关键词: Cu-sil dentures Papillon–Lefèvre syndrome palmoplantar keratosis

Mesh : Adolescent Cathepsin C Child, Preschool Dentures Female Humans Infant Papillon-Lefevre Disease

来  源:   DOI:10.4103/JISPPD.JISPPD_256_18

Abstract:
Papillon-Lefèvre syndrome (PLS) is a rare genetic disorder characterized by palmoplantar keratosis and premature loss of primary and permanent dentition. Its onset can be as early as 1-4 years of age. The genetic disorder is mutation in the cathepsin C gene. Hereby, we discuss the fabrication of Cu-sil dentures for the prosthetic rehabilitation of a 14-year-old girl with PLS. The case report describes the procedure and associated relevant information regarding the management protocols.
摘要:
Papillon-Lefèvre综合征(PLS)是一种罕见的遗传性疾病,其特征是掌plant角化病以及原发性和永久性牙列的过早丧失。其发病可早至1-4岁。遗传性疾病是组织蛋白酶C基因的突变。特此,我们讨论了Cu-sil假牙的制造,用于14岁的PLS女孩的假肢康复。病例报告描述了有关管理方案的程序和相关信息。
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