新生儿一过性重症肌无力1例并文献复习
Transient neonatal myasthenia gravis:a case report and literature review
侯丽 1尹琳琳 1蒋林 1赵婧1
作者信息
- 1. 川北医学院附属医院新生儿科,南充 637000
- 折叠
摘要
目的 总结新生儿一过性重症肌无力(transient neonatal myasthenia gravis,TNMG)的临床特点.方法 回顾性分析川北医学院附属医院新生儿科收治的1例TNMG患儿的临床资料.以"新生儿"、"怀孕"或"妊娠"或"孕产妇"、"肌无力"或"重症肌无力"、"一过性重症肌无力"或"暂时性重症肌无力"或"先天性重症肌无力"、"neonatal"、"pregnancy"OR"pregnant"OR"maternal"、"myasthenia"OR"myasthenia gravis"、"transient myasthenia gravis"OR"transitory myasthenia gravis"OR"temporary myasthenia gravis"、"congenital myasthenia gravis"OR"geneogenous myasthenia gravis"为关键词,检索中国知网、维普数据库、万方数据库、中华医学期刊全文数据库、中国生物医学文献数据库、PubMed、Embase、Web of Science、the Cochrane library、Medline,检索时间自建库至2023 年5 月 31 日,总结已报道的TNMG患儿的临床特点、治疗及预后.结果 本例患儿为足月男婴,母亲诊断重症肌无力(myasthenia gravis,MG)13年,患儿生后10 min出现肌张力减低、哭声弱、呼吸困难,生后2 d新斯的明试验阳性,诊断TNMG,予呼吸支持、静注人免疫球蛋白等治疗,生后10 d症状缓解,生后20 d治愈出院,随访至6月龄未见异常.共检索到符合条件的中文文献7篇,英文文献13篇,包括本例共39例.母亲产前确诊MG 34例(87.2％),产后确诊MG5例(12.8％).主要症状为肌张力减低29例(74.4％)、哭声弱23例(59.0％)、呼吸困难20例(51.3％)、喂养困难18例(46.2％),33例(84.6％)于出生后1d内出现症状,6例(15.4％)于生后2～4d出现症状,29例(74.4％)病程2～6周.27例(69.2％)行新斯的明试验,结果均阳性.30例(76.9％)应用胆碱酯酶抑制剂治疗,5例(12.8％)应用人免疫球蛋白,1例(2.6％)行换血治疗.39例中治愈出院33例(84.6％),死亡6例(15.4％),死因为肺部感染、呼吸衰竭.有随访记录者17例,除1例仅随防至2月龄、竖头稍差外,其余患儿体格生长和神经系统发育均正常.结论 MG母亲分娩的新生儿可能发生TNMG,TNMG患儿大多于生后1 d内出现症状,少数于生后3～4 d出现症状,有症状者需行新斯的明试验、血清MG抗体检测协助诊断,治疗上以胆碱酯酶抑制剂和对症支持为主,经积极治疗多数患儿预后良好.
Abstract
Objective To summarize the clinical features of transient neonatal myasthenia gravis(TNMG).Methods We retrospectively collected the clinical data of an neonate diagnosed with TNMG in the department of Neonatology at the Affiliated Hospital of North Sichuan Medical College.Literature was retrieved from databases including CNKI,VIP,Wanfang,the Chinese Medical Journal Full Text database,the Chinese biomedical literature database,PubMed,Embase,Web of Science,The Cochrane library,and Medline.Search terms were"neonatal"、"pregnancy"OR"pregnant"OR"maternal"、"myasthenia"OR"myasthenia gravis"、"transient myasthenia gravis"OR"transitory myasthenia gravis"OR"temporary myasthenia gravis"、"congenital myasthenia gravis"OR"geneogenous myasthenia gravis".Searching period was from establishment of database to May 31st 2023.We summarize the clinical characteristics,treatment,and prognosis of reported cases with TNMG.Results This patient is a full-term male infant who experienced decreased muscle tone,weak crying,and difficulty breathing 10 min after birth.His mother had a history of myasthenia gravis for 13 years.On the second day of birth,his neostigmine test was positive.So he was diagnosed with TNMG and treated with respiratory support and intravenous human immunoglobulin.His symptoms were relieved at day 10 and resolved by day 20.No abnormalities were observed during follow-up up to 6 months of age.A total of 7 Chinese papers and 13 English papers were retrieved,reporting 39 cases in total including this patient.Mothers of 34 cases(87.2％)were diagnosed with MG before delivery,and the other 5 mothers(12.8％)were diagnosed with MG after delivery.The main clinical manifestations in neonates were reduced muscle tone(29 cases,74.4％),weak crying(23 cases,59.0％),dyspnea(20 cases,51.3％),and feeding difficulties(18 cases,46.2％).33 cases(84.6％)developed symptoms within the first day after birth,6 cases(15.4％)had symptoms between day 2 and day 4.Most patients had a disease course for 2-6 weeks(29 cases,74.4％).27 cases(69.2％)underwent neostigmine test,all were positive.30 cases(76.9％)were treated with cholinesterase inhibitors,5 cases(12.8％)received IVIG and 1 case(2.6％)received an exchange transfusion.Out of the 39 cases,33 cases(84.6％)were cured and discharged,6 cases(15.4％)died.The causes of death were pulmonary infection and respiratory failure.Among the 17 patients with follow-up records,except for 1 case who only followed up to 2 months old and had slightly worse head upright,the rest of the children had normal physical and neurological development.Conclusions TNMG may occur in infants that were born to MG mothers,most of the patients with TNMG develop symptoms on the first day after birth,a few patients develop symptoms 3-4 d after birth.Neostigmine test,serum MG antibody test should be performed to assist diagnosis.Cholinesterase inhibitors and symptomatic support are the main treatment options.TNMG generally have a good prognosis after treatment.
关键词
新生儿/妊娠/重症肌无力/一过性Key words
Neonate/Pregnancy/Myasthenia gravis/Transient引用本文复制引用
基金项目
南充市校合作-国家自然科学基金预研项目(20SXZRKX0003)
川北医学院省级培育科研项目(CBY22-ZDA04)
出版年
2024
NETL
NSTL
万方数据