Oral Manifestations of Neurofibromatosis Type 1 — Oak Academic Publishing
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Oral Manifestations of Neurofibromatosis Type 1
Henri Mondor Hospital, Dental Department, Paris Descartes University, Paris, France
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Laboratory of Molecular Oral Pathophysiology, Paris, France
,
Rotschild Hospital, Centre de référence MAFACE, Paris Diderot University, Paris, France
,
Neuroimmunology Laboratory, Department of Symptom Research, Division of Internal Medicine, University of Texas MD Anderson Cancer Center, Houston, USA
,
Henri Mondor Hospital, Dental Department, Paris Descartes University, Paris, France
,
Henri Mondor Hospital, Dental Department, Paris Descartes University, Paris, France
1 Henri Mondor Hospital, Dental Department, Paris Descartes University, Paris, France
2 Laboratory of Molecular Oral Pathophysiology, Paris, France
3 Rotschild Hospital, Centre de référence MAFACE, Paris Diderot University, Paris, France
4 Neuroimmunology Laboratory, Department of Symptom Research, Division of Internal Medicine, University of Texas MD Anderson Cancer Center, Houston, USA
5 Henri Mondor Hospital, Dental Department, Paris Descartes University, Paris, France
6 Henri Mondor Hospital, Dental Department, Paris Descartes University, Paris, France
Neurofibromatosis type 1 (NF-1) is a common genetic disorder with a highly variable phenotype. The disease affects both proliferation and differentiation of cells of neurectodermal origin. The presence of tumors is very common like benign nodular neurofibromas. Tumors with unclear prognosis may be present like plexiform neurofibromas whose prognosis is more uncertain. While many organs exhibit pathologies, most commonly affected are the nervous system, skin, gastrointestinal tract and heart. Oral abnormalities are also very common: 72% of NF-1 patients exhibit pathologies in oral mucosa, gums, maxillary and temporomandibular joints, and teeth. The incidence of NF-1 and its relationship to the prevalence of caries ha ve been discussed in other research es . It is known that poor oral hygiene plays a key role in the development of periodontal disease and caries. Here we review the oral manifestations of neurofibromatosis type 1 that we illustrate by a patient followed in the center of rare diseases of the hospital Henri Mondor, clinical service in which we work.
KeywordsNeurofibromatosis Type 1Genetic DisorderOral TumorJaw AbnormalitiesPeriodontal Disease
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