Dental manifestation and management of hypophosphatasia.

ALP, alkaline phosphatase ERT, enzyme replacement therapy Early exfoliation HPP, hypophosphatasia Hypomineralization Hypophosphatasia Medical and dental collaboration Primary teeth QOL, quality of life TNSALP, tissue-nonspecific alkaline phosphatase

Journal

The Japanese dental science review
ISSN: 1882-7616
Titre abrégé: Jpn Dent Sci Rev
Pays: Netherlands
ID NLM: 101481371

Informations de publication

Date de publication:
Nov 2022
Historique:
received: 28 02 2022
revised: 22 06 2022
accepted: 26 06 2022
entrez: 11 7 2022
pubmed: 12 7 2022
medline: 12 7 2022
Statut: ppublish

Résumé

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.

Identifiants

pubmed: 35814738
doi: 10.1016/j.jdsr.2022.06.002
pii: S1882-7616(22)00014-X
pmc: PMC9260292
doi:

Types de publication

Journal Article Review

Langues

eng

Pagination

208-216

Informations de copyright

© 2022 The Authors.

Déclaration de conflit d'intérêts

The authors declare no conflicts of interest.

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Auteurs

Rena Okawa (R)

Department of Pediatric Dentistry, Osaka University Graduate School of Dentistry, Osaka, Japan.

Kazuhiko Nakano (K)

Department of Pediatric Dentistry, Osaka University Graduate School of Dentistry, Osaka, Japan.

Classifications MeSH