黄甲综合征二例报道并文献复习  

Yellow nail syndrome:two cases report and literature review

作　　者：季婷 成小亮 杨苗 杨小东[1] Ji Ting;Cheng Xiaoliang;Yang Miao;Yang Xiaodong(Department of Respiratory and Critical Care Medicine,Rare Diseases Center,West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院呼吸与危重症医学科华西医院罕见病中心,成都610041

出　　处：《国际呼吸杂志》2019年第19期1486-1491,共6页International Journal of Respiration

基　　金：国家重点研发项目精准专项精准医疗示范体系建设项目(2017YFC0910004);四川大学华西医院学科卓越发展1·3·5工程项目(ZYJC18003)。

摘　　要：目的为探讨我国黄甲综合征(YNS)临床特点、诊断及治疗现状,提高临床医师对YNS的认识和诊断意识,以减少漏诊和误诊.方法报道2例YNS患者的临床特点及诊疗经过,以"黄甲综合征"为检索词,在CNKI、万方、维普数据库中检索中文文献16篇,文献复习17例中国Y N S患者的临床特点、诊断及治疗现状.结果例1患者有黄甲、咳嗽、支气管扩张、双下肢淋巴水肿,符合YNS三联征诊断,给予抗感染、补充维生素E等对症处理,随访1年,现患者病情稳定.例2患者双下肢水肿,咳嗽、胸闷、乏力,双侧胸腔积液,淋巴管核素显像提示双下肢引流阻塞,符合三联征中的2个症状,给予多次胸腔穿刺抽液治疗,患者病情好转出院.文献复习我国自1976年报道首例YNS患者至今,共17例,其中88%患者有黄甲,82%患者有呼吸系统表现,82%患者有淋巴水肿,三联征同时存在的为71%,25%患者合并鼻窦炎;YNS目前无公认治疗方案,多为对症治疗,近年来有尝试使用奥曲肽治疗.结论YNS为罕见病,发病率很低,临床工作中容易漏诊、误诊,遇到不明原因指甲异常、胸腔积液、支气管扩张、淋巴水肿的患者应将YNS列入鉴别诊断.Objective To describe and highlight the clinical characteristics of yellow nail syndrome ( YNS ).Methods We reported a retrospective analysis of 2 patients with YNS .We searched CNKI ,Wan Fang ,Wei Pu and PubMed databases with the keywords "yellow nail syndrome" in Chinese and English .And the relevant literature ,including 16 articles in Chinese . Results The first case was a middle-aged male with yellow nail (YN),cough ,bronchiectasis and lymphedema .She received anti-infection ,vitamin E supplementation and other treatment .By the end of follow-up of one year ,the patient was stable .Another case was an old man with cough ,pleural effusions ,lymphedema .He received effusion drainage by thoracentesis repeatedly .Seventeen cases were reviewed in Chinese literatures . The percentage of YN , respiratory symptoms , and lymphedema were 88%,82% and 82%,respectively .And 71% had all the three features ,25% of them with sinusitis .There was no recognized treatment about YNS at present .But somatostatin analogs had been tried for YNS patients recently .Conclusions YNS is a very rare disorder .It is easy to be misdiagnosed in clinical work .We suggest patients with unexplained yellow nail ,pleural effusion ,bronchiectasis and lymphedema must be screened for YNS .

关 键 词：黄甲综合征 胸腔积液 支气管扩张症 淋巴水肿 

分 类 号：R561.3[医药卫生—呼吸系统] R758.72[医药卫生—内科学]