowel disease
  • Pulmonary involvement
  • Renal involvement
  • Biliary tree
  • Salivary glands – chronic sclerosing sialadenitis seen in IgG4 disease
• Pathology:
  
  • Periductal infiltrate of lymphocytes (CD4 and CD8) and plasma cells
  • Clusters of inflammatory cells in the walls of small veins, larger vessels and nerves
• IgG4-related sclerosing disease – the disease spectrum can include:
  
  • CNS:
    
    • Hypertrophic pachymeningitis
    • Hypophysitis
    • Chronic subdural haemorrhage
  • Head and neck:
    
    • Chronic sclerosing sialadenitis
    • Riedel’s thyroiditis
    • Orbital pseudotumour
    • Pansinusitis
  • Thoracic:
    
    • Interstitial pneumonia/IgG4 related lung disease
      
      • Solid nodular type
      • Rounded ground glass opacities
      • Alveolar interstitial type (honeycombing, bronchiectasis and diffuse ground glass opacity)
      • Bronchovascular type
    • IgG4 related cardiac disease
  • Abdominopelvic:
    
    • Autoimmune pancreatitis
    • Sclerosing cholangitis
    • Sclerosing mesenteritis
    • Cholecystitis
    • Tubulo-intersitial nephritis
    • Retroperitoneal fibrosis
    • Inflammatory aortic aneurysm
    • Prostatitis
  • Multisystem:
    
    • Inflammatory pseudotumours (e.g. presenting as a paravertebral mass)
    • Lymphadenopathy

ANSWER: Mediastinal fibrosis

Question 60

Pancreatic cysts are associated with which syndrome? (March 2016)

1. VHL
2. TS
3. NF1
   
   1. NF2

Answer 60

ANSWER: VHL

Question 61

Which pancreatic neoplasm has the least malignant potential?

1. Insulinoma
2. Islet cell tumour
3. IPMN
4. Solid pseudopapillary neoplasm
5. Pancreatic intraepithelial neoplasm

Answer 61

• Incidence of malignancy in lesions:
  
  • Insulinoma – 5-10%
  • Mucinous neoplasm – 33%
    
    • Considered a premalignant or malignant lesion and always excised
  • Main brain IPMN – 60%
  • Side branch IPMN – 5%
  • Pseudopapillary neoplasm – 15%
  • Pancreatic intraepithelial neoplasm – rare

ANSWER: Provided IPMN refers to both side and main branch lesions, insulinoma would have the least malignant potential of this list

Question 62

Which is false? (March 2015)

1. IPMN has jaundice
2. Malignant IPMN means lipase secretion
3. IPMN is typically in the body or tail

Answer 62

• Some cases of IPMN present with jaundice due to obstruction at the ampulla
• Lipase secretion:
  
  • Raised amylase/lipase marker of invasion in malignant IPMN
  • Lipase can be elevated if there is reactive pancreatitis in the setting of a benign IPMN
    
    • Intermittent obstruction of the pancreatic duct can lead to repeated bouts of acute pancreatitis and chronic pancreatitis
• Location:
  
  • Main branch IPMNs occur more commonly in the body and tail
  • Side branch IPMNs occur more commonly in the head and uncinated process

ANSWER: Malignant IPMN does not mean lipase secretion

Question 63

Which is least correct? (March 2015)

1. IPMN is more common in females

Answer 63

ANSWER: IPMN is not more common in females – most common in men over 60

Question 64

What diagnosis is most likely in a 20 year old with a pancreatic lesion?

1. Solid pseudopapillary neoplasm
2. Adenocarcinoma
3. Mucinous cystadenoma/adenocarcinoma
4. Serous cystadenoma/adenocarcinoma

Answer 64

• Solid pseudopapillary neoplasm
  
  • Very uncommon neoplasm
  • Women 20-30 years
  • Solid and cystic neoplasm with a capsule and early ‘haemangioma’ like enhancement
    
    • Central poor enhancement due to cystic or necrotic degeneration

ANSWER: Solid pseudopapillary epithelial neoplasm

Question 65

What is not a site of ectopic pancreas? (September 2013)

1. Stomach
2. Duodenum
3. Ileum
4. Jejunum
5. Colon

Answer 65

• Most common sites of ectopic pancreas:
  
  • Stomach (antrum)
  • Duodenum (proximal)
  • Within a Meckel’s diverticulum

ANSWER: Ectopic pancreas is not seen within the colon

Question 66

Polyarthralgia and skin fat necrosis is associated with: (August 2014)

1. Gastrinoma
2. Somatostatinoma
3. Islet cell tumour
4. Pancreatic ductal adenocarcinoma

Answer 66

• Clinical presentation of pancreatic ductal adenocarcinoma:
  
  • Pain (most common)
  • Couvoisier’s gallbladder: painless jaundice and an enlarged gallbladder
  • Trosseau syndrome: migratory thrombophlebitis
  • New onset diabetes mellitus;
  • Lipase hypersecretion syndrome (10-15% of pancreatic ductal adenocarcinoma)
    
    • Polyarthralgia and skin fat necrosis +/- lytic bone lesions
    • Elevated serum lipase and eosinophilia
• Risk factors for pancreatic ductal adenocarcinoma:
  
  • Cigarette smoking (strongest environmental risk factor)
  • Diet rich in animal fats and proteins
  • Obesity
  • Family history: three or more first order relatives increases risk by 20%
  • Hereditary syndromes:
    
    • HNPCC
    • Familial breast cancer (BRCA 1 and 2)
    • Familial atypical multiple mole melanoma (FAMMM)
    • Hereditary pancreatitis
    • Ataxia-telangectasia
    • Peutz-Jegher
  • Weak/no association with heavy alcohol use
• Precursor lesions to pancreatic ductal adenocarcinoma:
  
  • Pancreatic intraepithelial neoplasia (PanIN)
  • IPMN
  • Mucinous cystic neoplasm
• Histological subtypes:
  
  • Adenocarcinoma
  • Acinar cell carcinoma of the pancreas
  • Adenosquamous carcinoma of the pancreas
  • Undifferentiated with osteoclasts and giant cells

ANSWER: Pancreatic ductal adenocarcinoma is associated with a syndrome of polyarthralgias and skin necrosis – lipase hypersecretion syndrome, which is seen in 10-15%

Question 67

Adenocarcinoma of the head of the pancreas is associated with: (March 2016)

1. A normal parenchymal volume of the body and tail of the pancreas

Answer 67

ANSWER: False – upstream pancreatic atrophy is associated with chronic pancreatitis, adenocarcinoma of the pancreatic head and main branch IPMN

Question 68

Which of these is not associated? (August 2014)

1. VIPoma with hyperkalaemia

Answer 68

• VIPoma: WDHA
  
  • Watery diarrhoea, hypokalaemia, achlorhydria
• Gastrinoma:
  
  • Excessive acid secretion in the stomach
  • Hyperchlorhydria
  • Peptic ulcers disease
  • Zollinger-Ellison syndrome if multiple
  • Strongest association with MEN I
• Somatostatinoma:
  
  • Mild diabetes mellitus, cholelithiasis and diarrhoea/steatorrhoea
• Insulinoma:
  
  • Whipple’s triad: fasting hypoglycaemia, symptoms of hypoglycaemia, relief of symptoms with administration of glucose
  • Associated with MEN I
• Glucagonoma:
  
  • 4Ds
    
    • Dermatitis/necrotising migratory erythema
    • Diabetes mellitus
    • Deep vein thrombosis
    • Depression

ANSWER: VIPoma is associated with hypokalaemia