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Elements of morphology: Standard terminology for the teeth and classifying genetic dental disorders
Author(s) -
DureMolla Muriel,
Fournier Benjamin Philippe,
Manzanares Maria Cristina,
Acevedo Ana Carolina,
Hennekam Raoul C.,
Friedlander Lisa,
BoyLefèvre MarieLaure,
Kerner Stephane,
Toupenay Steve,
Garrec Pascal,
ViFane Brigite,
Felizardo Rufino,
Berteretche MarieViolaine,
Jordan Laurence,
Ferré François,
Clauss François,
Jung Sophie,
Chalendar Myriam,
Troester Sebastien,
Kawczynski Marzena,
Chaloyard Jessica,
Manière Marie Cécile,
Berdal Ariane,
BlochZupan Agnès
Publication year - 2019
Publication title -
american journal of medical genetics part a
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.064
H-Index - 112
eISSN - 1552-4833
pISSN - 1552-4825
DOI - 10.1002/ajmg.a.61316
Subject(s) - supernumerary , terminology , dental anomalies , medicine , dentistry , philosophy , linguistics
Dental anomalies occur frequently in a number of genetic disorders and act as major signs in diagnosing these disorders. We present definitions of the most common dental signs and propose a classification usable as a diagnostic tool by dentists, clinical geneticists, and other health care providers. The definitions are part of the series Elements of Morphology and have been established after careful discussions within an international group of experienced dentists and geneticists. The classification system was elaborated in the French collaborative network “TÊTECOU” and the affiliated O‐Rares reference/competence centers. The classification includes isolated and syndromic disorders with oral and dental anomalies, to which causative genes and main extraoral signs and symptoms are added. A systematic literature analysis yielded 408 entities of which a causal gene has been identified in 79%. We classified dental disorders in eight groups: dental agenesis, supernumerary teeth, dental size and/or shape, enamel, dentin, dental eruption, periodontal and gingival, and tumor‐like anomalies. We aim the classification to act as a shared reference for clinical and epidemiological studies. We welcome critical evaluations of the definitions and classification and will regularly update the classification for newly recognized conditions.