Abetalipoproteinemia complicating the puerperium.
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.


METHODS
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.


CONCLUSIONS
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.
Palmer
Andrea B
AB
Department of Obstetrics and Gynecology, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma 73190, USA. Andrea-Palmer@ouhsc.edu
Knudtson
Eric J
EJ
D000012:Abetalipoproteinemia / Q000150:complications / Q000175:diagnosis* / Q000628:therapy*; D000328:Adult; D004211:Disseminated Intravascular Coagulation / Q000175:diagnosis / Q000209:etiology / Q000628:therapy; D005260:Female; D006801:Humans; D011644:Puerperal Disorders / Q000175:diagnosis* / Q000209:etiology / Q000628:therapy*