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细尾半蝎蜇伤后ADAMTS - 13缺乏症

ADAMTS-13 deficiency following Hemiscorpius lepturus scorpion sting.

作者信息

Valavi Ehsan, Ansari Mohammad Javad Alemzadeh, Hoseini Sudabeh

机构信息

Department of Nephrology, Jundishapur University of Medical Sciences, Ahvaz, Iran.

出版信息

Saudi J Kidney Dis Transpl. 2011 Jul;22(4):792-5.

Abstract

Hemiscorpius lepturus is a lethal scorpion with potentially cytotoxic venom. Various degrees of local and systemic toxicity have been observed after its envenomation ranging from local erythema to disseminated intravascular coagulation, renal failure and severe pulmonary hemorrhage. In this case report, we report on a seven-year-old patient who developed the hemolytic uremic syndrome (HUS) after being stung by the scorpion H. lepturus. This condition is characterized by microangiopathic hemolytic anemia, thrombocytopenia, increased serum levels of lactate dehydrogenase and uremia. We evaluated the causes of HUS and found that the levels of C3, C4, CH50 and H factors were normal, but the activity of Von Willebrand factor cleaving protease was decreased (less than 5% of the normal activity). The patient improved after administering therapy with plasma exchange.

摘要

细尾半蝎是一种具有潜在细胞毒性毒液的致命蝎子。被其蜇伤后会观察到不同程度的局部和全身毒性,从局部红斑到弥散性血管内凝血、肾衰竭和严重肺出血不等。在本病例报告中,我们报道了一名七岁患者,被细尾半蝎蜇伤后出现了溶血尿毒综合征(HUS)。这种病症的特征是微血管病性溶血性贫血、血小板减少、乳酸脱氢酶血清水平升高和尿毒症。我们评估了HUS的病因,发现C3、C4、CH50和H因子水平正常,但血管性血友病因子裂解蛋白酶的活性降低(低于正常活性的5%)。患者在接受血浆置换治疗后病情好转。

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