TY - JOUR T1 - Severe vitamin deficiencies in pregnancy complicated by progressive familial intrahepatic cholestasis JF - BMJ Case Reports JO - BMJ Case Reports DO - 10.1136/bcr-2020-240248 VL - 14 IS - 3 SP - e240248 AU - Amanda Caroline Mahle AU - Bernard David Morris AU - Zane Frazer AU - Christopher Novak Y1 - 2021/03/01 UR - http://casereports.bmj.com/content/14/3/e240248.abstract N2 - 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. ER -