ABSTRACT
Inequalities within dentistry are common and are reflected in wide differences in the levels of oral health and the standard of care available both within and between countries and communities. Furthermore there are patients, particularly those with special treatment needs, who do not have the same access to dental services as the general public. The dental school should aim to recruit students from varied backgrounds into all areas covered by the oral healthcare team and to train students to treat the full spectrum of patients including those with special needs. It is essential, however, that the dental student achieves a high standard of clinical competence and this cannot be gained by treating only those patients with low expectations for care. Balancing these aspects of clinical education is difficult. Research is an important stimulus to better teaching and better clinical care. It is recognized that dental school staff should be active in research, teaching, clinical work and frequently administration. Maintaining a balance between the commitments to clinical care, teaching and research while also taking account of underserved areas in each of these categories is a difficult challenge but one that has to be met to a high degree in a successful, modern dental school.
Subject(s)
Delivery of Health Care , Dental Care , Dental Research , Medically Underserved Area , Schools, Dental , Teaching , Clinical Competence , Curriculum , Dental Care/standards , Dental Care for Disabled , Education, Dental , Health Services Accessibility , Health Services Needs and Demand , Humans , Oral Health , School Admission Criteria , Specialties, Dental/education , Teaching/methodsABSTRACT
Several ectodermal dysplasia syndromes, including Ectrodactyly-Ectodermal dysplasia-Clefting (EEC) and Ankyloblepharon-Ectodermal Dysplasia-Clefting (AEC) syndromes, are known to result from mutations in the p63 gene. We investigated whether Rapp-Hodgkin syndrome (RHS) is also caused by mutations in the p63 gene. We identified a heterozygous de novo germline missense mutation, S545P, in the sterile-alpha-motif (SAM) domain of p63, in a Thai patient affected with RHS. This is the first genetic abnormality to be described in RHS. The amino acid substitution is the most downstream missense mutation in p63 reported thus far. Histological assessment of a skin biopsy from the patient's palm showed hyperkeratosis and keratinocyte cell-cell detachment in the upper layers of the epidermis, along with numerous apoptotic keratinocytes. Collectively, these investigations demonstrate that RHS is also caused by mutations in p63 and that the clinical similarities to AEC syndrome are paralleled by the nature of the inherent mutation.
Subject(s)
Ectodermal Dysplasia/genetics , Genes, Tumor Suppressor , Germ-Line Mutation/genetics , Membrane Proteins , Mutation, Missense/genetics , Phosphoproteins/genetics , Trans-Activators/genetics , Adolescent , Amino Acid Motifs/genetics , Amino Acid Substitution/genetics , Apoptosis , Cell Adhesion , DNA-Binding Proteins , Ectodermal Dysplasia/pathology , Heterozygote , Humans , Keratinocytes/pathology , Keratosis/pathology , Male , Skin/pathology , Syndrome , Transcription Factors , Tumor Suppressor ProteinsABSTRACT
We report a case of a 19-year-old Thai woman who had fibrous dysplasia (FD) with a rare radiographic 'sunray' appearance. The sunray appearance could have been due to either an unusual pattern of calcification within the lesion or a periosteal reaction.
