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TAS Hepatology > Liver In Multi-System Disease > Flashcards

Liver In Multi-System Disease Flashcards

1
Q

What does the pathophysiology of hepatic dysfunction in cardiac disease relate to?

A
  • Hypoxia
  • Congestion
  • Low cardiac output
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2
Q

What does chronic low cardiac output lead to?

A

Reduced blood flow to the liver

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3
Q

How might the liver compensate for chronically reduced blood flow?

A

Increased portal blood flow

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4
Q

What may be found on bloods with hepatic dysfunction related to cardiac disease?

A

Jaundice and raised transaminases

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5
Q

What does chronic fluid retention lead to?

A

Sodium retention and increased circulating blood volume

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6
Q

What happens to the RAAS system in chronic fluid retention related to cardiac disease?

A

It is initially supressed, but as liver disease related to the cardiac disease progresses it becomes activated

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7
Q

When does hepatic congestion result from cardiac disease?

A

When there is an increase in right atrial or ventricular pressure

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8
Q

Give 2 examples of congenital heart diseases that can result in hepatic congestion

A
  • Pulmonary atresia
  • Tetralogy of Fallot
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9
Q

What does sinusoidal engorgement cause on blood tests?

A

Deranged transaminases with normal bilirubin

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10
Q

What are the clinical features of hepatic dysfunction associated with cardiac disease?

A
  • Hepatomegaly
  • Hypoalbuminaemia
  • Cirrhosis
  • Portal hypertension
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11
Q

What liver pathology might inflammatory bowel disease be associated with?

A
  • Chronic hepatitis
  • Autoimmune sclerosing colangitis
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12
Q

What is the pathogenesis of the association between inflammatory bowel disease and liver disease?

A
  • Exposure of cytokines produced in the lamina propria of the intestine
  • Production of autoantibodies
  • Reduction in suppressor T cells
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13
Q

How does liver disease associated with IBD present?

A
  • Hepatomegaly with or without jaundice
  • Stigmata of chronic liver disease
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14
Q

How does intestinal failure affect bile?

A
  • Reduced stimulation of bile flow
  • Impaired reabsorption of bile salts
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15
Q

What is the result of reduced stimulation to bile flow in children with intestinal failure?

A

Biliary sludge/gall stones form

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16
Q

What region of the intestine is responsible for reabsorption of bile salts?

A

Terminal ileum

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17
Q

What is the effect of terminal ileum disease on bile?

A

There is impaired processing, which leads to a reduced bile acid pool, altering the composition of bile and making it more lithogenic

18
Q

What are the clinical features of terminal ileum disease?

A
  • Steatorrhoea
  • Abdominal pain
  • Obstructive jaundice
  • Fat-soluble vitamin deficiency
  • Anaemia
19
Q

What causes intestinal failure-associated liver disease (IFALD)?

A
  • Increased risk of sepsis
  • Lack of extrahepatic circulation
20
Q

What does a lack of extrahepatic circulation in IFALD lead to?

A

Portal and pericellular fibrosis

21
Q

What is invariable present on presentation in IFALD?

A

Hepatosplenomegaly

22
Q

What is found on blood tests in IFALD?

A
  • Raised liver enzymes
  • Conjugated hyperbilirubinaemia
23
Q

What can help in IFALD?

A
  • Manipulation of type of lipid in parenteral nutrition
  • Reintroducing enteral intake
  • Reducing risk of sepsis
24
Q

How can IFALD be treated in severe cases?

A

Small bowel or multivisceral transplant

25
Q

Give 2 examples of syndromic conditions associated with liver and renal disease?

A
  • Alagille syndrome
  • Infantile polycystic disease (autosomal recessive)
26
Q

What renal problems are seen in Alagille syndrome?

A
  • Renal tubular dysfunction
  • Glomerular disease
27
Q

What hepatic problems can be seen in infantile polycystic disease?

A

Hepatic fibrosis

28
Q

How can sickle cell disease cause liver problems?

A

Sequesteration can occur as a result of HbS increasing blood viscosity, and hepatic sinusoids are a common site of sequesteration

29
Q

What does sequesteration in hepatic sinusoids cause?

A

Capillary obstruction

30
Q

What are the clinical features of sequesteration in hepatic sinusoids in sickle cell disease?

A
  • Haemolytic anaemia
  • Unconjugated jaundice
  • Splenomegaly
  • Hepatomegaly
  • Progressive liver disease due to necrosis and infarction
  • Gallstones
  • Viral hepatitis
31
Q

What is veno-occlusive disease?

A

A serious complication of bone marrow transplant

32
Q

When does VOD usually present?

A

In the first month after transplant

33
Q

What are the clinical features of VOD?

A
  • Variable degrees of jaundice
  • Hepatomegaly
  • Elevated JVP
  • Ascites
34
Q

What are the clinical features of VOD similar do?

A

Budd-Chiari syndrome

35
Q

What is Budd-Chiari syndrome?

A

A rare syndrome caused by hepatic vein thrombosis or obstruction

36
Q

How is a diagnosis of VOD made?

A
  • Abdominal USS
  • Liver biopsy
37
Q

What is found on abdominal USS in VOD?

A
  • Retrograde flow within the portal veins
  • Increased hepatic resistance
38
Q

What is found on liver biopsy in VOD?

A
  • Occlusion of hepatic venules
  • Necrotic hepatocytes
  • Fibrosis and cirrhosis
39
Q

What can be used in the management of VOD?

A
  • Supportive management
  • Defibrotide
  • Transplantation
40
Q

What is defibrotide?

A

A thrombolytic drug

41
Q

What can the use of defibrotide in VOD lead to?

A

Complete resolution of symptoms

42
Q

When might transplantation be considered in VOD?

A

Later stages with ongoing chronic liver disease

TAS Hepatology (10 decks)

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