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[静脉注射免疫球蛋白治疗中毒性表皮坏死松解症及史蒂文斯-约翰逊综合征/中毒性表皮坏死松解症重叠综合征。15例病例回顾]

[Use of intravenous immunoglobulin for the treatment of toxic epidermal necrolysis and Stevens-Johnson/toxic epidermal necrolysis overlap syndrome. Review of 15 cases].

作者信息

Molgó Montserrat, Carreño Néstor, Hoyos-Bachiloglu Rodrigo, Andresen Max, González Sergio

机构信息

Dermatología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

出版信息

Rev Med Chil. 2009 Mar;137(3):383-9. Epub 2009 Jun 15.

Abstract

BACKGROUND

Toxic epidemial necrolysis (TEN) is an acute adverse drug reaction, that has an unpredictableprogression and a 30% mortality. The incidence of TEN in the general population is approximately 0.4 to 1.2 cases/million/year. It is characterized pathologically by keratinocyte apoptosis which leads to epidemial detachment. Keratinocyte apoptosis is triggered by activation of the Fas-FasL, pathway and could be prevented by the use of intravenous immunoglobulin (IVIG).

AIM

To report the experience with the use of IVIG in TEN.

MATERIAL AND METHODS

Retrospective study of 15 patients with a diagnosis of Stevens-Johnson/TEN overlap (SJS/TEN) or TEN, that received a total dose of 23 +/- 0.6 mg/kg ofIVIG over aperiod of 3 to 4 days. The infusión was initiated during thefirst 24 hours after diagnosis and was associated with standard care for burn victims. Steroids were avoided if the patient was not in chronic steroidal therapy.

RESULTS

Allpatients responded to IVIG in a lapse of 46.4 +/- 14.2 hours from the beginning of infusión. Eighty percent of patients survived, but one developed acute renal failure due to IVIG, and another became blind due to corneal opacities, a complication of TEN. Those who survived were discharged after a lapse of 19-8 +/- 6.6 days from the beginning ofthe disease.

CONCLUSIONS

Despite the lack of blind, multicentric and randomized triáis, we agree with some international studies that TVIG is beneficial as a treatment for SSJ/NETand TEN .

摘要

背景

中毒性表皮坏死松解症(TEN)是一种急性药物不良反应,其病情发展不可预测,死亡率达30%。普通人群中TEN的发病率约为每年0.4至1.2例/百万。其病理特征为角质形成细胞凋亡,导致表皮剥脱。角质形成细胞凋亡由Fas - FasL途径激活触发,静脉注射免疫球蛋白(IVIG)可预防。

目的

报告使用IVIG治疗TEN的经验。

材料与方法

对15例诊断为史蒂文斯 - 约翰逊综合征/TEN重叠综合征(SJS/TEN)或TEN的患者进行回顾性研究,这些患者在3至4天内接受了总量为23±0.6mg/kg的IVIG。输注在诊断后的头24小时内开始,并与烧伤患者的标准护理相结合。如果患者未接受慢性类固醇治疗,则避免使用类固醇。

结果

所有患者在输注开始后的46.4±14.2小时内对IVIG有反应。80%的患者存活,但1例因IVIG发生急性肾衰竭,另1例因TEN的并发症角膜混浊而失明。存活患者在疾病开始后的19.8±6.6天出院。

结论

尽管缺乏盲法、多中心和随机试验,但我们同意一些国际研究的观点,即IVIG作为SJS/TEN和TEN的治疗方法是有益的。

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