| Short mandible, maxilla and cranial base are common in patients with neurofibromatosis 1. | |
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MedLine Citation:
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PMID: 21410551 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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Heervä E, Peltonen S, Pirttiniemi P, Happonen R-P, Visnapuu V, Peltonen J. Short mandible, maxilla and cranial base are common in patients with neurofibromatosis 1. Eur J Oral Sci 2011; 119: 121-127. © 2011 Eur J Oral Sci Neurofibromatosis type 1 (NF1) is an autosomal-dominant neuro-cutaneous-skeletal syndrome. Neurofibromatosis type 1 is one of the Rasopathies, and at the cellular level NF1 results in a hyperactive Ras pathway. In the current investigation, our aim was to study lateral skull X-rays (cephalograms) to assess NF1-related craniofacial morphology. A total of 85 Finnish patients with NF1, including four patients with plexiform neurofibroma of the 5th cranial nerve, and their age- and gender-matched controls, were enrolled in the study. The results showed that patients with NF1 typically had a short mandible, maxilla, and cranial base compared with healthy controls, irrespective of age, but the results were statistically significant only in adults. The length of the mandible, the maxilla and the cranial base correlated with the height of patients under 19 yr of age, but this correlation was absent in adult patients. Thus, a tall adult patient with NF1 may have short jaws and a short cranial base. In conclusion, the NF1 gene apparently influences the growth of craniofacial bones, thus contributing to the craniofacial morphology in NF1. |
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Authors:
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Eetu Heervä; Sirkku Peltonen; Pertti Pirttiniemi; Risto-Pekka Happonen; Vivian Visnapuu; Juha Peltonen |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: European journal of oral sciences Volume: 119 ISSN: 1600-0722 ISO Abbreviation: Eur. J. Oral Sci. Publication Date: 2011 Apr |
Date Detail:
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Created Date: 2011-03-17 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9504563 Medline TA: Eur J Oral Sci Country: Denmark |
Other Details:
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Languages: eng Pagination: 121-7 Citation Subset: D; IM |
Copyright Information:
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© 2011 Eur J Oral Sci. |
Affiliation:
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Department of Cell Biology and Anatomy, University of Turku, Kiinamyllynkatu, Turku Department of Dermatology, University of Turku and Turku University Hospital, Turku Department of Oral Development and Orthodontics, University of Oulu, Oulu Department of Oral Diseases, Turku University Hospital, Lemminkäisenkatu, Turku, Finland. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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