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目的:分析总结家族性巨大型牙骨质瘤家系的临床特征。方法 :对2013年发现的一个家族性巨大型牙骨质瘤家系的临床特点进行总结,采集家族中患者病史、临床表现、影像学和组织病理学资料,绘制家系谱,并结合相关文献,总结该家系的临床特点和遗传方式。结果:该家系4代共33人,患者13例,其中男8例,女5例。所有患病者均在11~13岁开始发病,14~16岁进入迅速增长期,18~20岁病变发展趋于停滞。发病时间集中在青春发育期,自限性明显。13例患病者中,8例有下肢长骨骨折病史,均集中出现在13~16岁,大多骨折3~4次,原因多为受到轻微外力。结论:该家族性巨大型牙骨质瘤家系符合常染色体显性遗传特征,根据其临床特征我们把病程分为3个时期:1发病初期;2迅速增长期;3生长停滞期。
Objective: To analyze and summarize the clinical features of pedigree of familial giant tooth tumor. Methods: To summarize the clinical features of a pedigree of familial giant tooth-based oncofetoma found in 2013, collect the family history, clinical manifestations, imaging and histopathology data and draw pedigree, and summarize the relevant literature The pedigree’s clinical features and patterns of inheritance. Results: The family of 4 generations a total of 33 people, 13 patients, including 8 males and 5 females. All patients were onset at 11 to 13 years of age, 14 to 16 years into the rapid growth period, 18 to 20-year-old lesions tend to stagnate. The onset time concentrated in the puberty, self-limiting obvious. Of the 13 patients, 8 had a history of long bone fractures of the lower extremities, all of them concentrated in the age of 13 to 16 years. Most of them fractured 3 to 4 times, mostly due to slight external force. CONCLUSION: The familial giant tooth tumor has a family of autosomal dominant inheritance. According to its clinical characteristics, we divided the course into three stages: 1 early onset; 2 rapid growth; 3 growth arrest.