Review Question - QID 221287

This pregnant patient with nausea, vomiting, severe abdominal pain, hyperammonemia, hypoglycemia, elevated aminotransferases, and thrombocytopenia, most likely has acute fatty liver disease of pregnancy (AFLP).

AFLP is a rare disease that typically occurs in the third trimester of pregnancy. This disease occurs due to failure of fatty acid beta-oxidation and presents with right upper quadrant pain, nausea, vomiting, and jaundice. Pathology demonstrates acute liver failure secondary to extensive microvesicular fatty infiltration of the liver. Like other pregnancy-related disorders of the liver such as pre-eclampsia and HELLP syndrome, AFLP presents with elevated aminotransferases and leukocytosis. AFLP is distinguished by signs of acute-onset fulminant liver failure manifesting through laboratory findings of hypoglycemia, thrombocytopenia, hyperammonemia, and possible disseminated intravascular coagulopathy. Patients with AFLP can decompensate quickly with multi-organ failure and fetal compromise. Management is immediate delivery.

Natarajan et al. review the role of 3-hydroxy fatty acid-induced hepatic lipotoxicity in AFLP. They find that maternal AFLP is highly associated with a mutation in mitochondrial long-chain hydroxy acyl-CoA dehydrogenase. They recommend further study of the effects of 3-hydroxy fatty acids in causing placental toxicity and fetal retinal pigmented epithelial lipoapoptosis.

Figure/Illustration A is an ultrasound of a fetus showing normal facial morphology (red circle). There is no evidence of fetal abnormalities on this image.

Incorrect Answers:
Answer 1: Acetaminophen toxicity can present with signs of fulminant liver failure, although laboratory values are typically notable for aminotransferase values > 1000 U/L. This patient’s symptoms preceded her ingestion of acetaminophen.

Answer 3: Eclampsia is a pregnancy disorder defined as pre-eclampsia with new-onset seizures. While this patient is hypertensive, she also has a normal urinalysis and signs of fulminant liver failure, which would not be explained by eclampsia alone.

Answer 4: Hemolysis, elevated liver enzymes, and low platelets syndrome also causes hepatic inflammation and can present similarly with right upper quadrant pain, vomiting, thrombocytopenia, and elevated aminotransferases in a patient with pre-eclampsia. HELLP syndrome can be distinguished from AFLP because it lacks the symptoms of acute liver failure (hyperammonemia, hyperbilirubinemia, and hypoglycemia) found in patients with AFLP.

Answer 5: Preeclampsia is a hypertensive pregnancy disorder that typically occurs during the third trimester. It is defined as hypertension with a systolic blood pressure > 140 mmHg or a diastolic blood pressure > 90 mmHg and proteinuria of more than 300 mg/24 hours.

Bullet Summary:
Acute fatty liver disease of pregnancy develops during the third trimester and presents with abdominal pain, vomiting, and laboratory findings of acute fulminant liver failure.