CAJ | 학술논문

目的探讨经导管动脉硬化栓塞术联合长春新碱治疗激素抵抗型婴幼儿Kasabach-Merritt综合征(KMS)的疗效及其安全性.方法回顾性分析2007年6月至2013年6月接受治疗的17例婴幼儿KMS临床资料.17例患儿中,病灶位于头颈部5例、躯干7例、四肢5例,血小板均低于100× 109/L,最少为3×109/L.17例均表现为激素抵抗型,均接受经导管动脉硬化栓塞术,即将导管插入病灶供血动脉,注入平阳霉素+地塞米松+超液化碘油形成的乳剂,最后用聚乙烯醇颗粒(PVA)或无水乙醇栓塞供血动脉,同时给予长春新碱灌注化疗,术后每周再给予1次长春新碱静脉化疗.4周后评估患儿血小板及瘤体变化情况,以此作为一个治疗周期,若仍未达治愈标准可重复进行上述治疗方案.术后随访6个月至2年,观察治疗情况.结果 17例患儿共接受经导管动脉硬化栓塞术治疗1～3次,共36次,技术操作成功率100％.接受长春新碱化疗5～15次,共180次.术后5例瘤体局部出现水疱、溃烂,给予对症处理1～2周后可好转,1例出现瘤体明显坏死,及时给予抗感染、消毒换药后恢复良好.治愈14例、好转2例、死亡1例.17例患儿均无骨髓抑制、神经毒性及心、脑、肺严重并发症.结论经导管动脉硬化栓塞术联合长春新碱治疗婴幼儿Kasabach-Merritt综合征是一种安全、有效的实用方法,值得在临床推广应用.
목적 탐토경도관동맥경화전새술연합장춘신감치료격소저항형영유인Kasabach-Merritt종합정(KMS)적료효급기안전성.방법 회고성분석2007년6월지2013년6월접수치료적17례영유인KMS림상자료.17례환인중,병조위우두경부5례、구간7례、사지5례,혈소판균저우100× 109/L,최소위3×109/L.17례균표현위격소저항형,균접수경도관동맥경화전새술,즉장도관삽입병조공혈동맥,주입평양매소+지새미송+초액화전유형성적유제,최후용취을희순과립(PVA)혹무수을순전새공혈동맥,동시급여장춘신감관주화료,술후매주재급여1차장춘신감정맥화료.4주후평고환인혈소판급류체변화정황,이차작위일개치료주기,약잉미체치유표준가중복진행상술치료방안.술후수방6개월지2년,관찰치료정황.결과 17례환인공접수경도관동맥경화전새술치료1～3차,공36차,기술조작성공솔100％.접수장춘신감화료5～15차,공180차.술후5례류체국부출현수포、궤란,급여대증처리1～2주후가호전,1례출현류체명현배사,급시급여항감염、소독환약후회복량호.치유14례、호전2례、사망1례.17례환인균무골수억제、신경독성급심、뇌、폐엄중병발증.결론 경도관동맥경화전새술연합장춘신감치료영유인Kasabach-Merritt종합정시일충안전、유효적실용방법,치득재림상추엄응용.
Objective To evaluate the therapeutic effect and safety of transcather arterial scleroembolization(TASE) and vincristine for steroid-resistant Kasabach-Merritt in infants.Methods The clinical data of 17 infants with steroid-resistant Kasabach-Merritt syndrome treated between June 2007 and June 2013 in our hospital were reviewed retrospectively.Among the 17 cases,5 were located in the head and neck region,7 were located in the trunk and 5 were located in the limbs.The platelet count was all lower than 100× 109/L and the lowest was 3×109/L.After the diagnosis was made,transcather arterial scleroembolization (TASE) was performed in all 17 cases.At the time of angiography,pingyangmycin(PYM) and dexamethasone (DXM) dispersed in iodized oil contrast medium were injected into the lesions with a few minutes through the feeding artery followed by polyvinyl(PVA) pieces or absolute ethanol until the main feeding artery was embolized.Vincristine was injected through the feeding artery at the last.Besides,vincristine was used for systemic chemotherapy through intravenous injection once weekly after TASE.Four weeks after TASE,treatment effects were observed.It was necessary to perform TASE and vincristine therapy once again if the patient was not cured well.All cases were followed-up from 6 months to 2 years.Results Thirty-six courses of TASE+VCR treatment were performed in 17 cases.The success rate of operation was 100％.Fourteen patients were cured,2 improved and 1 died.The common complications were local blister or ulccr in five cases and hemangioma necrosis in one case.There was no bone marrow suppression,neurotoxicithy and cardiovascular and pulmonary complications.Conclusions The therapy of transcather arterial scleroembolization combined with vincristine is a safe,useful and effective method for steroid-resistant Kasabach-Merritt syndrome in infants and it is worthy of clinical application.