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  • Title: Abetalipoproteinemia complicating the puerperium.
    Author: Palmer AB, Knudtson EJ.
    Journal: Obstet Gynecol; 2008 Feb; 111(2 Pt 2):575-7. PubMed ID: 18239027.
    Abstract:
    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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