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先天性耳前瘘管的遗传与临床特征分析

Inheritance and clinical characteristics of congenital preauricular fistula

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【作者】 王英歌阳菊华张榕林昶许元腾叶胜难

【Author】 WANG Yingge;YANG Juhua;ZHANG Rong;LIN chang;XU Yuanteng;YE Shengnan;Department of Otolaryngology, First Affiliated Hospital of FuJian Medical University;School of Biomedical Engineering, FuJian Medical University;

【机构】 福建医科大学附属第一医院耳鼻咽喉科福建省耳鼻咽喉研究所福建医科大学生物医学工程学院福建省生物医药工程研究中心

【摘要】 目的明确耳前瘘管的遗传方式及临床特征。方法调查我院11年来先天性耳前瘘管就诊138个患者及其家系,分析就诊患者临床特征,对138个家系进行系谱分析。结果 82个家系有家族史,其中垂直传递的47个家系,隔代传递的有8个家系,家族聚集的有27个家系。发病侧别无明显遗传规律。耳前瘘管患者感染好发于30岁以下。结论先天性耳前瘘管患者为常染色体显性遗传,可表现为外显不全;反复感染的耳前瘘管患者需手术彻底切除瘘管组织才能获得痊愈。

【Abstract】 Objective To determine the inheritance and clinical characteristics of congenital preauricular fistula.Methods Inheritance and clinical characteristics of 138 cases of congenital preauricular fistula with familial incidence treated in our hospital in the past 11 years were reviewed and analyzed. Results A family history was revealed in 82 cases,among which 47 showed vertical inheritance, 8 showed an atavistic pattern, and 27 showed familial susceptibility. No hereditary pattern could be determined regarding the affected sides. Infection of preauricular fistula was noticed especially in patients below 30 years of age. Conclusion Congenital preauricular fistula is probably inherited by incomplete autosomal dominance. Patients with recurrent infections may be best managed by complete excision of fistula tissues.

【基金】 福建省科技厅(2013J01301)
  • 【文献出处】 中华耳科学杂志 ,Chinese Journal of Otology , 编辑部邮箱 ,2015年04期
  • 【分类号】R764.7
  • 【被引频次】12
  • 【下载频次】299
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