Journal
AMERICAN JOURNAL OF MEDICAL GENETICS PART C-SEMINARS IN MEDICAL GENETICS
Volume 187, Issue 4, Pages 520-526Publisher
WILEY
DOI: 10.1002/ajmg.c.31941
Keywords
Ehlers-Danlos; hypermobility; periodontitis; pulp stones; root anomalies
Categories
Funding
- EDS Society [2019.02]
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Ehlers-Danlos syndromes (EDS) are inherited connective tissue disorders that manifest distinct pathologies in the teeth and oral cavity, affecting oral health-related quality of life. Standardization of diagnostic criteria is essential for resolving remaining questions, with specialized centers needing to apply congruent approaches to characterize clinical features and conduct genetic validation uniformly.
Ehlers-Danlos syndromes (EDS) are a group of inherited connective tissue disorders. Patients with EDS exhibit distinct pathologies of the teeth and the oral cavity. Here, we summarize the current knowledge in the various EDS types, in particular regarding severe changes in oral health-related quality of life, the differential emergence of periodontitis, characteristic yet highly cumbersome dental manifestations, apparent anomalies of oral soft tissues, and relevant issues related to dental implantology. Resolution of remaining open questions will primarily rely on the standardization of diagnostic criteria. Clinical centers that specialize on this rare pathology need to apply congruent approaches for exact characterization of clinical features in conjunction with genetic validation that should be reached without exception in all patients and relevant family members.
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