OROFACIAL-DIGITIAL SYNDROME; REPORT OF A CASE.
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In this case report a patient with multiple duplications in the cervical spine, skull-base and oral cavity is described and a discussion on the possible origin of the duplications is presented.
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Much debate has taken place over the relative importance of heredity and environment in the development of various diseases and conditions. Dental problems are not often easily categorised into either genetic or environmental, because most anomalies involve the interaction of both factors. Since many genetic disorders involve the orofacial region, the importance of the subject is paramount in dentistry to enable a proper understanding of the development of the various conditions and the treatment of patients presenting with those conditions. The incidence of newborn children with any congenital anomaly is estimated to be as high as 2 to 3 per cent, with more than half of these abnormalities, including minor manifestations, found in the head and neck region. It is therefore not unusual for dentists to be the first to recognise such conditions in their patients and be responsible for providing the necessary treatment. This paper describes many conditions, genetically or partly genetically mediated, which affect the oro-facial tissues, and offers descriptions by which the clinician can start to identify those conditions.
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This review of the literature on Down syndrome focuses on various systemic anomalies and oral anomalies, its clinical manifestations, and recommendations for persons with Down syndrome. From the time Down syndrome was diagnosed by phenotype to the present when karyotyping distinguishes chromosomal subgroups, increasingly sophisticated tests and treatments have influenced the lives of Down syndrome patients. Medical advances, special educational programs, and increasing social acceptance of disabled people in the community have resulted in current trends of normalization and deinstitutionalization of these patients. Once a dentist is familiar with a patient's medical history and takes needed precautions, these patients can be treated routinely in a dental office.
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