Kelly Alicia, Pearson Gregory D
NICU, Nationwide Children’s Hospital.
Neonatal Netw. 2011 May-Jun;30(3):153-9. doi: 10.1891/0730-0832.30.3.153.
Protein C (PC) deficiency is a rare but life-threatening bleeding disorder that can present in the immediate neonatal period. This article presents the case of a baby girl with acute and progressive neonatal purpura fulminans as the presenting feature of PC deficiency. Other common complications of this disease include ophthalmic problems and central nervous system (CNS) changes. Management consists of correcting the coagulopathy, intensive wound care including negative-pressure dressings and skin grafting, and supportive care for the ophthalmic and CNS issues. Long-term follow-up consists of lifelong anticoagulant therapy to avoid recurrence of these complications.
蛋白C（PC）缺乏症是一种罕见但危及生命的出血性疾病，可在新生儿早期出现。本文介绍了一名女婴的病例，其以急性进行性新生儿暴发性紫癜为蛋白C缺乏症的首发特征。该疾病的其他常见并发症包括眼科问题和中枢神经系统（CNS）改变。治疗包括纠正凝血病、强化伤口护理（包括负压敷料和皮肤移植）以及针对眼科和中枢神经系统问题的支持性护理。长期随访包括终身抗凝治疗，以避免这些并发症的复发。
