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Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis

This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia gravis (MG) and address the prognosis through follow-up. The clinical data of 135 children with MG were grouped according to clinical type and therapeutic drugs, retrospectively analyzed an...

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Autores principales: YANG, ZHI-XIAO, XU, KAI-LI, XIONG, HUI
Formato: Online Artículo Texto
Lenguaje:English
Publicado: D.A. Spandidos 2015
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4353784/
https://www.ncbi.nlm.nih.gov/pubmed/25780436
http://dx.doi.org/10.3892/etm.2015.2256
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author YANG, ZHI-XIAO
XU, KAI-LI
XIONG, HUI
author_facet YANG, ZHI-XIAO
XU, KAI-LI
XIONG, HUI
author_sort YANG, ZHI-XIAO
collection PubMed
description This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia gravis (MG) and address the prognosis through follow-up. The clinical data of 135 children with MG were grouped according to clinical type and therapeutic drugs, retrospectively analyzed and prospectively monitored. Of the 135 MG patients, 85.2% had type I (ocular type), with only 4.2% progressing to systemic MG; 13.4% had type II (general type); and 1.5% had type III (fulminating type). Relapse occurred in 46.1% of the 102 patients that were followed up. The positive rate for the primary acetylcholine receptor antibody was 40.19%, without significant differences among clinical subtypes. The positive rate of the repetitive nerve stimulation frequency test by electromyography was 37.97%. Decreased expression of CD4(+), CD8(+), or CD3(+) was present in 71% of the patients. Thymic hyperplasia was present in 5.93% of the patients, while 1.48% had thymoma. Steroid treatment was effective in the majority of the patients. Ocular type MG was common in this cohort of patients. The incidence and mortality of myasthenia crisis were low, the presence of concurrent thymoma was rare and only a limited number of children developed neurological sequelae.
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spelling pubmed-43537842015-03-16 Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis YANG, ZHI-XIAO XU, KAI-LI XIONG, HUI Exp Ther Med Articles This study aimed to analyze the clinical characteristics, classification and treatment of childhood myasthenia gravis (MG) and address the prognosis through follow-up. The clinical data of 135 children with MG were grouped according to clinical type and therapeutic drugs, retrospectively analyzed and prospectively monitored. Of the 135 MG patients, 85.2% had type I (ocular type), with only 4.2% progressing to systemic MG; 13.4% had type II (general type); and 1.5% had type III (fulminating type). Relapse occurred in 46.1% of the 102 patients that were followed up. The positive rate for the primary acetylcholine receptor antibody was 40.19%, without significant differences among clinical subtypes. The positive rate of the repetitive nerve stimulation frequency test by electromyography was 37.97%. Decreased expression of CD4(+), CD8(+), or CD3(+) was present in 71% of the patients. Thymic hyperplasia was present in 5.93% of the patients, while 1.48% had thymoma. Steroid treatment was effective in the majority of the patients. Ocular type MG was common in this cohort of patients. The incidence and mortality of myasthenia crisis were low, the presence of concurrent thymoma was rare and only a limited number of children developed neurological sequelae. D.A. Spandidos 2015-04 2015-02-03 /pmc/articles/PMC4353784/ /pubmed/25780436 http://dx.doi.org/10.3892/etm.2015.2256 Text en Copyright © 2015, Spandidos Publications http://creativecommons.org/licenses/by/3.0 This is an open-access article licensed under a Creative Commons Attribution-NonCommercial 3.0 Unported License. The article may be redistributed, reproduced, and reused for non-commercial purposes, provided the original source is properly cited.
spellingShingle Articles
YANG, ZHI-XIAO
XU, KAI-LI
XIONG, HUI
Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
title Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
title_full Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
title_fullStr Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
title_full_unstemmed Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
title_short Clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
title_sort clinical characteristics and therapeutic evaluation of childhood myasthenia gravis
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4353784/
https://www.ncbi.nlm.nih.gov/pubmed/25780436
http://dx.doi.org/10.3892/etm.2015.2256
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