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Protein C 결핍에서 발생한 소모성 응고질환으로 인한 뇌내출혈로 야기된 뇌성마비 환자에 대한 증례기록

Other Titles
 Cerebral Palsy due to Intracranial Hemorrhage Caused by Consumptive Coagulopathy in Protein C Deficiency: A Case Report 
Authors
 조유나  ;  이영목  ;  박은숙  ;  최자영  ;  박천웅  ;  나동욱 
Citation
 Journal of the Korean Child Neurology Society (대한소아신경학회지), Vol.25(1) : 44-47, 2017 
Journal Title
 Journal of the Korean Child Neurology Society (대한소아신경학회지) 
ISSN
 1226-6884 
Issue Date
2017
Keywords
Purpura fulminans ; Cerebral palsy ; Protein C deficiency
Abstract
Protein C (PROC) is a potent anticoagulant inactivating coagulation factors Va and VIIIa. PROC deficiency is very rare condition inherited as an autosomal dominant or recessive trait, and associated with various thromboembolic and ischemic conditions. Moreover, severe form of PROC deficiency can cause fatal hemorrhagic complications due to consumptive coagulopathy. We reported two children with hemorrhagic stroke who were diagnosed as severe PROC deficiency caused by two different types of compound heterozygous PROC gene mutations. We described results of laboratory tests, genetic analysis, brain magnetic resonance images, and functional outcomes. Both children received prophylactic anticoagulation therapy and presented with purple-colored skin lesions during rehabilitation. Purpura fulminans caused by insufficient anticoagulation should be differentiated from hematoma caused by excessive anticoagulation therapy in these children.
URI
http://ir.ymlib.yonsei.ac.kr/handle/22282913/161712
Appears in Collections:
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Rehabilitation Medicine (재활의학교실)
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실)
Yonsei Authors
나동욱(Rha, Dong Wook)
박은숙(Park, Eun Sook)
박천웅(Park, Chunung)
이영목(Lee, Young Mock)
최자영(Choi, Ja Young)
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