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婴儿和幼儿的后部可逆性脑病综合征。

Posterior Reversible Encephalopathy Syndrome in infants and young children.

机构信息

Unit of Child Neurology and Psychiatry, Department of Medical and Surgical Sciences (DIMEC), S. Orsola Hospital, University of Bologna, Italy.

IRCCS Istituto Delle Scienze Neurologiche di Bologna, UOC Neuroradiologia, Bologna, Italy.

出版信息

Eur J Paediatr Neurol. 2021 Jan;30:128-133. doi: 10.1016/j.ejpn.2020.10.009. Epub 2020 Oct 28.

Abstract

AIM

The aim of this study was to describe the characteristics of Posterior Reversible Encephalopathy Syndrome (PRES) in infants and young children (<6 years) and to compare them with the older pediatric population affected by PRES.

METHODS

we retrospectively reviewed records of 111 children (0-17 years) diagnosed with PRES from 2000 to 2018 in 6 referral pediatric hospitals in Italy. The clinical, radiological and EEG features, as well as intensive care unit (ICU) admission rate and outcome of children aged <6 years were compared to those of older children (6-17 years). Factors associated with ICU admission in the whole pediatric cohort with PRES were also evaluated.

RESULTS

Twenty-nine patients younger than 6 years (26%) were enrolled with a median age at onset of PRES of 4 years (range: 6 months-5 years). Epileptic seizures were the most frequent presentation at the disease onset (27/29 patients). Status epilepticus (SE) was observed in 21/29 patients: in detail, 11 developed convulsive SE and 10 presented nonconvulsive SE (NCSE). SE was more frequent in children <6 years compared with older children (72% vs 45%) as well as NCSE (35% vs 10%). Seventeen children aged <6 years required ICU admission. Prevalence of ICU admissions was higher within younger population compared to older (59% vs 37%). In the whole study population SE was significantly associated with ICU admission (p = 0.001).

CONCLUSIONS

PRES in children < 6 years differs from older children in clinical presentation suggesting a more severe presentation at younger age.

摘要

目的

本研究旨在描述婴儿和幼儿(<6 岁)中后部可逆性脑病综合征(PRES)的特征,并将其与受 PRES 影响的较大儿科人群进行比较。

方法

我们回顾性分析了 2000 年至 2018 年意大利 6 家转诊儿科医院诊断为 PRES 的 111 例(0-17 岁)儿童的记录。比较了年龄<6 岁儿童的临床、放射学和脑电图特征,以及重症监护病房(ICU)入院率和结局,与年龄较大的儿童(6-17 岁)进行比较。还评估了 PRES 患儿 ICU 入院的相关因素。

结果

共纳入 29 例年龄<6 岁(26%)的患者,PRES 发病中位年龄为 4 岁(范围:6 个月-5 岁)。癫痫发作是发病时最常见的表现(27/29 例患者)。21/29 例患者出现癫痫持续状态(SE):其中 11 例为惊厥性 SE,10 例为非惊厥性 SE(NCSE)。年龄<6 岁的患儿 SE 发生率高于年龄较大的患儿(72%比 45%),NCSE 发生率也较高(35%比 10%)。17 例年龄<6 岁的患儿需要 ICU 入院。较年轻人群 ICU 入院率高于年龄较大的人群(59%比 37%)。在整个研究人群中,SE 与 ICU 入院显著相关(p=0.001)。

结论

6 岁以下儿童 PRES 的临床表现与年龄较大的儿童不同,提示年龄较小的儿童病情更严重。

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