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囊性纤维症( Cystic fibrosis ,CF)是一种多见于白种人的系统性疾病,其在活产婴儿中的发病率约为1/2500；相关临床资料显示,该病在我国也有一定发病率。 CF是由囊性纤维化跨膜转导调节因子( cystic fibrosis transmembrane conductance regulator,CFTR)基因突变引起的其编码的CFTR蛋白功能缺陷所致,可导致多脏器、多种疾病的发生。有研究发现,CF病人的患龋率比健康人低,更易发生釉质缺损。进一步研究显示,患CF小鼠的切牙釉质矿化较低,表现为白垩色,说明CFTR与切牙的发育异常有关。本文就CF的釉质异常表现及其相关研究作一综述。“,”Cystic fibrosis ( CF) is a systemic disorder commonly seen in Caucasian in approximately one out of 2,500 live births. Clinical data show that there is also a certain incidence of the disease in China. The etiology of CF is that mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR) gene cause dysfunction of CFTR protein. Studies have found a higher prevalence of enamel defects but a lower dental caries incidence in CF pa-tients than in healthy population, and CF mice has chalky white incisor enamel. Further molecular studies have shown that the CFTR gene is associated with abnormal development of incisors. Specific oral manifestations may be risk indi-cators of systemic disorders. This article is committed to review the enamel abnormalities and related research of CF.