Mesenteric Venous Thrombosis in a Child With Type 2 Protein S Deficiency
Mesenteric Venous Thrombosis in a Child With Type 2 Protein S Deficiency
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DOI：
10.1097/mph.0b013e3181fce4d4
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发表时间：
2011-03-01
影响因子：
1.2
通讯作者：
Momoi, Mariko Y.
中科院分区：
文献类型：
--
作者：
Hayakawa, Takahiro;Morimoto, Akira;Momoi, Mariko Y.
一名五岁女童感染甲型流感两星期后出现腹痛及便血。增强计算机断层扫描显示广泛的内脏静脉血栓形成和小肠坏死。坏死的肠切除后，她恢复了健康。患者继续接受抗凝治疗。完全溶解后的血栓性疾病筛查一致显示蛋白S （PS）活性轻度降低，PS抗原水平正常。序列分析发现PROS1基因存在K196E杂合突变。诊断为2型PS缺乏。这是第一个报告肠系膜静脉血栓形成的儿童与2型PS缺乏症。
A 5-year-old girl presented with abdominal pain and bloody stools 2 weeks after suffering from influenza A infection. Enhanced computed tomographic scan showed widespread splanchnic venous thrombosis and small intestine necrosis. She recovered after the necrotic bowel was resected. The patient continues to receive anticoagulant therapy. Thrombophilia screening after the complete resolution consistently showed mildly decreased protein S (PS) activity with normal PS antigen levels. Sequence analysis detected a heterozygous K196E mutation in the PROS1 gene. Type 2 PS deficiency was diagnosed. This is the first report of mesenteric vein thrombosis in a child with a type 2 PS deficiency.