Kasabach-Merritt 综合征 , 婴幼儿 , 血管瘤," /> Kasabach-Merritt 综合征 , 婴幼儿 , 血管瘤,"/> Kasabach-Merritt syndrome ,  Infant , Angioma,"/> <span style="line-height:2;font-size:14px;">婴幼儿Kasabach-Merritt综合征8例</span><span style="line-height:2;font-size:14px;">临床研究</span>
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发育医学电子杂志  2018, Vol. 6 Issue (2): 86-90    
  结构畸形   论著 |
婴幼儿Kasabach-Merritt综合征8例临床研究
余莉华 林丹娜 巫一立 王小兰 吴莉 胡秋磊 王斌 杨丽华
余莉华 林丹娜 巫一立 王小兰 吴莉 胡秋磊 王斌 杨丽华(南方医科大学珠江医院 儿科,广东 广州 510280)
Clinical investigation on 8 cases of infants with Kasabach-Merritt syndrome
YU Li-hua, LIN Dan-na, WU Yi-li, WANG Xiao-lan, WU Li, HU Qiu-lei, WANG Bin, YANG Li-hua
(Department of Pediatrics, Zhujiang Hospital of Southern Medical University, Guangdong, Guangzhou 510280, China)
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摘要 目的 探讨婴幼儿 Kasabach-Merritt 综合征(Kasabach-Merritt syndrome,KMS)的临床特征及治疗方法,为临床提供参考。 方法 2013 年 1 月至 2016 年 12 月,南方医科大学珠江医院儿科中心共收治 8 例婴幼儿 KMS 患儿。采用回顾性分析的方法,记录 8 例患儿的病史、临床表现、辅助检查结果、治疗方案及疗效等相关资料,并随访患儿预后。 结果 8 例患儿中,血管瘤分布分别为:颌下 3 例,下肢 2 例,内脏器官 3 例(肝脏、胰腺和肠系膜各 1 例)。6 例患儿经药物综合治疗(糖皮质激素、α-2b 干扰素、雷帕霉素和长春新碱等单药或联合治疗)后,瘤体缩小变软、颜色变淡,其中肠系膜和胰腺血管瘤患儿在药物治疗前分别因危及生命的瘤体内大出血和器官压迫接受缩瘤手术。血小板完全恢复正常的时间为 4 ~ 30 天,凝血功能完全恢复正常的时间为 2 ~ 50 天,瘤体完全消失的时间为 1 ~ 2 年。2 例患儿因经济原因放弃治疗后死亡。存活的 6 例患儿随诊 2 年均治愈,身高、体重和器官功能均良好。 结论 婴幼儿 KMS 病情危重、进展迅速,糖皮质激素、长春新碱和雷帕霉素等联合治疗可快速控制出血症状,改善预后。对于发生危及生命的大出血、巨大血管瘤压迫重要器官的患儿,可考虑手术治疗以快速减少瘤体面积从而缓解出血。
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关键词:  Kasabach-Merritt 综合征 ')" href="#">Kasabach-Merritt 综合征   婴幼儿 ')" href="#"> 婴幼儿   血管瘤')" href="#"> 血管瘤    
Abstract: 

 Objective   To investigate clinical features and therapeutic methods for infants with Kasabach-Merritt syndrome (KMS) .  Methods From January 2013 to December 2016, eight cases of infants with KMS were admitted to the Department of Pediatrics, Zhujiang Hospital of Southern Medical University. The medical history, clinical manifestations, results of laboratory tests, treatment methods and curative effect of eight cases were recorded by retrospective analysis method and the prognosis of the infants were followed up. Results Among the eight cases, the lesion location of angioma was three cases in submandibular, two cases in lower limbs, and three cases in internal organs (one case in liver, one case in pancreas, one case in mesentery). Angioma of six cases  became shrunken and soft,  and the color of the tumor became light after receiving drug therapy, which were glucocorticoid, α-2b interferon, rapamycin and vincristine single or combination therapy. Among them, mesenteric and pancreas angioma patients were treated with operation respectively because of life-threatening hemorrhage and giant hemangioma oppresses. The platelet was fully restored to normal in 4-30 days. The time of fully recovery of blood coagulation function was 2-50 days. The time of tumor completely disappeared was 1-2 years. Two cases due to economic reasons gave up treatment and died. Through two years follow-up, six cases were allcured. And the height, weight and organ function of the six cases were good.  Conclusions  Infants with KMS are in a critical condition and progress rapidly. Glucocorticoid, vincristine and rapamycin combined treatment can quickly control bleeding and improve prognosis. For the infants with critical bleeding and
vital organs oppressed by huge angioma, surgery can quickly reduce tumor size so as to relieve bleeding.

Key words:  Kasabach-Merritt syndrome ')" href="#">Kasabach-Merritt syndrome     Infant    Angioma
收稿日期:  2017-12-01                出版日期:  2018-04-30      发布日期:  2018-05-22      期的出版日期:  2018-04-30
通讯作者:  杨丽华http://www.cnkang.com/yyk/docindex/59191/    E-mail:  dryanglihua@163.com
引用本文:    
余莉华 林丹娜 巫一立 王小兰 吴莉 胡秋磊 王斌 杨丽华. 婴幼儿Kasabach-Merritt综合征8例临床研究[J]. 发育医学电子杂志, 2018, 6(2): 86-90.
YU Li-hua, LIN Dan-na, WU Yi-li, WANG Xiao-lan, WU Li, HU Qiu-lei, WANG Bin, YANG Li-hua. Clinical investigation on 8 cases of infants with Kasabach-Merritt syndrome. Journal of Developmental Medicine(Electronic Version), 2018, 6(2): 86-90.
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婴幼儿肠衰竭的治疗现状与展望
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[2] 刘宇航 文平 朱全伟 等. 低龄低体重先天性心脏病患儿实施经胸微创介入术的临床研究[J]. 发育医学电子杂志, 2016, 4(3): 144-148.
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