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儿童严重皮肤不良反应(SCARs)的病因发病机制:三级儿童保健医院 9 年经验。

Aetiopathogenesis of severe cutaneous adverse reactions (SCARs) in children: A 9-year experience in a tertiary care paediatric hospital setting.

机构信息

Allergy Unit, Department of Paediatrics, Anna Meyer Children's University Hospital, Florence, Italy.

Department of Clinical and Experimental Medicine, Unit of Internal Medicine, University of Florence, Florence, Italy.

出版信息

Clin Exp Allergy. 2020 Jan;50(1):61-73. doi: 10.1111/cea.13513. Epub 2019 Oct 28.

Abstract

BACKGROUND

Severe cutaneous adverse reactions (SCARs) are delayed-type hypersensitivity reactions to drugs including as follows: Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS), Stevens-Johnson syndrome (SJS), Toxic Epidermal Necrolysis (TEN) and Acute Generalized Exanthematous Pustulosis (AGEP). Incidence, triggers and management of SCARs have not been investigated in large-scale epidemiological studies on children.

OBJECTIVE

The aim of our study was to collect epidemiological, clinical and aetiological data from children with SCARs referred to our tertiary care paediatric hospital of Florence.

METHODS

From 2010 to 2018 charts of children with diagnosis of SCAR were reviewed, and data collected during the acute phase and/or the subsequent allergy evaluation. Patients underwent patch tests, intradermal tests and lymphocyte transformation tests. All children were investigated for infectious diseases.

RESULTS

Incidence of SCARs in hospitalized children was 0.32% over a 9-year period. Fifty-four children were enrolled (31 M; 23 F; median age 6.5 years): 17 cases of DRESS, 30 SJS, 3 TEN, 2 AGEP, 1 linear immunoglobulin A bullous disease (LABD) and 1 pemphigus. Twenty-eight out of 54 patients underwent drug allergy investigations, and 50% of them resulted positive. Combining clinical history and results of allergy work-up, 74% SCARs seem to be caused by drugs, 18.6% by both drugs and infections, 3.7% by infections, and 3.7% remained idiopathic. No deaths occurred.

CONCLUSIONS

In this study, SCARs incidence is in line with literature data. Drugs were most commonly the leading cause. Management of SCARs requires cooperation among professional figures for an early diagnosis and a prompt treatment. Mortality rate seems to be lower in children.

摘要

背景

严重皮肤不良反应(SCARs)是药物引起的迟发型超敏反应,包括药物反应伴嗜酸性粒细胞增多和全身症状(DRESS)、史蒂文斯-约翰逊综合征(SJS)、中毒性表皮坏死松解症(TEN)和急性泛发性发疹性脓疱病(AGEP)。在针对儿童的大规模流行病学研究中,尚未调查 SCAR 的发病率、诱因和治疗方法。

目的

本研究的目的是从我们佛罗伦萨的三级儿科医院就诊的儿童 SCAR 患者中收集流行病学、临床和病因数据。

方法

回顾 2010 年至 2018 年诊断为 SCAR 的儿童病历,并在急性期和/或随后的过敏评估期间收集数据。患者接受了斑贴试验、皮内试验和淋巴细胞转化试验。所有儿童均接受了传染病检查。

结果

9 年间,住院儿童 SCAR 的发病率为 0.32%。共纳入 54 例患儿(31 例男性;23 例女性;中位年龄 6.5 岁):17 例 DRESS、30 例 SJS、3 例 TEN、2 例 AGEP、1 例线性免疫球蛋白 A 大疱性皮病(LABD)和 1 例天疱疮。54 例患者中有 28 例进行了药物过敏检查,其中 50%呈阳性。结合临床病史和过敏检查结果,74%的 SCAR 似乎是由药物引起的,18.6%是药物和感染共同引起的,3.7%是由感染引起的,3.7%仍然是特发性的。无死亡病例发生。

结论

在这项研究中,SCAR 的发病率与文献数据相符。药物是最常见的主要原因。SCAR 的治疗需要专业人员的合作,以便早期诊断和及时治疗。儿童的死亡率似乎较低。

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