Acute fatty liver of pregnancy

• acute fatty liver of pregnancy has a rapid onset in the third trimester. It has an unknown aetiology
  • there are however case reports revealing a correlation between pregnancy complications such as acute fatty liver of pregnancy and long-chain 3-hydroxyacyl-coenzyme A dehydrogenase (LCHAD) deficiency in the fetus (1,2)
    
    
• this disease results form accumulation of microvesicular fat in hepatocytes - this condition is associated with potentially rapid onset of liver failure
  
  
• it is rare - 1 in 7000 to 1 in 16000 pregnancies (3,4). Note however that a UK study revealed a significantly higher incidence of about 1 in 1,000 pregnancies (5)
  
  
• it is associated with raised bilirubin and transaminase levels; other possible features include hyperuricaemia, thrombocytopaenia and pancreatitis
  
  
• clinical features include vomiting, abdominal pain, and headache. The mother will be jaundiced
  
  
• disease progression may lead to renal failure, clotting disorders and hypoglycaemia
  
  
• treatment involves hospitalisation, immediate delivery, and treatment of liver failure (and renal failure). There is a high maternal and fetal mortality associated with this condition
  • maternal mortality rate has been estimated at 18%, and neonatal mortality rates have ranged from 7% to 58% (3,4)

Reference:

1. Castro MA et al. Reversible peripartum liver failure: a new perspective on the diagnosis, treatment, and cause of acute fatty liver of pregnancy based on 28 cases, Am J Obstet Gynecol 1999;181: 389–395.
2. Reyes H et al. Acute fatty liver of pregnancy: a clinical study of 12 episodes in 11 patients. Gut 1994; 35:101–106.
3. Tyni T et al. Pregnancy complications are frequent in longchain 3-hydroxyacyl-coenzyme A dehydrogenase deficiency. Am J Obstet Gynecol 1998;178:603-8.
4. Ibdah JA et al. A fetal fatty-acid oxidation disorder as a cause of liver disease in pregnant women. N Engl J Med 1999;340:1723- 31.
5. Ch'ng L et al. Prospective study of liver dysfunction in pregnancy in Southwest Wales Gut, Dec 2002; 51: 876 - 880.