8.18 Acute Fatty Liver of Pregnancy

Question 1

What is the incidence and mortality of acute fatty liver of pregnancy?

Answer 1

1 in 6,600-13,000 so rare
 high mortality:
18% mother
23% fetus
usually after 30 weeks

Question 2

What condition does the fetus have if the mother has acute fatty liver of pregnancy?

Answer 2

long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency

Question 3

What does LCHAD deficiency mean for fetus?

Answer 3

Impaired metabolism of fatty acids for fuel by fetus and placenta.
FAs accumulate in mums circulation.

Question 4

How is LCHAD deficiency inherited?

Answer 4

autosomal recessive
(so mother will have one defective gene copy too - may impair her clearance too)

Question 5

What accumulation of FAs in mothers liver lead to?

Answer 5

Inflammation and liver failure

Question 6

How does acute fatty liver of pregnancy present?

Answer 6

• abdo pain
• ascites
• jaundice
• headache
• vomiting
• (pancreatitis)

Question 7

What bloods would you expect to see in acute fatty liver of pregnancy?

Answer 7

elevated liver enzymes - ALT and AST.

Others:

• Raised bilirubin
• Raised WBC count
• Deranged clotting (raised prothrombin time and INR)
• Low platelets

Question 8

What should you think if you see elevated liver enzymes and low platelets in pregnancy?

Answer 8

think HELLP syndrome as this is more common
(haemolysis, elevated liver enzyme, low platelets - complication of pre/eclampsia)

acute fatty liver of pregnancy should also be a differential

Question 9

How do you manage acute fatty liver of pregnancy?

Answer 9

obstetric emergency, prompt delivery of baby

haematologists, correct clotting.
CI regional anaesthesia and epidural

treat liver failure, consider liver transplant

v high mortality