Výsledky vyhledávání - "Henri, Plauchu"

Classifying Ectopia Lentis in Marfan Syndrome into Five Grades of Increasing Severity

Autor: Sophie Dupuis-Girod, Patrick Edery, Evelyne Decullier, Jean-Christophe Zech, Henri Plauchu, Audrey Putoux, Anne-Emmanuelle Fargeton

Publikováno v: Journal of Clinical Medicine, Vol 9, Iss 3, p 721 (2020)
Journal of Clinical Medicine
Volume 9
Issue 3

Purpose: To describe a five-grade classification of ectopia lentis in Marfan syndrome (MFS) and to evaluate the positive predictive value of the early grades of ectopia lentis. Methods: We prospectively included MFS patients and their healthy relativ

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::52cd4eb9f4f96812d8af389bdd249059
https://www.mdpi.com/2077-0383/9/3/721

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Cerebral abscesses in hereditary haemorrhagic telangiectasia: A clinical and microbiological evaluation

Autor: Kim Heang Ly, Gaëlle Godeneche, Henri Plauchu, Brigitte Gilbert, Pierre Ingrand, Bruno Barroso, Sophie Dupuis-Girod, Maurice Giroud, Jean-Philippe Neau, Stéphane Mathis

Publikováno v: Clinical Neurology and Neurosurgery. 114:235-240

Objectives Hereditary haemorrhagic telangiectasia (HHT) is a rare autosomal dominant disorder that can lead to neurological manifestations including strokes and cerebral abscesses. Our objectives were to describe clinical, radiological, bacteriologic

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a192f4ef36243a9c536ab33c2977018c
https://doi.org/10.1016/j.clineuro.2011.10.036

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How to improve specific databases for clinical data in rare diseases? The example of hereditary haemorrhagic telangiectasia

Autor: Sophie Dupuis-Girod, Evelyne Decullier, François Chapuis, Henri Plauchu, Jacques Perret

Publikováno v: Journal of Evaluation in Clinical Practice. 18:523-527

Introduction The objectives of reference centres for rare diseases are multiple and mainly concern disease management and coordination between specialties, but they also have to improve knowledge through epidemiological studies and biomedical researc

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::186c14a3e6e64ecdcf18fc05aca0f1a8
https://doi.org/10.1111/j.1365-2753.2010.01627.x

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Effect of celiprolol on prevention of cardiovascular events in vascular Ehlers-Danlos syndrome: a prospective randomised, open, blinded-endpoints trial

Autor: Jérôme Perdu, Kim-Thanh Ong, Anne De Paepe, G. Georgesco, Pierre Boutouyrie, Jean-Sébastien Hulot, Anne-Laure Fauret, Patrick Collignon, Joseph Emmerich, Jean Noel Fiessinger, Henri Plauchu, Erwan Bozec, Dominique P. Germain, Julie De Backer, Stéphane Laurent, Xavier Jeunemaitre

Publikováno v: The Lancet. 376:1476-1484

Summary Background Vascular Ehlers-Danlos syndrome is a rare severe disease that causes arterial dissections and ruptures that can lead to early death. No preventive treatment has yet been validated. Our aim was to assess the ability of celiprolol, a

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::9f7f6e09ed72d814d590a1ef644e4e52
https://doi.org/10.1016/s0140-6736(10)60960-9

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Maladie de Rendu-Osler

Autor: Sabine Bailly, Henri Plauchu, Sophie Dupuis-Girod

Publikováno v: médecine/sciences. 26:855-860

La maladie de Rendu Osler, encore appelee telangiectasie hemorragique hereditaire (HHT) touche une personne sur 5 000 a 8 000. Une large etude epidemiologique realisee en France a montre une concentration geographique dans le Jura. Cette pathologie s

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::d10eefa6527fd95f550917989d17f550
https://doi.org/10.1051/medsci/20102610855

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Long-term outcome of patients with hereditary hemorrhagic telangiectasia and severe hepatic involvement after orthotopic liver transplantation: A single-center study

Autor: Isabelle Ginon, Gérard Finet, Jérôme Dumortier, Anne-Laure Chesnais, Evelyne Decullier, Denis Marion, Olivier Boillot, Sophie Dupuis-Girod, Henri Plauchu, Jean-Christophe Saurin

Publikováno v: Liver Transplantation. 16:340-347

Hepatic involvement occurs in up to 74% of patients with hereditary hemorrhagic telangiectasia (HHT) and is characterized by a spectrum of arteriovenous malformations. Three different types of intrahepatic shunting may be present: hepatic artery to h

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::44d493f3c0e6b294d314af5474e986fb
https://doi.org/10.1002/lt.21990

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Thirty Years of Research into Rendu-Osler-Weber Disease in France: Historical Demography, Population Genetics and Molecular Biology

Autor: Guy Brunet, Gaëtan Lesca, Emmanuelle Génin, Sophie Dupuis-Girod, Alain Bideau, Henri Plauchu

Publikováno v: Population (english edition). 64(2):273-291

Rendu-Osler-Weber disease, also known as hereditary hemorrhagic telangiectasia (HHT), has been the focus of several interdisciplinary studies over the last thirty years. An initial epidemiological survey conducted in the 1980s revealed that the preva

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=od_______645::0bf1ee16d523b2daaf068d560d2fb888
http://www.cairn.info/revue-population-english-2009-2-page-273.htm

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Acute paraplegia due to spinal arteriovenous fistula in two patients with hereditary hemorrhagic telangiectasia

Autor: Henri Plauchu, Alice Poisson, Francis Brunelle, Sophie Dupuis-Girod, Ashok Vasdev

Publikováno v: European Journal of Pediatrics. 168:135-139

Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant disorder characterized by recurrent epistaxis, cutaneous telangiectasia, and visceral arteriovenous malformations (AVM). Of these, spinal AVM is a rare manifestation that concerns m

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https://doi.org/10.1007/s00431-008-0863-2

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Bone morphogenetic protein-9 is a circulating vascular quiescence factor.: BMP9 is present in serum and inhibits angiogenesis

Autor: Jean-Jacques Feige, Jean-Marie Gasc, Michelle Keramidas, Noël Lamandé, Sabine Bailly, Laurent David, Christine Mallet, Henri Plauchu, Sophie Dupuis-Girod

Publikováno v: Circulation Research
Circulation Research, American Heart Association, 2008, 102 (8), pp.914-22. ⟨10.1161/CIRCRESAHA.107.165530⟩
Circulation Research, 2008, 102 (8), pp.914-22. ⟨10.1161/CIRCRESAHA.107.165530⟩

Angiogenesis is a complex process, requiring a finely tuned balance between numerous stimulatory and inhibitory signals. ALK1 (activin receptor like-kinase 1) is an endothelial-specific type 1 receptor of the transforming growth factor-β receptor fa

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::eda00c8ee43037b58d7d32ddd41aaa1e
https://doi.org/10.1161/circresaha.107.165530

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