RT Journal Article
SR Electronic
T1 Severe vitamin deficiencies in pregnancy complicated by progressive familial intrahepatic cholestasis
JF BMJ Case Reports
JO BMJ Case Reports
FD BMJ Publishing Group Ltd
SP e240248
DO 10.1136/bcr-2020-240248
VO 14
IS 3
A1 Amanda Caroline Mahle
A1 Bernard David Morris
A1 Zane Frazer
A1 Christopher Novak
YR 2021
UL http://casereports.bmj.com/content/14/3/e240248.abstract
AB Progressive familial intrahepatic cholestasis (PFIC) is a rare disease of impaired bile acid excretion which can lead to nutritional deficiencies. Vitamin deficiencies during pregnancy can result in adverse maternal and fetal outcomes. A 20-year-old primiparous woman at 30 4/7 weeks with PFIC type 2 presented with worsening cholestasis, coagulopathy and fat-soluble vitamin deficiency. She developed visual deficits and was found to have severe vitamin A deficiency. Her coagulopathy and visual deficits improved following vitamin K and A supplementation, respectively. She delivered at 32 2/7 weeks following preterm labour. This case highlights several unique aspects in the care of pregnant women with liver disease. These patients are at risk for fat-soluble vitamin deficiencies which can result in significant coagulopathy and rarely, visual deficits due to vitamin A deficiency. Prompt treatment is necessary to prevent permanent sequelae.