Extreme oral manifestations in a Marfan-type syndrome

Abstract A 12-year-old girl with an otherwise typical Marfan syndrome (Ghent criteria fulfilled) presented with highly unusual oral manifestations consisting of supernumerary teeth and severe dental crowding. Pathological examination of the supernumerary teeth revealed an elevated number of pulpolit...

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Bibliographic Details
Published in:	International journal of oral and maxillofacial surgery Vol. 39; no. 6; pp. 622 - 625
Main Authors:	Khonsari, R.H, Corre, P, Boukerma-Vernex, Z, Schmidt, J, Renaudin, K, Frayssé, C, Gayet-Delacroix, M, Khau Van Kien, P, David, A
Format:	Journal Article
Language:	English
Published:	Kidlington Elsevier Ltd 01-06-2010 Elsevier
Subjects:	Biological and medical sciences Child dental crowding Dental Pulp Diseases - etiology Dental Pulp Diseases - genetics Dentistry FBN1 Female Fibrillin-1 Fibrillins Humans Loeys–Dietz syndrome Malocclusion - etiology marfan syndrome Marfan Syndrome - complications Medical sciences Microfilament Proteins - genetics Otorhinolaryngology. Stomatology Protein-Serine-Threonine Kinases - genetics pulpoliths Receptors, Transforming Growth Factor beta - genetics Sarcoidosis. Granulomatous diseases of unproved etiology. Connective tissue diseases. Elastic tissue diseases. Vasculitis supernumerary teeth Surgery TGF-beta sub-unit receptors Tooth, Supernumerary - etiology Tooth, Supernumerary - genetics dental crowding pulpoliths marfan syndrome supernumerary teeth TGF-beta sub-unit receptors Loeys–Dietz syndrome FBN1 Connective tissue disease Skin disease Elastic tissue disease Stomatology Supernumerary tooth Marfan syndrome Genetic disease Loeys-Dietz syndrome Treatment Surgery Systemic disease Oral cavity disease Biological receptor
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Summary:	Abstract A 12-year-old girl with an otherwise typical Marfan syndrome (Ghent criteria fulfilled) presented with highly unusual oral manifestations consisting of supernumerary teeth and severe dental crowding. Pathological examination of the supernumerary teeth revealed an elevated number of pulpoliths. No mutation in the FBN1 , TGFBR1 and TGFBR2 genes was identified despite exhaustive screening, suggesting that another gene defect could explain this association of marfanoid features with dental abnormalities.
Bibliography:	ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3
ISSN:	0901-5027 1399-0020
DOI:	10.1016/j.ijom.2010.01.006