Atif Zaman, MD, MPH reviewing Liu J et al. Am J Gastroenterol 2017 Mar 14.
The state of the science and clinical practice for early recognition and management of this potentially fatal condition
Acute fatty liver disease of pregnancy (AFLP) is an uncommon but serious obstetrical emergency, usually occurring during the third trimester. The current review covering etiology, diagnosis, clinical outcomes, and management of AFLP includes these key take-home points:
Fetal fatty acid oxidation defects (FAODs), which are deficiencies of enzymes related to mitochondrial metabolism of fatty acids (e.g., fetal long-chain 3-hydroxyacyl-CoA dehydrogenase), are probable risk factors for AFLP.
Established risk factors include multigravida state, male sex of fetus, previous AFLP episode, and co-occurrence of another liver disease during pregnancy (e.g., preeclampsia).
Worldwide maternal mortality estimates have decreased from nearly 100% to <10% due to early recognition and prompt delivery of the fetus. Mothers recover in about 1 to 3 weeks post-delivery, and long-term follow-up data show no serious maternal adverse events. Conversely, fetal mortality remains high, estimated at 20%. Fetal/newborn screening for FAODs has been proposed, but further studies are needed to determine if screening improves outcomes.
Unlike other common liver diseases in pregnancy, AFLP presents with liver dysfunction manifested as elevated international normalized ratio, ascites, and encephalopathy. The Swansea criteria, which include many elements of liver dysfunction, is a diagnostic tool with 100% sensitivity and 57% specificity. A liver biopsy is not necessary to diagnose AFLP.
Once a diagnosis of AFLP is made, prompt delivery of the fetus and supportive care are indicated. In case of deteriorating maternal clinical condition or instability, liver transplantation should be considered.
CITATION(S):
Liu J et al. Acute fatty liver disease of pregnancy: Updates in pathogenesis, diagnosis, and management. Am J Gastroenterol 2017 Mar 14; [e-pub].
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