Retrospective French nationwide survey of childhood aggressive vascular anomalies of bone, 1988-2009

<p>Abstract</p> <p>Objective</p> <p>To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children.</p> <p>Study design</p> <p>Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009.</p> <p>Results</p> <p>21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications.</p> <p>Conclusion</p> <p>Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.</p

Introduction to French Culture

Ce cours est une introduction &agrave; la culture et la soci&eacute;t&eacute; fran&ccedil;aises depuis la R&eacute;volution, mais surtout &agrave; partir du Second Empire.&nbsp;Nous tacherons de cerner ce qui d&eacute;finit la singularit&eacute; francaise dans une perspective historique.&nbsp;Nous commencerons avec la notion "d'exception francaise" et de ce qui la constitue depuis la R&eacute;volution (La R&eacute;publique, L'Universalisme, La Laicit&eacute;, etc.) Nous explorerons l'impact de l'industrialisation, ainsi que la tension entre la France pays de la modernit&eacute; dans les arts et la technologie et la France nostalgique de sa grandeur pass&eacute;e.&nbsp;Nous discuterons les moments charni&egrave;res de cette tension avec les grands d&eacute;bats d'id&eacute;es autour des impressionistes, de "Fleurs du Mal", du Paris de Haussmann, de la construction de la Tour Eiffel, des expositions universelles et coloniales.&nbsp;Nous analyserons la repr&eacute;sentation litt&eacute;raire et filmique des moments historiques et des crises hexagonales qui ont marqu&eacute; le 20e si&egrave;cle: l'Affaire Dreyfus, les deux guerres mondiales le colonialisme et la guerre d'Alg&eacute;rie, Mai 68.&nbsp;A travers des textes, des images, des articles de journaux, des films, nous tenteront de faire le lien entre cette histoire et les d&eacute;bats contemporrains sur l'identit&eacute; Fran&ccedil;aise

Violette Leduc : L'enfermée pèlerine

de Courtivron Isabelle. Violette Leduc : L'enfermée pèlerine. In: Les Cahiers du GRIF, n°39, 1988. recluses vagabondes. pp. 49-53