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Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу

BACKGROUND: 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: AIM: To evaluate neurophysiological characteristi...

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Autores principales: Саракаева, Л. Р., Рыжкова, Д. В., Митрофанова, Л. Б., Баиров, В. Г., Сухоцкая, А. А., Смородин, А. П., Ефтич, Е. А., Кельмансон, И. А., Никитина, И. Л.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Endocrinology Research Centre 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9978872/
https://www.ncbi.nlm.nih.gov/pubmed/36842080
http://dx.doi.org/10.14341/probl13174
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author Саракаева, Л. Р.
Рыжкова, Д. В.
Митрофанова, Л. Б.
Баиров, В. Г.
Сухоцкая, А. А.
Смородин, А. П.
Ефтич, Е. А.
Кельмансон, И. А.
Никитина, И. Л.
author_facet Саракаева, Л. Р.
Рыжкова, Д. В.
Митрофанова, Л. Б.
Баиров, В. Г.
Сухоцкая, А. А.
Смородин, А. П.
Ефтич, Е. А.
Кельмансон, И. А.
Никитина, И. Л.
author_sort Саракаева, Л. Р.
collection PubMed
description BACKGROUND: 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: 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: 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: 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: 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.
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spelling pubmed-99788722023-03-03 Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу Саракаева, Л. Р. Рыжкова, Д. В. Митрофанова, Л. Б. Баиров, В. Г. Сухоцкая, А. А. Смородин, А. П. Ефтич, Е. А. Кельмансон, И. А. Никитина, И. Л. Probl Endokrinol (Mosk) Research Article BACKGROUND: 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: 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: 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: 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: 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. Endocrinology Research Centre 2023-02-25 /pmc/articles/PMC9978872/ /pubmed/36842080 http://dx.doi.org/10.14341/probl13174 Text en Copyright © Endocrinology Research Centre, 2023 https://creativecommons.org/licenses/by/4.0/This work is licensed under a Creative Commons Attribution 4.0 License.
spellingShingle Research Article
Саракаева, Л. Р.
Рыжкова, Д. В.
Митрофанова, Л. Б.
Баиров, В. Г.
Сухоцкая, А. А.
Смородин, А. П.
Ефтич, Е. А.
Кельмансон, И. А.
Никитина, И. Л.
Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу
title Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу
title_full Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу
title_fullStr Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу
title_full_unstemmed Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу
title_short Параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу
title_sort параметры электроэнцефалограммы у детей с врожденным гиперинсулинизмом, пролеченных по международному протоколу
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9978872/
https://www.ncbi.nlm.nih.gov/pubmed/36842080
http://dx.doi.org/10.14341/probl13174
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