CAJ | 학술논문

目的分析Kasabach-Merritt综合征(KMS)的治疗方法。方法对2005-2009年采用综合序贯疗法治疗的51例KMS患儿的临床和随访资料进行回顾性分析。其中男27例，女24例；年龄2d至2.5岁，中位年龄1.8个月。病变部位：颌面部20例，躯干4例，上肢11例，下肢16例。结果 51例KMS患儿中，3例（右颌面、左前臂、右大腿部各1例）因血管瘤意外破溃行急诊血管瘤切除手术，全部痊愈；余48例首先接受激素联合尿素局部注射治疗，其中激素敏感12例，有效15例，无效21例；对激素无效的21例患儿序贯给予丙种球蛋白治疗，敏感5例，有效7例，无效9例。单纯药物加局部尿素治疗共14例，11例痊愈，3例有效；血小板计数4～7 d恢复到正常的4例，8～14 d恢复的5例，15～28 d恢复的2例，超过28 d恢复的3例；瘤体6～ 12个月消失的6例，13～24个月消失的8例。对颌面部血管瘤行颈外动脉结扎置管介入治疗，共19例，全部痊愈；血小板计数术后4～7d恢复到正常的13例，8～14 d恢复的6例；瘤体6～ 12个月消失的12例，13～24个月消失的7例。药物治疗反应无效或病情反复的躯干、四肢血管瘤行择期血管瘤切除手术，共15例，痊愈14例，死亡1例。所有血管瘤切除手术中，行病变完全切除者10例，术后1～3d血小板恢复正常；病变大部切除者8例，术后8～ 14 d 血小板恢复正常。结论综合序贯疗法治疗KMS效果确切，治愈率高，无明显不良反应。
목적 분석Kasabach-Merritt종합정(KMS)적치료방법。방법 대2005-2009년채용종합서관요법치료적51례KMS환인적림상화수방자료진행회고성분석。기중남27례，녀24례；년령2d지2.5세，중위년령1.8개월。병변부위：합면부20례，구간4례，상지11례，하지16례。결과 51례KMS환인중，3례（우합면、좌전비、우대퇴부각1례）인혈관류의외파궤행급진혈관류절제수술，전부전유；여48례수선접수격소연합뇨소국부주사치료，기중격소민감12례，유효15례，무효21례；대격소무효적21례환인서관급여병충구단백치료，민감5례，유효7례，무효9례。단순약물가국부뇨소치료공14례，11례전유，3례유효；혈소판계수4～7 d회복도정상적4례，8～14 d회복적5례，15～28 d회복적2례，초과28 d회복적3례；류체6～ 12개월소실적6례，13～24개월소실적8례。대합면부혈관류행경외동맥결찰치관개입치료，공19례，전부전유；혈소판계수술후4～7d회복도정상적13례，8～14 d회복적6례；류체6～ 12개월소실적12례，13～24개월소실적7례。약물치료반응무효혹병정반복적구간、사지혈관류행택기혈관류절제수술，공15례，전유14례，사망1례。소유혈관류절제수술중，행병변완전절제자10례，술후1～3d혈소판회복정상；병변대부절제자8례，술후8～ 14 d 혈소판회복정상。결론 종합서관요법치료KMS효과학절，치유솔고，무명현불량반응。
ObjectiveTo review and summarize the clinical characteristics and therapeutic approaches of Kasabach-Merritt syndrome ( KMS). MethodsThe combined and sequential therapy was used to for 51 KMS infants between 2005 and 2009. And all cases were retrospectively analyzed. There were 27 males and 24 females with a median age of 1. 8 months ( range: 2 days to 2. 5 years).Among them,lesions were located in maxillofacial ( n = 20), truck ( n = 4), upper extremities ( n = 11 ) and lower extremities (n = 16). ResultsAmong them, 47 of 51 patients were cured, 3 cases were effectively treated and only 1 patient died of multiple organ failure post-operation.Of the 51 cases, emergency surgery was given in 3 cases while other 48 cases were initially treated with methylprednisolone. The responses were varied : excellent and rapid improvement ( n = 12 ) ; moderate response ( n = 15 ) and total failure ( n = 21 ).Immunoglobulin was used a second-line therapy for those (n = 21 ) unresponsive to steroid, with sensitive response( n = 5 ), effective response ( n = 7 ) and invalid response ( n = 9 ) respectively.And adjunct carbonyldiamide therapy was combined. Conservative treatment was tried in 14 patients. Then 11 curative cases and 3 effective cases were observed. The thrombocyte count in 4 cases returned to normal within 4 -7days, 5 cases within 8 - 14 days, and 2 cases within 15 -28 days. In the remaining 3 cases, it normalized more than 28 days.The hemangiomas in 6 cases disappeared completely in 6 - 12 months and 8 cases disappeared in 13 - 24 months.19 patients were treated with the ligation of external carotid artery, the insertion of a tube inside the lesion and the injections of carbonyldiamide and methylprednisolone. The platelet counts of 13 cases increased to normal in 4 -7 days of the treatment, and 6 cases in 8 - 14 days.The tumors of 12 cases disappeared within 6 - 12 months, and 7 cases within 13 - 24 months. Combined surgical resection including emergency operation (n = 3 ) and selective operation (n = 15 ) was performed in 18 patients. And all cases except for one were cured. The platelet counts of that the hemangiomas of 10cases were excised surgically completely increased rapidly and reached the normal range in post-operative 1 - 3 days. The counts of 8 cases after subtotal resection increased to normal within 8 - 14 days. Conclusion A stepwise multimodal approach is recommended for the treatment of KMS.With a higher curative rate, it has minimal side effects.