Liver In Multi-System Disease

Question 1

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

Answer 1

• Hypoxia
• Congestion
• Low cardiac output

Question 2

What does chronic low cardiac output lead to?

Answer 2

Reduced blood flow to the liver

Question 3

How might the liver compensate for chronically reduced blood flow?

Answer 3

Increased portal blood flow

Question 4

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

Answer 4

Jaundice and raised transaminases

Question 5

What does chronic fluid retention lead to?

Answer 5

Sodium retention and increased circulating blood volume

Question 6

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

Answer 6

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

Question 7

When does hepatic congestion result from cardiac disease?

Answer 7

When there is an increase in right atrial or ventricular pressure

Question 8

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

Answer 8

• Pulmonary atresia
• Tetralogy of Fallot

Question 9

What does sinusoidal engorgement cause on blood tests?

Answer 9

Deranged transaminases with normal bilirubin

Question 10

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

Answer 10

• Hepatomegaly
• Hypoalbuminaemia
• Cirrhosis
• Portal hypertension

Question 11

What liver pathology might inflammatory bowel disease be associated with?

Answer 11

• Chronic hepatitis
• Autoimmune sclerosing colangitis

Question 12

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

Answer 12

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

Question 13

How does liver disease associated with IBD present?

Answer 13

• Hepatomegaly with or without jaundice
• Stigmata of chronic liver disease

Question 14

How does intestinal failure affect bile?

Answer 14

• Reduced stimulation of bile flow
• Impaired reabsorption of bile salts

Question 15

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

Answer 15

Biliary sludge/gall stones form

Question 16

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

Answer 16

Terminal ileum

Question 17

What is the effect of terminal ileum disease on bile?

Answer 17

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

Question 18

What are the clinical features of terminal ileum disease?

Answer 18

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

Question 19

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

Answer 19

• Increased risk of sepsis
• Lack of extrahepatic circulation

Question 20

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

Answer 20

Portal and pericellular fibrosis

Question 21

What is invariable present on presentation in IFALD?

Answer 21

Hepatosplenomegaly

Question 22

What is found on blood tests in IFALD?

Answer 22

• Raised liver enzymes
• Conjugated hyperbilirubinaemia

Question 23

What can help in IFALD?

Answer 23

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

Question 24

How can IFALD be treated in severe cases?

Answer 24

Small bowel or multivisceral transplant

Question 25

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

Answer 25

- Alagille syndrome - Infantile polycystic disease (autosomal recessive)

Question 26

What renal problems are seen in Alagille syndrome?

Answer 26

- Renal tubular dysfunction - Glomerular disease

Question 27

What hepatic problems can be seen in infantile polycystic disease?

Answer 27

Hepatic fibrosis

Question 28

How can sickle cell disease cause liver problems?

Answer 28

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

Question 29

What does sequesteration in hepatic sinusoids cause?

Answer 29

Capillary obstruction

Question 30

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

Answer 30

- Haemolytic anaemia - Unconjugated jaundice - Splenomegaly - Hepatomegaly - Progressive liver disease due to necrosis and infarction - Gallstones - Viral hepatitis

Question 31

What is veno-occlusive disease?

Answer 31

A serious complication of bone marrow transplant

Question 32

When does VOD usually present?

Answer 32

In the first month after transplant

Question 33

What are the clinical features of VOD?

Answer 33

- Variable degrees of jaundice - Hepatomegaly - Elevated JVP - Ascites

Question 34

What are the clinical features of VOD similar do?

Answer 34

Budd-Chiari syndrome

Question 35

What is Budd-Chiari syndrome?

Answer 35

A rare syndrome caused by hepatic vein thrombosis or obstruction

Question 36

How is a diagnosis of VOD made?

Answer 36

- Abdominal USS - Liver biopsy

Question 37

What is found on abdominal USS in VOD?

Answer 37

- Retrograde flow within the portal veins - Increased hepatic resistance

Question 38

What is found on liver biopsy in VOD?

Answer 38

- Occlusion of hepatic venules - Necrotic hepatocytes - Fibrosis and cirrhosis

Question 39

What can be used in the management of VOD?

Answer 39

- Supportive management - Defibrotide - Transplantation

Question 40

What is defibrotide?

Answer 40

A thrombolytic drug

Question 41

What can the use of defibrotide in VOD lead to?

Answer 41

Complete resolution of symptoms

Question 42

When might transplantation be considered in VOD?

Answer 42

Later stages with ongoing chronic liver disease