儿童卡梅现象13例回顾性分析并文献复习
发布时间：2018-07-25 10:02
【摘要】：目的讨论卡梅现象(KMP)的临床表现、诊断与治疗。方法回顾分析2010年1月-2016年1月收治的13例卡梅现象患儿的临床资料,并进行文献复习。结果 13例患儿中男10例、女3例,发病年龄均6个月。12例患儿有出血点、瘀斑、黄疸和皮肤包块等皮肤表现,1例表现为胸腔积液;病灶位置分布多样;血小板严重减少,纤维蛋白原减少,D-二聚体升高;起病至确诊中位时间为60天。采用手术及糖皮质激素、心得安、干扰素、西罗莫司等药物治疗,10例缓解、3例死亡,其中6例给予西罗莫司治疗者疗效均满意。结论卡梅现象以血管肿瘤合并严重血小板减少及消耗性凝血异常为特点,发病年龄小,起病至确诊时间较长;手术治疗疗效确切;西罗莫司或可成为卡梅现象的有效治疗手段。
[Abstract]:Objective to discuss the clinical manifestations, diagnosis and treatment of kame phenomenon (KMP). Methods to review and analyze the clinical data of 13 cases of kame phenomenon in January January 2010 -2016, and review the literature. Results 13 children were 10 males and 3 women, and the age of the onset of.12 had bleeding spots, ecchymosis, jaundice and skin mass. At present, 1 cases were pleural effusion, the location of the lesion was varied, the platelets were severely reduced, the fibrinogen was reduced, the D- two polymer was raised, the onset to the median time of diagnosis was 60 days. The operation and glucocorticoid, propranolol, interferon, sirolimus, 10 cases of remission, and 3 cases of death were used, of which 6 cases were treated with sirolimus. Conclusion kame phenomenon is characterized by vascular tumor associated with severe thrombocytopenia and depleting coagulopathy. The onset age is small, the onset of the disease is longer, and the operative treatment is effective. Cicrolimus may be an effective treatment for the phenomenon of Kali.
【作者单位】： 上海交通大学医学院附属上海儿童医学中心血液肿瘤科;
【分类号】：R732.2
本文编号：2143453
[Abstract]:Objective to discuss the clinical manifestations, diagnosis and treatment of kame phenomenon (KMP). Methods to review and analyze the clinical data of 13 cases of kame phenomenon in January January 2010 -2016, and review the literature. Results 13 children were 10 males and 3 women, and the age of the onset of.12 had bleeding spots, ecchymosis, jaundice and skin mass. At present, 1 cases were pleural effusion, the location of the lesion was varied, the platelets were severely reduced, the fibrinogen was reduced, the D- two polymer was raised, the onset to the median time of diagnosis was 60 days. The operation and glucocorticoid, propranolol, interferon, sirolimus, 10 cases of remission, and 3 cases of death were used, of which 6 cases were treated with sirolimus. Conclusion kame phenomenon is characterized by vascular tumor associated with severe thrombocytopenia and depleting coagulopathy. The onset age is small, the onset of the disease is longer, and the operative treatment is effective. Cicrolimus may be an effective treatment for the phenomenon of Kali.
【作者单位】： 上海交通大学医学院附属上海儿童医学中心血液肿瘤科;
【分类号】：R732.2
【相似文献】
相关会议论文 前1条
1 毛建华;傅海东;方澄清;陈一芳;沈辉君;钱古柃;刘爱民;;西罗莫司治疗儿童难治性肾病综合征的初步体会:2例报道[A];2011年浙江省中医药学会肾病分会学术年会暨国家级中医药继续教育项目“慢性肾脏病临床实践与新进展”学习班暨慢性肾脏病中医临床路径学术研讨会文集[C];2011年
相关硕士学位论文 前1条
1 白涛敏;缺氧诱导因子-1α在儿童非霍奇金淋巴瘤组织中的表达及西罗莫司对Raji细胞增殖、凋亡影响的研究[D];河北医科大学;2009年
，本文编号：2143453
本文链接：https://www.wllwen.com/yixuelunwen/eklw/2143453.html
最近更新
教材专著
