Presse Med
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Comparative Study
[Impact of a hospice home visit team on unwanted hospitalization of terminally-ill patients at home in acute medical emergencies].
This study was undertaken to evaluate the impact of paramedical hospice specialists on hospitalization of terminally-ill patients in acute medical emergencies. ⋯ A team of paramedical hospice specialists, acting on request of the SAMU, provides a concrete and useful response to problems of unwanted hospitalization in acute emergencies for home-based terminally ill patients. This type of organization is consistent with respect for the patient's choice to remain at home until the end of life. Prevention of unwanted hospitalization and heroic measures should undoubtedly result in cost savings more than sufficient to fund this program.
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Results of meta-analyses may differ from those of megatrials only because of the bias that could be introduced by a unadequate methodology of some meta-analyses. A meta-analysis must be based on an exhaustive review of the literature - of all studies, those with negative as well as positive results. The quality of a meta-analysis depends on the methodological quality of the studies it analyzes; accordingly they must be selected according to strict methodological inclusion and exclusion criteria, defined a priori. ⋯ Different analytic techniques must be used to assess possible publication or selection bias (the so-called "funnel plot" method) and potential sources of heterogeneity (heterogeneity test, meta-regression, etc.). Meta-analyses of individual data make it possible to assess the treatment effect according to patient characteristics. Prospective meta-analyses or those planned before clinical trials can help to limit data heterogeneity by making study protocols less varied (treatment, follow-up, evaluation, etc.).
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Hypertrophic cardiomyopathies have an estimated prevalence of 1/500. The analysis of the genes coding for the 10 most commonly involved sarcomeric proteins, fails to detect a mutation in about one third of cases. In some of these cases, cardiomyopathy can be attributed to a genetics storage disease with enlarged glycogen vacuolss (PRKAG2 deficiency, Danon disease, Pompe disease) and/or lysosomol vacuoles (Donon disease, Pompe disease, Fabry disease). ⋯ Danon disease is an X-linked dominant inherited disease characterized by cardiomyopathy, squeletal myopathy and mental retardation. Cardiac transplantation is indicated in both affected men and women. In the infantile form of Pompe disease, enzyme replacement therapy with olglucosidase alpha shows efficacy on cardiac failure with a significant regression of ventricular hypertrophy on ECG, echocardiography and radiography
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Fabry disease, rare X-linked disorder with deficient activity of alpha-galactosidase A, leads to a multiple organ failure caused by a progressive accumulation of the substrat globotriasocylceramide in cells. Peripheral nerve involvement, neuropathic pain and chronic acroparesthesiae, are the most frequently reported signs and often revealing the disease. They are secondary to the small nerve fibres (fibres 5), that explained the normality of electroneuromyography. ⋯ Affecting essentially the posterior circulation, their etiologies have to be clarified. MRI shows numerous silent lesions, increasing with age, mainly in small perforant arteries and pulvinar calcifications, due to on increase in cerebral perfusion with an impaired cerebral autoregulation, secondary to the glycosphingolipid storage in vascular endothelial cells. Enzyme replacement therapy could improve cerebral regional blood flow disturbances and painful neuropathy