中毒性表皮坏死松解症免疫球蛋白治疗临床分析
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摘要
目的:探讨大剂量静脉用免疫球蛋白(IVIG)治疗中毒性表皮坏死松解症(TEN)的临床疗效与安全性。
     资料和方法:对12例应用IVIG治疗的TEN患者的临床资料进行回顾性分析。12例TEN患者均为2000年1月1日~2007年4月30日期间山东省立医院皮肤性病科的住院病人,其中男3例,女9例,年龄(3~64)岁,平均37.7岁,表皮剥脱面积占总体表面积(TBSA)的30%~90%,平均63.7%,同时存在粘膜受累者10例(平均83.3%),给予大剂量人免疫球蛋白(0.1~0.4)g/kg·d[总量为(0.1~2.0)g/kg,平均1.7g/kg]治疗(1~6)天,平均4.8天,观察应用免疫球蛋白治疗后皮肤及粘膜停止松解剥脱时间、表皮完全修复时间及患者最终恢复情况。
     结果:TEN患者住院天数(2~42)天,平均21.3天,住院期间7例患者痊愈(58.3%),3例患者基本痊愈(25%)[其中1例出院后失访(8.3%)],1例好转自动出院(8.3%),1例死亡(8.3%);起用IVIG治疗后,11例存活者的起效时间为(2~7)天,平均4.8天;8例痊愈患者的获痊愈时间为(17~40)天,平均24.3天;出院时,8例患者的粘膜损害痊愈(66.7%);10例患者分别于出院后1、2、4周行门诊复查,无复发病例,1例患者于出院后1周发生睑球粘连,余均无任何TEN后遗症或并发症发生,10例患者于出院后4周时均获痊愈。
     结论:1.早期应用IVIG[总量2g/kg(0.4g/kg·d×5d或者0.5g/kg·d×4d)]配合糖皮质激素治疗TEN安全有效。
     2.IVIG治疗效果受到多方面因素的影响。
     3.临床医师在治疗TEN过程中,应注意预防并及时发现IVIG副作用的发生。
Objective: To evaluate the effect and safety of high dose intravenous immunoglobulin(IVIG) in toxic epidermal necrolysis(TEN).
     Methods: A retrospective analysis of 12 consecutive patients with TEN who were treated with IVIG. Retrospective cohort review of the records of all TEN patients admitted to Shandong Provincial Hospital Dermatology Department between January 1, 2000 and April 30, 2007. Of the 12 patients, 3 male and 9 female, aged from 3 to 64 (mean 37.7 yaers) , with skin detachment≥30% of their totle body surface(TBSA)[mean,63.7%;range,30%~90%] and 10 patients had mucous complications(83.3%). Infusion of IVIG (mean totle dose, 1.7g/kg[range,0.1~2.0g/kg] ) in all patients daily for 1~6 days( mean,4.8 days ) and observe the clinical response and final outcome.
     Results: The mean length of saty(LOS) was 21.3 days( range,2~42days ). 7 patients discharged after complete recovery(58.3%) and 1 patient dischargevoluntarily(8.3%). One patient died and the survival rate come to 91.7%. Infusion of IVIG was associate-d with a rapid cessationa( mean,4.8 days; range,2~7days ) of skin and mucosal detachment in 11 patients . The mean time to complete skin healing was 24.3 days(range, 17~40days ).Only 8 patients achieved complete mucous healing(66.7%) when discharged. Follow-up (1,2,4 weeks after discharging ) in 10 patients showed that 3 more patients obtained complete recovery 4 weeks after discharg- ing and no recurrence happened. 1 patient progressed symblepharon within 1 week after discharging and the others developped no any sequelae or complication of TEN.
     Conclusion: 1. Early treatment with combination of high-dose IVIG at a total dose of 2g/kg(0.4g/kg per day for 5 days or 0.5g/kg per day for 4 days) and glucocorticoids is a safe and effective therapeutic method for TEN.
     2. Patients' responsiveness to IVIG treatment is associated with several parameters.
     3. Any patient treated with IVIG should be adequately monitored for the development of complications.
引文
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