B Acad Nat Med Paris
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As their name implies, orphan diseases are rare, often poorly studied diseases. Patients with orphan diseases are often deprived of adequate diagnosis and treatment. To promote a comprehensive study of orphan pulmonary diseases, we established a network of pulmonologists, allowing us to collect and study several series of patients. Based on the examples of lymphangioleiomyomatosis and idiopathic eosinophilic pneumonia, we emphasize the value of a global approach to orphan diseases within the context of a medical specialty.
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B Acad Nat Med Paris · Jan 2004
Review[Advances in comparative pathology: some zoonoses threatening man].
The last major human epidemics of infectious diseases have arisen from animals. Some of them are especially threatening. ⋯ They underline the role of animals in the epidemiology of SARS, West Nile virus, hepatitis E, NIPA and Hendra virus, ehrlichiosis and Lyme disease. The authors recommend health surveillance not only in humans but also in animals; the teaching of zoonoses, and research on animal diseases transmissible to humans.
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Sickle cell disease is an inherited disease characterized by the presence of an abnormal haemoglobin. It is the more prevalent genetic disease at birth in Ile-de France area. Sickle cell disease can be complicated by acute vaso-occlusive crisis, chronic visceral involvement related to ischemic process, and infectious complications. ⋯ It mainly manifests by osteoarticular pain and acute chest syndrome and can be complicated by multiorgan failure. Acute vaso-occlusive crisis can be also manifested by priapism, ischemic or haemorrhagic stroke, or abdominal pain. The main treatment of severe acute vaso-occlusive crisis is based on transfusion.
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Physicians and surgeons who treat patients with gastrointestinal or hepatic disease must prescribe the most appropriate diagnostic tests, together with an accurate prognosis and effective and safe therapy. This paper examines the best modalities of surgical treatment for cancer of the pancreas, in an evidence-based approach. Evidence was classified as follows: Grade A : evidence from large randomized controlled trials (RCT) or systematic reviews (including meta-analyses) of multiple randomized trials which collectively have at least as much data as one single well-defined trial. ⋯ Surgical resection and reconstruction procedures for pancreatic cancer must be based on evidence-based studies. However, the most important prognostic factor is the surgeon's experience, not only with regard to the post-operative course, but also survival. Specific teaching and training is thus essential.
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B Acad Nat Med Paris · Jan 2004
Review[The (pro)renin receptor: biology and functional significance].
Our model of the renin-angiotensin system has become increasingly complex with the identification of new components and additional roles for angiotensin peptides and their receptors. A functional (pro)renin receptor has been cloned. It acts as (pro)renin co-factor on the cell surface, enhancing the efficiency of angiotensinogen cleavage by (pro)renin and unmasking prorenin catalytic activity. ⋯ Immunofluorescence studies have located the receptor on mesangial and vascular smooth-muscle cells in human heart and kidney. This suggests that the renin receptor may represent a mean of capturing (pro)renin from the circulation and concentrating it at the interface between smooth-muscle and endothelial cells. This recent discovery of a functional (pro)renin receptor forces the emergence of a new concept that casts renin as potentially playing a direct role in tissue damage, especially in situations where the tissue RAS is activated.