Shu Ye,Huang Wenya,Luo Yongqi,et al.Summary of clinical features and treatment protocols of 10 infants with Kasabach-Merritt syndrome[J].Journal of Clinical Pediatric Surgery,2019,18(09):775-778.[doi:10.3969/j.issn.1671-6353.2019.09.014]
10例婴幼儿卡梅综合征临床特征及治疗经验总结
- Title:
- Summary of clinical features and treatment protocols of 10 infants with Kasabach-Merritt syndrome
- 关键词:
- 卡梅综合征; 血管瘤; 病理状态、体征和症状; 治疗; 婴儿
- Keywords:
- Kasabach-Merritt Syndrome; Hemangioma; Pathological Conditions; Signs and Symptoms; Therapy; Infant
- 分类号:
- R726;R732.2
- 摘要:
- 目的 总结婴幼儿卡梅综合征的临床治疗经验。方法 回顾性分析湖南省儿童医院皮肤科2014年1月至2018年9月收治的10例卡梅综合征住院患儿的临床资料，并对患儿的治疗经过和治疗结局进行总结归纳。结果 10例均于出生后1年内发病（包括2例新生儿），其中男5例，女5例。血管瘤分布情况：头面部6例，躯干2例，臀部、外阴2例。4例患儿经过甲泼尼龙治疗后，2例好转，2例未愈；4例患儿采用甲泼尼龙+平阳霉素栓塞治疗的患儿病情均有所好转；2例患儿采用甲泼尼龙+长春新碱治疗，病情均有所好转。结论 卡梅综合征患儿发病年龄普遍偏小，病情凶险。药物治疗首选糖皮质激素，部分出现激素抵抗的患儿可采用激素联合长春新碱或平阳霉素栓塞治疗。
- Abstract:
- Objective To summarize the clinical treatments of infantile Kasabach-Merritt syndrome.Methods The clinical data were retrospectively analyzed for 10 hospitalized children with Kamei syndrome from January 2014 to September 2018.The treatment and treatment outcomes were summarized.Results All 10 cases fulfilled the diagnostic criteria of Kamei syndrome and occurred within 1 year post-birth,including 2 newborns.There were 5 boys and 5 girls.Hemangioma was located at head & face (n=6),trunk (n=2) and buttocks & vulva (n=2).In 4 cases,2 cases improved after treating with methylprednisolone while 2 cases were unhealed; 4 cases were treated with methylprednisolone and bleomycin embolization; 2 cases received methylprednisolone and vincristine.Conclusion With a young age of onset,Kasabach-Merritt syndrome has an aggressive course.Glucocorticoids are preferred for drug therapy and some patients with steroid resistance may be treated with steroid combined vincristine or bleomycin embolizayim.
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备注/Memo
收稿日期:2018-10-19。
基金项目:湖南省医药卫生科研计划项目（编号：B2016039）
通讯作者:汤建萍,Email:jpingtang@126.com