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目的总结新生儿MN血型不合溶血病的临床特点、诊治经验及发病机理。方法报告我科近年诊治的3例新生儿MN血型不合溶血病,复习我科曾报道的另外2例,并通过中国知网、万方数据库和维普中文期刊数据库,检索国内近24年来报告的所有病例报告,对患儿的临床表现和实验室检查进行总结分析。结果包括本文报道的3例,国内共报道31例新生儿MN血型不合溶血病,MN型25例,M型6例;直接抗人球蛋白试验阳性13例,抗体释放试验阳性17例,患儿血型抗体IgG抗M型阳性28例;母亲血型抗体IgG抗M型阳性30例,IgM抗M阳性16例。31例患儿中6例(19.4%)为第1胎发病,贫血发生率67.7%,其中重度贫血发生率29.0%,重度高胆红素血症发生率22.6%;较多患儿需要换血(29.0%)和输血(45.2%);病死率9.7%。结论新生儿MN血型不合溶血病容易出现黄疸、贫血,可导致患儿急性死亡。光疗、输注丙种球蛋白、白蛋白等治疗效果良好,严重病例需要换血、输血治疗。为了提高MN溶血病的诊断率,应检测孕妇MN血型及抗M抗体效价。
Objective To summarize the clinical features, diagnosis and treatment experience and pathogenesis of neonatal MN with hemolytic incompetence. Methods Three cases of MN hemolytic disease of newborns treated in recent years in our department were reviewed. The other two cases reported in our department were reviewed. All the reports reported in the past 24 years in China were retrieved through CNKI, Wanfang Database and VIP Chinese Periodical Database. Case reports, the clinical manifestations of children and laboratory tests were summarized. The results included 3 cases reported in this paper, 31 cases of MN were reported in China, including 25 cases of MN type and 6 cases of M type. Thirteen cases were positive in direct anti-human globulin test and 17 cases were positive in antibody release test. The blood group antibody IgG anti-M type positive in 28 cases; mother blood group antibody IgG anti-M type positive in 30 cases, IgM anti-M positive in 16 cases. Among 31 cases, 6 cases (19.4%) had the first birth and the incidence of anemia was 67.7%, of which the incidence of severe anemia was 29.0% and the incidence of severe hyperbilirubinemia was 22.6% 29.0%) and transfusion (45.2%); the case fatality rate was 9.7%. Conclusion Neonatal MN blood group incompatible hemolytic disease prone to jaundice, anemia, can lead to acute death in children. Phototherapy, infusion of gamma globulin, albumin and other treatment effect is good, serious cases need to change blood transfusion therapy. In order to improve the diagnostic rate of MN hemolytic disease, pregnant women should be tested MN blood type and anti-M antibody titer.