Acute Intermittent Porphyria and Pregnancy / 대한산부인과학회잡지

ABSTRACT

The Porphyria are a group of inherited and acquired disorders characterized by partial defects in the heme biosynthetic pathway. Among the hepatic forms, acute intermittent porphyria(AIP) is the most severe and common type in western hemisphere. Though its association with pregnancy is rare, it presents the obstetrician with challenging problems in diagnosis and management and it is probable that pregnancy had some deleterious effect in acute porphyria. The authors present a cae of AIP in pregnancy with a review of literature.