Solitary median maxillary central incisor, short stature, choanal atresia/midnasal stenosis (SMMCI) syndrome
| Autor: | James O Lucas, Michael J. Aldred, Karen Kan, Agnes Bankier, Roger K. Hall, A.Graeme B. Perks |
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| Rok vydání: | 1998 |
| Předmět: |
Proband
Heart Defects Congenital Male Dentistry Choanal atresia Constriction Pathologic Short stature Choanal Atresia Incisor Intellectual Disability Oral and maxillofacial pathology Holoprosencephaly Nose Diseases medicine Maxilla Humans Maxillary central incisor General Dentistry Growth Disorders Orthodontics business.industry Infant Syndrome medicine.disease Body Height medicine.anatomical_structure Otorhinolaryngology Atresia Surgery Female Oral Surgery medicine.symptom Nasal Obstruction business Forecasting |
| Zdroj: | Oral surgery, oral medicine, oral pathology, oral radiology, and endodontics. 84(6) |
| ISSN: | 1079-2104 |
| Popis: | This article describes a series of 21 consecutive cases, each involving a solitary median maxillary central incisor; the patients were seen in the Department of Dentistry or the Victorian Clinical Genetics Unit, Murdoch Institute, at the Royal Children's Hospital, Melbourne, from 1966 to 1997. The spectrum of anomalies and associated features present in these cases--solitary median maxillary central incisor, choanal atresia, and holoprosencephaly--is described, and the literature related to the features, including genetic studies in these conditions, is reviewed. We relate our findings in these cases to current knowledge of developmental embryology. It is hoped that the findings, together with our interpretation of them, will help to clarify understanding of solitary median maxillary central incisor syndrome. This syndrome was previously considered a simple midline defect of the dental lamina, but it is now recognized as a possible predictor of holoprosencephalies of varying degrees in the proband, in members of the proband's family, and in the family's descendants. |
| Databáze: | OpenAIRE |
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