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Published on: November 20, 2015
Abetalipoproteinemia complicating the puerperium
Andrea B Palmer1, Eric J Knudtson
1Department of Obstetrics and Gynecology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73190, USA. Andrea-Palmer@ouhsc.edu
Background:
Abetalipoproteinemia is a rare, autosomal recessive disease, in which the absence of beta-lipoprotein results in the malabsorption of fat-soluble vitamins. There are few reported complications from abetalipoproteinemia during pregnancy. We present a case of untreated abetalipoproteinemia complicating the puerperium.
Case:
A 23-year-old, gravida 3, para 0020 woman presented to an outside facility in labor, and her delivery was complicated by postpartum hemorrhage and a large vulvar hematoma. She was coagulopathic and transferred for suspected disseminated intravascular coagulation. Her preexisting medical history was not appreciated by the transferring facility.
Conclusion:
Abetalipoproteinemia in pregnancy is rare. Untreated disease conveys multi-system organ dysfunction and has ramifications in labor and delivery. Clinicians must elicit a comprehensive medical history to properly manage complications in the puerperium.
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