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以色列金蝎蜇伤儿童溶血后获得性ADAMTS13缺乏症的高发生率

High frequency of acquired ADAMTS13 deficiency after hemolysis in Hemiscorpius Lepturus (scorpion) stung children.

作者信息

Valavi Ehsan, Ahmadzadeh Ali, Amoori Parisa, Daneshgar Aidin

机构信息

Division of Pediatric Nephrology, Abuzar Children's Hospital, Ahvaz Jundishapur University of Medical Sciences, Ahvaz, 6163933874, Iran,

出版信息

Indian J Pediatr. 2014 Jul;81(7):665-9. doi: 10.1007/s12098-013-1089-5. Epub 2013 Jul 28.

DOI:10.1007/s12098-013-1089-5
PMID:23893367
Abstract

OBJECTIVE

To estimate the frequency of acquired ADAMTS13 deficiency in severe cases of Hemiscorpius lepturus stung patients and the frequency of acute kidney injury (AKI) in these patients.

METHODS

Sixty scorpion stung children who were referred with severe hemolysis and hemoglobinuria were studied. None of them had received blood products and no one had a past medical history of renal failure.

RESULTS

Plasma levels of ADAMTS13 and ADAMTS13 antibody (IgG) were measured using ELISA. ADAMTS13 was decreased in 91.7 % of patients and the anti-ADAMTS13 antibody (Ab) was increased in 98.3 %. ADAMTS13 decreased in all of the patients with acute kidney injury and none of those with normal levels of ADAMTS13 developed renal failure; all patients with AKI had also increased levels of ADAMTS13Ab. Acute kidney injury was found in 23.3 % and had significant association with severe anemia, thrombocytopenia, pyuria, hematuria and considerable proteinuria (p < 0.001). Disseminated intravascular coagulation (DIC) and Hemolytic uremic syndrome (HUS) developed in 6.7 % and 10 % respectively.

CONCLUSIONS

The index findings demonstrate that Hemiscorpius lepturus sting is usually associated with ADAMTS13 deficiency, and increased ADAMTS13 autoantibody. These combined mechanisms may contribute to scorpion sting-induced coagulopathies and may predispose patients to develop DIC and HUS.

摘要

目的

评估粗尾半蝎蜇伤重症患者获得性ADAMTS13缺乏症的发生率以及这些患者急性肾损伤(AKI)的发生率。

方法

对60例因严重溶血和血红蛋白尿转诊的蝎蜇伤儿童进行研究。他们均未接受过血液制品,且既往均无肾衰竭病史。

结果

采用酶联免疫吸附测定法(ELISA)检测血浆中ADAMTS13和ADAMTS13抗体(IgG)水平。91.7%的患者ADAMTS13水平降低,98.3%的患者抗ADAMTS13抗体升高。所有急性肾损伤患者的ADAMTS13水平均降低,ADAMTS13水平正常的患者均未发生肾衰竭;所有急性肾损伤患者的ADAMTS13抗体水平也升高。23.3%的患者出现急性肾损伤,且与严重贫血、血小板减少、脓尿、血尿和大量蛋白尿显著相关(p<0.001)。分别有6.7%和10%的患者发生弥散性血管内凝血(DIC)和溶血尿毒综合征(HUS)。

结论

本研究结果表明,粗尾半蝎蜇伤通常与ADAMTS13缺乏及ADAMTS13自身抗体升高有关。这些联合机制可能导致蝎蜇伤诱发的凝血病,并可能使患者易发生DIC和HUS。

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