急性门静脉血栓形成作为蛋白C缺乏症的首发表现：一例报告
Acute Portal Vein Thrombosis as an Initial Presentation of Protein C Deficiency: A Case Report.
作者信息
Proskuriakova Ekaterina, Jasaraj Ranjit B, Shrestha Dhan B, Reddy Vijay Ketan, Khosla Pam
机构信息
Internal Medicine, Mount Sinai Hospital, Chicago, USA.
Hematology and Oncology, Mount Sinai Hospital, Chicago, USA.
出版信息
Cureus. 2023 Jun 14;15(6):e40407. doi: 10.7759/cureus.40407. eCollection 2023 Jun.
Protein C (PC) is an essential vitamin K-dependent protein that regulates thrombosis and hemostasis in the body. A mutation in the PROC gene on chromosome 2q14.3 results in PC deficiency. The clinical presentation of PC deficiency can vary, ranging from a single vein thrombosis to disseminated intravascular coagulation, purpura fulminans, or even life-threatening complications such as sepsis. Here, we present a case of a 37-year-old female who was found to have acute portal vein thrombosis as an initial presentation of PC deficiency. She presented to the hospital with acute onset of abdominal pain associated with nausea, blood-streaked emesis, and bloody bowel movement.
蛋白C（PC）是一种必需的维生素K依赖蛋白，可调节体内的血栓形成和止血过程。位于2号染色体q14.3区域的PROC基因突变会导致蛋白C缺乏。蛋白C缺乏的临床表现各不相同，从单一静脉血栓形成到弥散性血管内凝血、暴发性紫癜，甚至是败血症等危及生命的并发症。在此，我们报告一例37岁女性病例，该患者最初表现为急性门静脉血栓形成，这是蛋白C缺乏的首发症状。她因突发腹痛伴恶心、呕血带血丝及便血而入院。
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