Congenital protein C deficiency in a newborn

Abstract

Protein C deficiency is a rare but life-threatening bleeding disorder that may present in the nearest neonatal period. This article presents the case of a newborn girl with acute and progressive neonatal fulminant purpura, thrombosis, DICsyndrome, intracranial hemorrhage, which developed within 4 days after birth as a manifestation of protein С deficiency. Protein С activity was below 5%. Treatment includes correction of coagulopathy, intensive wound care including negative pressure dressings and skin grafting, and supportive care for central nervous system problems. Long-term follow-up consists of lifelong anticoagulant therapy to avoid recurrence of these complications.