Clinical analysis of amplitude integration electroencephalography of neonatal genetic metabolic diseases
Objective Summarize the clinical characteristics and prognosis of different degrees of amplitude inte-grated electroencephalography(aEEG)in inborn errors of metabolism(IEM).Methods Retrospective analysis was made on the perinatal data,clinical characteristics of different degrees of amplitude integrated electoencephalography,cranial MRI findings and follow-up outcomes of children diagnosed with IEM and monitored by aEEG in the Neonatology Department of Hunan Children's Hospital from January 2018 to December 2021.Results A total of 31 cases were included that met the di-agnostic criteria for IEM,with a total of 15 types of IEM diagnosed.There were 23 cases with severe abnormalities,7 cases with mild abnormalities,and 1 case with normal aEEG results;aEEG in severe children is mostly found in amino acid me-tabolism disorders(4/4100％),urea cycle disorders(5/5100％),organic acid metabolism(10/12,83.3％),most of which have clinical manifestations of abnormal muscular tone,convulsion,respiratory failure,no other malformations,abnormal increase in blood ammonia,skull MRI abnormalities,and high mortality in severe aEEG,and statistical analysis using SPSS 26.0 soft-ware has statistical significance(P＜0.05).Conclusion IEM is a kind of disease with the clinical manifestations of severe ab-normality of aEEG,often accompanied by convulsion,respiratory failure,and hyperammonemia.Patients with severe abnor-malities in aEEG have a poor prognosis and a high mortality rate.
inborn errors of metabolismamplitude integrated electroencephalographynewborn
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