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-  2017 

Mounier-Kuhn 综合征三例报告并文献复习

DOI: doi:10.7507/1671-6205.201611013

Keywords: Mounier-Kuhn 综合征, 病因, 诊断, 文献复习

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Abstract:

目的 总结 Mounier-Kuhn 综合征(MKS)的病因和临床特征,提高对 MKS 的认知。 方法 报道四川大学华西医院确诊的 3 例 MKS 患者,并检索 CNKI、维普、Pubmed 数据库,筛出高质量 MKS 文章进行文献复习。 结果 共纳入 214 例 MKS 患者,其中我国目前仅 14 例。其病因不明,病理特征表现为气管、支气管的弹性组织缺如,胸部 CT、纤维支气管镜检可见显著的气管支气管扩大征象。男女比为 8.5︰1,临床表现无特异性,常合并遗传性缺陷疾病。目前尚无根治方法。 结论 MKS 是一种遗传学起源的罕见疾病,与吸烟史无关,可能与弹性组织解离综合征具有共同病因。临床工作中需重视皮肤、软组织方面的体格检查。诊断 MKS 除依据气道管径外,还需结合临床、病理、纤维支气管镜检查

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