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
{"title":"重度先天性蛋白C缺乏的新生儿暴发性紫癜1例","authors":"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","doi":"10.1016/j.jdds.2016.10.001","DOIUrl":null,"url":null,"abstract":"<div><p>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.</p></div>","PeriodicalId":43409,"journal":{"name":"Journal of Dermatology & Dermatologic Surgery-JDDS","volume":"21 2","pages":"Pages 104-106"},"PeriodicalIF":0.2000,"publicationDate":"2017-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jdds.2016.10.001","citationCount":"3","resultStr":"{\"title\":\"Neonatal purpura fulminans in newborn with severe congenital protein C deficiency: Case report\",\"authors\":\"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\",\"doi\":\"10.1016/j.jdds.2016.10.001\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<div><p>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.</p></div>\",\"PeriodicalId\":43409,\"journal\":{\"name\":\"Journal of Dermatology & Dermatologic Surgery-JDDS\",\"volume\":\"21 2\",\"pages\":\"Pages 104-106\"},\"PeriodicalIF\":0.2000,\"publicationDate\":\"2017-07-01\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://sci-hub-pdf.com/10.1016/j.jdds.2016.10.001\",\"citationCount\":\"3\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Journal of Dermatology & Dermatologic Surgery-JDDS\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://www.sciencedirect.com/science/article/pii/S2352241016300275\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"Q4\",\"JCRName\":\"DERMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Dermatology & Dermatologic Surgery-JDDS","FirstCategoryId":"1085","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S2352241016300275","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q4","JCRName":"DERMATOLOGY","Score":null,"Total":0}
Neonatal purpura fulminans in newborn with severe congenital protein C deficiency: Case report
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.