Neonatal purpura fulminans in newborn with severe congenital protein C deficiency: Case report

IF 0.2 Q4 DERMATOLOGY
Sultan A. Jafarri , Khalid M. AlAttas , Sultan M. Bajawi , Mohammad K. Ahsan , Ali M. Al-Sheikh , Mohammed A. Buraik , Amr Mohammed Gamal , Nadia S. Mustafa
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引用次数: 3

Abstract

Neonatal purpura fulminans (PF) is a rare, life-threatening condition, caused by congenital or acquired deficiencies of protein C or S. PF describes a clinico-pathological entity of dermal microvascular thrombosis associated with disseminated intravascular coagulation (DIC) and perivascular hemorrhage occurring in the newborn period. Here we describe a newborn with PF due to severe congenital protein C deficiency. The lesions started 5 h after birth but the infant was brought to our emergency department 20 h later. The infant was admitted in neonatal intensive care unit (NICU) and treated with fresh frozen plasma (FFP), enoxaparin along with other supportive cares. In spite of impressive improvement of skin lesions, coordination with numerous subspecialties and aggressive NICU support, the infant died one month after admission due to multiorgan failure and septicaemia.

重度先天性蛋白C缺乏的新生儿暴发性紫癜1例
新生儿暴发性紫癜(PF)是一种罕见的、危及生命的疾病,由先天性或获得性蛋白C或s的缺乏引起。PF描述了新生儿期皮肤微血管血栓形成相关的弥散性血管内凝血(DIC)和血管周围出血的临床病理实体。这里我们描述了一个新生儿与PF由于严重的先天性蛋白C缺乏。病变在出生后5小时开始,但婴儿在20小时后被带到我们的急诊科。该婴儿入住新生儿重症监护病房(NICU),接受新鲜冷冻血浆(FFP)、依诺肝素及其他支持性护理。尽管皮肤病变得到了显著的改善,许多亚专科的配合和积极的NICU支持,但婴儿在入院后一个月因多器官衰竭和败血症死亡。
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