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| 引用本文： | 夏红萍,何振娟,朱建幸,朱月钮,朱晓东,谢伟,欧阳天祥.��-÷�ۺ���10���ٴ�����[J].中国实用儿科杂志,2011,26(2):125. |
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| 作者姓名： | 夏红萍 何振娟 朱建幸 朱月钮 朱晓东 谢伟 欧阳天祥 |
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| 作者单位： | ???????????????????????a ????? b ????? c ?????????????200092 |
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| 摘 要： | 目的提高对卡-梅综合征(Kasabach-Merritt syndrome,KMS)的临床表现和治疗的认识。方法 2006年1月至2010年6月上海交通大学医学院附属新华医院收治KMS患儿10例,结合临床资料和文献,详细分析该病的病因、临床表现、诊断、治疗及预后。结果 KMS病因不明,病理生理基础是血小板减少和弥漫性血管内凝血(DIC),与巨大血管肿瘤密切相关;肿块的临床表现多样化;病理表现主要为Kaposiform血管内皮瘤和蔓状血管瘤;治疗方案包括纠正DIC和血小板减少,根据具体情况选择外科治疗(手术切除、栓塞等)或内科药物治疗(糖皮质激素、α-干扰素、免疫抑制剂等)去除血管肿瘤。结论婴儿血管瘤尤其是巨大血管瘤伴血小板减少时应警惕KMS的发生,一旦诊断明确,在对症治疗的基础上,根据不同血管瘤的大小、部位,采取不同的去除血管肿瘤治疗方法。
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| 关 键 词： | ??-÷????? ????? ?С????? |
Kasabach-Merritt syndrome: report of ten cases |
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| XIA Hong-ping,HE Zhen-juan,ZHU Jian-xing,ZHU Yue-niu,ZHU Xiao-dong,XIE Wei,OUYANG Tian-xiang. Xin Hua Hospital,Shanghai ,China.Kasabach-Merritt syndrome: report of ten cases[J].Chinese Journal of Practical Pediatrics,2011,26(2):125. |
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| Authors: | XIA Hong-ping HE Zhen-juan ZHU Jian-xing ZHU Yue-niu ZHU Xiao-dong XIE Wei OUYANG Tian-xiang Xin Hua Hospital Shanghai China |
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| Institution: | Xin Hua Hospital??Shanghai 200092??China |
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| Abstract: | Objective To improve comprehension of the clinical manifestation and treatment of Kasabach-Merritt syndrome (KMS). Methods Ten patients with KMS who were admitted to Xinhua Hospital between January 2006 and June 2010 were studied. Their clinical characteristics,laboratory test,therapy as well as response to treatment were analyzed respectively.The related literature was also reviewed. Results The etiology of KMS remained unknown.The histopathology of KMS was thrombocytopenia and disseminated intravascular c... |
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| Keywords: | Kasabach-Merritt syndrome haemangioma thrombocytopenia |
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