Dental profile of patients with Gaucher disease

BACKGROUND: This study was conducted to determine whether patients with Gaucher disease had significant dental pathology because of abnormal bone structure, pancytopenia, and coagulation abnormalities. METHODS: Each patient received a complete oral and periodontal examination in addition to a routine hematological evaluation. RESULTS: Gaucher patients had significantly fewer carious lesions than otherwise healthy carriers. Despite prevalence of anemia, there was no increase in gingival disease; despite the high incidence of thrombocytopenia, gingival bleeding was not noted; and despite radiological evidence of bone involvement, there was no greater incidence loss of teeth or clinical tooth mobility. CONCLUSIONS: These data represent the first survey of the oral health of a large cohort of patients with Gaucher disease. It is a pilot study of a unique population and the results of the investigation are indications for further research. Based on our findings, we recommend regular oral examinations with appropriate dental treatment for patients with Gaucher disease as for other individuals. Consultation between the dentist and physician, preferably one with experience with Gaucher disease, should be considered when surgical procedures are planned

Iron Behaving Badly: Inappropriate Iron Chelation as a Major Contributor to the Aetiology of Vascular and Other Progressive Inflammatory and Degenerative Diseases

The production of peroxide and superoxide is an inevitable consequence of aerobic metabolism, and while these particular "reactive oxygen species" (ROSs) can exhibit a number of biological effects, they are not of themselves excessively reactive and thus they are not especially damaging at physiological concentrations. However, their reactions with poorly liganded iron species can lead to the catalytic production of the very reactive and dangerous hydroxyl radical, which is exceptionally damaging, and a major cause of chronic inflammation. We review the considerable and wide-ranging evidence for the involvement of this combination of (su)peroxide and poorly liganded iron in a large number of physiological and indeed pathological processes and inflammatory disorders, especially those involving the progressive degradation of cellular and organismal performance. These diseases share a great many similarities and thus might be considered to have a common cause (i.e. iron-catalysed free radical and especially hydroxyl radical generation). The studies reviewed include those focused on a series of cardiovascular, metabolic and neurological diseases, where iron can be found at the sites of plaques and lesions, as well as studies showing the significance of iron to aging and longevity. The effective chelation of iron by natural or synthetic ligands is thus of major physiological (and potentially therapeutic) importance. As systems properties, we need to recognise that physiological observables have multiple molecular causes, and studying them in isolation leads to inconsistent patterns of apparent causality when it is the simultaneous combination of multiple factors that is responsible. This explains, for instance, the decidedly mixed effects of antioxidants that have been observed, etc...Comment: 159 pages, including 9 Figs and 2184 reference

Special Theme: Project Management in E-Science: Challenges and Opportunities

In this introduction to the special theme: Project Management in e-Science: Challenges and Opportunities, we argue that the role of project management and different forms of leadership and facilitation can influence significantly the nature of cooperation and its outcomes and deserves further research attention. The quality of social interactions such as communication, cooperation, and coordination, have emerged as key factors in developing and deploying e-science infrastructures and applications supporting large-scale and distributed collaborative scientific research. If software is seen to embody the relational web within which it evolves, and if the processes of software design, development and deployment are seen as ongoing transformations of this dynamic web of relationships between technology, people and environment, the role of managers becomes crucial: it is their responsibility to balance and facilitate the dynamics of these relationships

Resident Research and Scholarly Activity in Internal Medicine Residency Training Programs

OBJECTIVES: 1) To describe how internal medicine residency programs fulfill the Accreditation Council for Graduate Medical Education (ACGME) scholarly activity training requirement including the current context of resident scholarly work, and 2) to compare findings between university and nonuniversity programs. DESIGN: Cross-sectional mailed survey. SETTING: ACGME-accredited internal medicine residency programs. PARTICIPANTS: Internal medicine residency program directors. MEASUREMENTS: Data were collected on 1) interpretation of the scholarly activity requirement, 2) support for resident scholarship, 3) scholarly activities of residents, 4) attitudes toward resident research, and 5) program characteristics. University and nonuniversity programs were compared. MAIN RESULTS: The response rate was 78%. Most residents completed a topic review with presentation (median, 100%) to fulfill the requirement. Residents at nonuniversity programs were more likely to complete case reports (median, 40% vs 25%; P =.04) and present at local or regional meetings (median, 25% vs 20%; P =.01), and were just as likely to conduct hypothesis-driven research (median, 20% vs 20%; P =.75) and present nationally (median, 10% vs 5%; P =.10) as residents at university programs. Nonuniversity programs were more likely to report lack of faculty mentors (61% vs 31%; P <.001) and resident interest (55% vs 40%; P =.01) as major barriers to resident scholarship. Programs support resident scholarship through research curricula (47%), funding (46%), and protected time (32%). CONCLUSIONS: Internal medicine residents complete a variety of projects to fulfill the scholarly activity requirement. Nonuniversity programs are doing as much as university programs in meeting the requirement and supporting resident scholarship despite reporting significant barriers