[Advances and emphasis in the surgery of abnormalities].
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Partial trisomy of 19q has been reported in only 13 patients, of which all but one have been due to unbalanced translocations. Only one previous report of a de novo duplication of distal 19q has been described in a fetal chorionic villus sample. There was no description of clinical phenotype in this report. We describe the clinical manifestations and cytogenetic analysis in a child with an inverted duplication of 19q 13.3 to 13.4 confirmed by FISH using a chromosome 19 whole chromosome probe. This case represents the first report of a liveborn with "pure" distal trisomy 19q. Findings defining this uncommon aneusomy are a flat facies, down turned mouth, abnormal ears, and a short neck with redundant skin folds.
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Moebius syndrome is an extremely rare disorder characterized by a lifetime facial paralysis, involving sixth and seventh cranial nerves with malformations of orofacial structures and the limbs. In this case, an 8 year old girl with Moebius syndrome is presented, clinical findings are described and management aspects are discussed. Early dental evaluation and parental counselling as a part of preventive dental regimen can go a long way in providing complete psychosocial rehabilitation for such physically disabled children.
In addition to several other chronic diseases, tobacco use is a primary cause of many oral diseases and adverse oral conditions. For example, tobacco is a risk factor for oral cancer, periodontal disease, and congenital defects in children whose mothers smoke during pregnancy. The epidemic of tobacco use is one of the greatest threats to global health; sadly the future appears worse because of the globalization of marketing. The World Health Organization (WHO) has strengthened the work for effective control of tobacco use. At the World Health Assembly in May 2003 the Member States agreed on a groundbreaking public health treaty to control tobacco supply and consumption. The treaty covers tobacco taxation, smoking prevention and treatment, illicit trade, advertising, sponsorship and promotion, and product regulation. Oral health professionals and dental associations worldwide should consider this platform for their future work for tobacco prevention since in several countries they play an important role in communication with patients and communities. The WHO Oral Health Programme gives priority to tobacco control in many ways through the development of national and community programmes which incorporates oral health and tobacco issues, tobacco prevention through schools, tobacco risk assessment in countries, and design of modern surveillance systems on risk factors and oral health. Systematic evaluation of coordinated efforts should be carried out at country and inter-country levels.
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We report a case of a 17-month-old child affected by Sturge-Weber syndrome who had unusually rapid overgrowth of the left frontal, temporal, orbital, and maxillary regions. CT angiography illustrated osteohypertrophy with periostitis and associated soft tissue hypertrophy directly corresponding to the distribution of the cutaneous port-wine stain. Extended maxillectomy was performed because of rapid growth and clinical debilitation, with surgical pathology revealing juvenile ossifying fibroma.
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