[Electroencephalogram features in children with congenital hyperinsulinism treated according to the international protocol in Russian Federation].

Q4 Medicine
L R Sarakaeva, D V Ryzhkova, L B Mitrofanova, V G Bairov, A A Sukhotskaya, A P Smorodin, E A Eftich, I A Kelmanson, I L Nikitina
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引用次数: 1

Abstract

Background: Congenital hyperinsulinism (CHI) is a rare life-threatening disease characterised by persistent hypoglycaemia as a result of inappropriate insulin secretion, which can lead to irreversible neurological defects in infants.

Aim: To evaluate neurophysiological characteristics of central nervous system in children with congenital hyperinsulinism treated according to the international protocol in Russian Federation.

Materials and methods: Our retrospective, prospective cohort study included 73 patients who received treatment for CHI according to the current international protocol at different departments of the Almazov National Medical Research Centre from 2017 to 2022. All patients underwent a comprehensive examination, including electroencephalography (EEG).

Results: Among 73 patients with CHI, 35% (23) had focal form of the disease, 65% had non-focal form (49% (39) - diffuse form, 16% (11) - atypical form). All patients with focal form of CHI had a recovery as an outcome.Analysing the EEG data we found that paroxysmal activity was recorded in 23 patients (32%), 50 patients did not have paroxysmal activity (68%). Diffuse changes were observed in 47 patients (64%), whereas 26 patients (36%) were absent of it. By constructing Kaplan-Meier curves we found that the alpha rhythm is formed significantly (p=0.026) earlier in patients with a focal form of CHI.

Conclusion: CHI patients treated according to the international guidelines in Russian Federation show rather positive neurological outcome. We established that alpha rhythm earliest formation is associated with focal form of CHI.

Abstract Image

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[俄罗斯联邦按照国际方案治疗先天性高胰岛素血症儿童的脑电图特征]。
背景:先天性高胰岛素血症(CHI)是一种罕见的危及生命的疾病,其特征是胰岛素分泌不当导致持续低血糖,可导致婴儿不可逆转的神经缺陷。目的:评价俄罗斯联邦按照国际方案治疗先天性高胰岛素血症患儿中枢神经系统的神经生理特点。材料和方法:我们的回顾性、前瞻性队列研究纳入了2017年至2022年在Almazov国家医学研究中心不同部门根据现行国际方案接受CHI治疗的73例患者。所有患者均接受全面检查,包括脑电图(EEG)。结果:73例CHI患者中,35%(23例)为局灶型,65%为非局灶型(49%(39例)为弥漫性,16%(11例)为非典型)。所有局灶性CHI患者的预后均为恢复。分析脑电图资料发现23例(32%)患者有发作性活动,50例(68%)患者无发作性活动。47例(64%)患者可见弥漫性改变,26例(36%)患者未见弥漫性改变。通过构建Kaplan-Meier曲线,我们发现局灶性CHI患者的α节律形成明显早(p=0.026)。结论:俄罗斯联邦根据国际指南治疗的CHI患者显示出相当积极的神经预后。我们确定α节律最早形成与局灶型CHI有关。
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来源期刊
Problemy endokrinologii
Problemy endokrinologii Medicine-Endocrinology, Diabetes and Metabolism
CiteScore
1.40
自引率
0.00%
发文量
59
期刊介绍: Since 1955 the “Problems of Endocrinology” (or “Problemy Endocrinologii”) Journal publishes timely articles, balancing both clinical and experimental research, case reports, reviews and lectures on pressing problems of endocrinology. The Journal is aimed to the most topical issues of endocrinology: to chemical structure, biosynthesis and metabolism of hormones, the mechanism of their action at cellular and molecular level; pathogenesis and to clinic of the endocrine diseases, new methods of their diagnostics and treatment. The Journal: features original national and foreign research articles, reflecting world endocrinology development; issues thematic editions on specific areas; publishes chronicle of major international congress sessions and workshops on endocrinology, as well as state-of-the-art guidelines; is intended for scientists, endocrinologists diabetologists and specialists of allied trade, general practitioners, family physicians and pediatrics.
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