Oral findings in patient with lethal hypophosphatasia treated with enzyme replacement therapy. Issue 3 (December 2017)

• Record Type: Journal Article
• Authors: Okawa, Rena; Kokomoto, Kazuma; Yamamura-Miyazaki, Natsumi; Michigami, Toshimi; Nakano, Kazuhiko
• Is Part Of: Pediatric dental journal. Volume 27:Issue 3(2017)
• Page Start: 153
• Page End: 156
• Publication Date: 2017-12
• Subjects: Hypophosphatasia -- Enzyme replacement therapy -- Primary teeth -- Disturbed cementum formation -- Hypomineralization; Pedodontics -- Periodicals; Pediatric Dentistry -- Abstracts; Pediatric Dentistry -- Periodicals; 617.645005
• DOI: 10.1016/j.pdj.2017.04.002
• Languages: English
• ISSNs: 0917-2394

Abstract

Abstract: Hypophosphatasia (HPP) is a skeletal disorder, with hypocalcification of bone and early exfoliation of primary teeth displayed in affected individuals. We report here a 3-year-old female diagnosed with perinatal HPP who had received enzyme replacement therapy starting from 1 day after birth. Oral and radiographic examinations revealed deep periodontal pockets, severe mobility, and dentinogenesis imperfecta with root formation defects, especially in the mandibular primary second molars, while the tooth germs of permanent teeth except for the first molars were unclear. Dental manifestations of lethal HPP following treatment with enzyme replacement therapy were markedly different from those of mild HPP.