| Enamel dysplasia with hamartomatous atypical follicular hyperplasia (EDHFH) syndrome: suggested pathogenic mechanisms. | |
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MedLine Citation:
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PMID: 18561809 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The syndrome of enamel dysplasia with hamartomatous atypical follicular hyperplasia (EDHFH) is an unusual syndrome and is unique to black South Africans. Major criteria for the syndrome are enamel dysplasia with generalized amelogenesis imperfecta-like features and atypical hyperplastic dental follicles with microscopic features of central odontogenic fibroma WHO-type (follicle analogue) attached to the crowns of multiple impacted teeth. Minor features of some cases are anterior open-bite malocclusion, supernumerary teeth, pulpal calcification, aberrant roots with hypercementosis, and hypodontia. The pathogenic mechanisms that lead to the development of EDHFH are unknown. We speculate that faulty synthesis of enamel matrix proteins may interfere with enamel formation and play a role in the generalized enamel hypoplasia described in this syndrome. Alterations in inductive signalling by the odontogenic epithelium mediated by enamel matrix proteins may explain the development of the follicle analogues, the root hypercementosis and the presence of dysplastic cementum deposition juxtaposed to odontogenic epithelium in the gingival overgrowth. Thus, alterations in the function of enamel matrix protein function, may be the common denominator responsible for the development of the EDHFH phenotype. |
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Authors:
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L Feller; B Kramer; E J Raubenheimer; J Lemmer |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: SADJ : journal of the South African Dental Association = tydskrif van die Suid-Afrikaanse Tandheelkundige Vereniging Volume: 63 ISSN: 1029-4864 ISO Abbreviation: SADJ Publication Date: 2008 Mar |
Date Detail:
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Created Date: 2008-06-19 Completed Date: 2008-08-18 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9812497 Medline TA: SADJ Country: South Africa |
Other Details:
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Languages: eng Pagination: 102-5 Citation Subset: D |
Affiliation:
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Department of Periodontology and Oral Medicine, School of Dentistry, University of Limpopo, Medunsa Campus, South Africa. lfeller@medunsa.ac.za |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Ameloblasts
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metabolism Dental Enamel Hypoplasia / etiology* Dental Enamel Proteins / biosynthesis*, physiology Dental Pulp Calcification / etiology Dental Sac / pathology* Epithelial Cells / metabolism Hamartoma / etiology* Humans Hypercementosis / etiology Hyperplasia Odontogenesis Signal Transduction South Africa Syndrome Tooth, Impacted / etiology* |
| Chemical | |
Reg. No./Substance:
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0/Dental Enamel Proteins |
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