遗传性牙龈纤维瘤病（附2例报告）

目的通过探讨遗传性牙龈纤维瘤病（HGF）的临床特点及治疗方法,增进对本病的认识,从而提高诊断治疗水平。方法先证者法收集两个HGF家系全部成员资料,观察不同家系及同一家系不同个体的临床表型和发病特点,绘制系谱图,分析可能的遗传方式。对两名先证者采用手术治疗。结果两家系发病患者均符合非综合征型HGF特征。发病患者不同个体间的表现度不同。两家系均符合常染色体显性遗传特征。经随访,手术患者治疗效果良好。结论 HGF遗传方式以常染色体显性遗传为主,且同一家系的不同受累个体其增生程度轻重不一,极具差异,具有高度遗传异质性。手术是治疗该病的有效的方法。

Hereditary gingival fibromatosis: A report of 2 cases

Objective To investigate the clinical features and treatment of herediatry gingival fibromatosis,to promote and enhance our understanding,diagnostic capability and therapeutic level of HGF.Methods Two pedigrees were collected through the identified probands.Clinical manifestations between and within each family were recorded.Two genealogical trees were drafted and possible inheritance modes were analysed.Two identified probands accepted surgery.Results Of the two pedigrees investigated,an autosomal dominant inheritance mode which presented mainly as non-syndromic form was suggested.All patients had typical symbols but were phenotypically distinguishable.Surgery effects were satisfactory comparatively.Conclusion Hereditary gingival fibromatosis in our study is dominantly inherited with variable expressivity.Surgery was the effective treatment method of HGF.