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一项针对 2006 年至 2016 年新南威尔士州转诊医院中毒性表皮坏死松解症和史蒂文斯-约翰逊综合征管理的 10 年回顾性队列研究。

A 10-year retrospective cohort study of the management of toxic epidermal necrolysis and Stevens-Johnson syndrome in a New South Wales state referral hospital from 2006 to 2016.

机构信息

Concord Repatriation General Hospital, Sydney Local Health District, Concord, New South Wales, Australia.

Concord Repatriation General Hospital, The Sydney University, Concord Clinical School Concord, Concord, New South Wales, Australia.

出版信息

Int J Dermatol. 2019 Oct;58(10):1141-1147. doi: 10.1111/ijd.14426. Epub 2019 Mar 25.

Abstract

IMPORTANCE

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are life-threatening severe cutaneous drug reactions. There have been no Australian data published since 2007.

OBJECTIVE

To explore whether there is an association between prognosis and the type of systemic immune-modulation treatment administered in a cohort of patients with SJS, SJS-TEN overlap, and TEN at the New South Wales State SJS/TEN referral center - Concord Repatriation General Hospital.

METHODS

This is a retrospective, single center, cohort study of patients admitted with SJS/TEN from January 1, 2006, to December 31, 2016, at Concord Repatriation General Hospital. Data on demographic information, the causative agent, treatment, and final survival outcome were analyzed.

RESULTS

Forty-two patients included: 26 (62%) with TEN, six (14%) with SJS/TEN overlap, and 10 (24%) with SJS. Overall mortality was 19% (n = 8), and seven suffered TEN. The average age of those who died was 60 years. Eighty-one percent of patients were managed within the burn unit. Twenty-nine patients (70%) received IVIG within this group; 13 individuals also received systemic corticosteroids. Seven (17%) were managed with corticosteroid therapy alone. The incidence of death was 0% in the combined IVIG and corticosteroid group.

CONCLUSION

This series of 42 patients contributes valuable information to a serious condition with low global incidence and high mortality. There appears to be an apparent reduced mortality in the group of SJS/TEN patients managed with combined IVIG and corticosteroid. Larger cohorts are required to validate this relationship due to the risk of bias inherent to the retrospective study design and small sample size.

摘要

重要性

中毒性表皮坏死松解症(TEN)和史蒂文斯-约翰逊综合征(SJS)是危及生命的严重皮肤药物反应。自 2007 年以来,澳大利亚尚未公布任何数据。

目的

在新南威尔士州 SJS/TEN 转诊中心-康科德退伍军人综合医院,探讨 SJS、SJS-TEN 重叠和 TEN 患者队列中接受全身性免疫调节治疗的类型与预后之间是否存在关联。

方法

这是一项回顾性、单中心队列研究,纳入了 2006 年 1 月 1 日至 2016 年 12 月 31 日期间在康科德退伍军人综合医院因 SJS/TEN 住院的患者。分析了人口统计学信息、致病药物、治疗方法和最终生存结局。

结果

共纳入 42 例患者:26 例(62%)为 TEN,6 例(14%)为 SJS/TEN 重叠,10 例(24%)为 SJS。总体死亡率为 19%(n=8),其中 7 例死于 TEN。死亡患者的平均年龄为 60 岁。81%的患者在烧伤病房接受治疗。在这一组中,29 例(70%)患者接受了静脉注射免疫球蛋白(IVIG);13 例患者还接受了全身皮质类固醇治疗。7 例(17%)仅接受皮质类固醇治疗。联合 IVIG 和皮质类固醇治疗组的死亡率为 0%。

结论

本系列 42 例患者为发病率低但死亡率高的严重疾病提供了有价值的信息。在接受 IVIG 和皮质类固醇联合治疗的 SJS/TEN 患者中,死亡率似乎明显降低。由于回顾性研究设计和样本量小固有的偏倚风险,需要更大的队列来验证这种关系。

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