Dental manifestation and management of hypophosphatasia.

Content Provider	Europe PMC
Author	Okawa, Rena Nakano, Kazuhiko
Copyright Year	2022
Abstract	Hypophosphatasia is an inherited metabolic disorder characterized by defective mineralization of bones and teeth with a wide variety of manifestations, ranging from stillbirth to dental symptoms alone. Recently, the prognosis of severe hypophosphatasia patients has been greatly improved by the introduction of enzyme replacement therapy. The typical dental manifestation is early exfoliation of primary teeth due to disturbed cementum formation, so dentures are recommended to ensure that important oral functions are acquired. Some studies have shown that enzyme replacement therapy improves dental mineralization, resulting in the stabilization of periodontal tissues and better growth of tooth roots. A nationwide Japanese survey revealed the common genetic and dental manifestations of patients with mild hypophosphatasia, which markedly differ from those of the severe forms. There may be many undiagnosed mild patients, so dentists should contribute to the early diagnosis by screening possible cases based on the typical finding of early exfoliation of primary teeth. Early diagnosis is important for patients to receive early intervention in both medical and dental fields. The establishment of fundamental dental therapy to solve the dental problems is still underway and is eagerly anticipated.
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ISSN	18827616
Journal	The Japanese Dental Science Review [Jpn Dent Sci Rev]
Volume Number	58
DOI	10.1016/j.jdsr.2022.06.002
PubMed Central reference number	PMC9260292
PubMed reference number	35814738
e-ISSN	22136851
Language	English
Publisher	Elsevier
Publisher Date	2022-07-02
Access Restriction	Open
Rights License	This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/). © 2022 The Authors
Subject Keyword	HPP, hypophosphatasia TNSALP, tissue-nonspecific alkaline phosphatase ALP, alkaline phosphatase ERT, enzyme replacement therapy QOL, quality of life Hypophosphatasia Primary teeth Early exfoliation Hypomineralization Medical and dental collaboration
Content Type	Text
Resource Type	Article
Subject	Dentistry