Hereditary paraganglioma-pheochromocytoma syndromes associated with SDHD and RET mutations.

BACKGROUND: Hereditary paraganglioma-pheochromocytoma syndromes (PGL/PCC) are rare tumors arising from neuroendocrine cells. METHODS AND RESULTS: The proband, a 59-year-old white man and his 42-year-old elder son had a medical history of bilateral carotid body PGL and both presented for treatment of abdominal PGLs. His 36-year-old daughter had excision of recurrent malignant carotid body PGL and vertebral metastasis. His 33-year-old youngest son presented for excision of a unilateral carotid body PGL. All 4 members had succinate dehydrogenase subunit D (SDHD) mutations, whereas the proband and youngest son also had concurrent rearranged during transfection (RET) mutation. CONCLUSION: This is the first report of PGL/PCC with SDHD and RET mutations. The role of the RET gene as a modifier remains speculative. Additionally, the family pedigree suggests maternal inheritance of disease from the probands' paternal grandmother. Clinicians should refer PGL/PCC families for mutation analysis as well as being alert to changes in the classification of mutations.

Surgical handover in a tertiary hospital: a working model.

The trend, in the last few years, of shorter working hours for junior doctors has been driven by the need for safer working conditions. This has led to the loss of continuity of care and the introduction of shift work for residents and registrars, resulting in up to three handovers per 24-h period. Many sentinel events occurring in hospitals can be attributed to a breakdown in communication. Clinical handover is important because it not only facilitates continuity in the transfer of patient information between healthcare professionals but also helps identify potential problems that may occur in upcoming shifts. Methods of handover include verbal-only reports, verbal reports with note-taking and the use of printed handouts containing relevant patient information. This paper presents an exposition of a working model for morning surgical handovers in a tertiary teaching hospital that uses a printed handover sheet, is consultant-led and conference-based, and with an educational focus. A survey of resident staff confirmed the morning handover as important in both patient care and education. There was a marked improvement in tertiary trauma survey completion rates after the introduction of the morning handover. It is now an accepted and important part of the working day in the surgical unit of the hospital.

A working model for the extraordinary review of clinical privileges for doctors and dentists in the Australian Capital Territory.

The extraordinary (unplanned) review of clinical privileges is the means by which an organisation can manage specific complaints about individual practitioners' clinical competence that require immediate investigation. To date, the extraordinary review of clinical privileges for doctors and dentists has not been the subject of much research and there is a pressing need for the evaluation and review of how different legislated and non-legislated administrative processes work and what they achieve. Although it seems a fair proposition that comprehensive processes for the evaluation of the clinical competence of doctors and dentists may improve the overall delivery of an organisation's clinical services, in fact, little is known about the relationship between the safety and quality of specific clinical services, procedures and interventions and the efficiency or effectiveness of established methodologies for the routine or the extraordinary review of clinical privileges. The authors present a model of a structured approach to the extraordinary review of clinical privileges within a clinical governance framework in the Australian Capital Territory. The assessment framework uses a primarily qualitative methodology, underpinned by a process of systematic review of clinical competence against the agreed standards of the CanMEDS Physician Competency Framework. The model is a practical, working framework that could be implemented on a hospital-, area health service- or state- and territory-wide basis in any other Australian jurisdiction.

Age-related pseudocapillarization of the human liver.

Age-related changes in liver function are important because they may promote susceptibility to adverse drug reactions, neurotoxicity, atherosclerosis, and other important diseases in older people. Age-related changes in the rat hepatic sinusoidal endothelium, termed pseudocapillarization, have been described recently and these may contribute to hepatic impairment. The present study has examined surgical and post-mortem specimens with immunohistochemistry and transmission electron microscopy to determine whether pseudocapillarization also occurs in older humans. The age of the subject, independent of systemic disease or hepatic pathology in surgical and post-mortem samples of human liver, was associated with increased peri-sinusoidal expression of von Willebrand's factor, collagen I, collagen IV, and staining with Masson's trichrome. Electron microscopy revealed significant age-related thickening of the sinusoidal endothelium (young 165 +/- 17 nm, middle age 222 +/- 11 nm, older 289 +/- 9 nm, p < 0.001) with loss of fenestrations (young 7.7 +/- 0.7 per 10 micro m, middle age 3.6 +/- 0.5 per 10 micro m, older 1.5 +/- 0.4 per 10 micro m, p < 0.001), and age-related deposition of basal lamina and collagen. In conclusion, ageing in humans is associated with morphological changes in the sinusoidal endothelium and space of Disse which are presumptively related to the ageing process and potentially represent an important link between the ageing process and disease susceptibility.