非综合征型先天性多数牙缺失1例

摘要

Congenitally teeth absence, including individual, majority and all teeth congenital absence, is one kind of teeth number abnormality. Majority teeth congenital absence is often a manifestation of syndrome or an independent disease, which is rare. The main syndromes accompanied by congenitally teeth absence are Rieger syndrome, Ectodermal dysplasias and EEC syndrome. The etiology is unknown. The environmental factors and genetic factors are usually considered, and the genetic is main. Some studies say the box gene 9 ( PAX9) and the sarcomere homeobox gene 1 (MSX1) is associated with premolars and molar missing, respectively. Majority teeth congenital absence can cause malocclusion. Comprehensive diagnosis is necessary for multi-disciplinary consultations to determine the long-term treatment goals%先天性牙齿缺失是牙齿数目异常的一种,分为个别、多数和全部牙齿先天缺失.多数牙先天缺失常为综合征的表现之一或为一种独立的疾病,较为少见.伴有先天缺牙的综合征以Rieger综合征、外胚叶发育不全、EEC综合征等常见.病因不明,通常认为是环境因素和遗传因素参与,以遗传因素为主.有研究认为,成对盒基因9(PAX9)和肌节同源盒基因1(MSX1)分别与前磨牙、磨牙的缺失有关.多数牙齿先天缺失可造成错(牙合)畸形,治疗时需全面诊断,多学科咨询以利于确定长期治疗的目标.