{"title":"Scoliose et syndrome d’Ehlers-Danlos (SED)","authors":"F. Guillou","doi":"10.1016/j.jrm.2015.09.001","DOIUrl":"10.1016/j.jrm.2015.09.001","url":null,"abstract":"<div><p>La maladie d’Ehlers-Danlos reste une pathologie peu connue avec souvent un retard diagnostique important. La scoliose est un des symptômes inconstants du SED mais tous les auteurs reconnaissent le SED comme une étiologie des scolioses. Il faut donc pouvoir évoquer le diagnostic de SED devant une scoliose atypique. Cependant, très peu de références existent sur la scoliose et le SED dans la littérature.</p></div><div><p>Ehlers-Danlos syndrome (EDS) remains a little known pathology with often an important diagnosis delay. Scoliosis is an uncommon symptom of EDS but all the authors recognize the EDS as an etiology of scoliosis. Therefore, the diagnosis of EDS should be suspected in case of atypical scoliosis; however, very little references exist on scoliosis and EDS in the literature.</p></div>","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Pages 46-51"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jrm.2015.09.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"77143459","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"La dysautonomie dans le syndrome Ehlers-Danlos type III","authors":"J.F. Bravo","doi":"10.1016/j.jrm.2016.01.001","DOIUrl":"10.1016/j.jrm.2016.01.001","url":null,"abstract":"<div><p>La dysautonomie est une manifestation extrêmement fréquente, associée généralement au syndrome d’Ehlers-Danlos de type III (connu aussi sous le nom de syndrome d’hypermobilité articulaire [SHA]) à l’origine de complications et d’une altération de la qualité de vie du patient, généralement non diagnostiquées. Nous présentons ici les signes et les symptômes qui facilitent le diagnostic ainsi que les traitements de ces patients.</p></div><div><p>Dysautonomia is an extremely frequent condition, generally associated to Ehlers–Danlos type III (also known as joint hypermobility syndrome [JHS]), that causes complications and poor quality of life to the patient and that usually goes undiagnosed. In this article, we present signs and symptoms that help the diagnosis and treatment of these patients.</p></div>","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Pages 52-55"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jrm.2016.01.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"90788294","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Le syndrome d’hypermobilité","authors":"R. Grahame","doi":"10.1016/j.jrm.2015.09.008","DOIUrl":"10.1016/j.jrm.2015.09.008","url":null,"abstract":"","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Pages 7-8"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jrm.2015.09.008","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"75751962","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
C. Hamonet , E. Vlamyncx , R. Haidar , C. Bonny , G. Mazaltarine
{"title":"Le syndrome d’Ehlers-Danlos de type III. De la physiopathologie à la thérapeutique","authors":"C. Hamonet , E. Vlamyncx , R. Haidar , C. Bonny , G. Mazaltarine","doi":"10.1016/j.jrm.2015.11.001","DOIUrl":"10.1016/j.jrm.2015.11.001","url":null,"abstract":"<div><p>Depuis 17<!--> <!-->ans, nous suivons une cohorte patients atteints d’une maladie d’Ehlers-Danlos de type III répondant aux critères de Brighton (Grahame) et de Villefranche (Beighton). Ils sont maintenant 2350. Les manifestations cliniques observées, plus sévères chez les femmes, sont l’expression d’un syndrome de fragilités tissulaires et de désordres proprioceptifs. L’atteinte héréditaire à transmission non mendélienne du tissu collagène compromet la solidité et la réactivité de l’ensemble du dispositif conjonctif du corps humain. De ce constat découlent des conséquences physiopathologiques qui permettent de mieux interpréter la clinique de cette maladie du <em>sixième sens</em> : celui de la perception de son propre corps et d’orienter les thérapeutiques. Celles-ci ont pour objectif de restaurer la proprioception, notamment à l’aide des orthèses et particulièrement des vêtements compressifs. L’oxygénothérapie apparaît comme le meilleur traitement de la fatigue. La chirurgie a de très rares indications et peut provoquer des aggravations importantes. L’apport de la psychologie est important.</p></div><div><p>Since 17 years, we follow a cohort of patients with Ehlers-Danlos disease type III in concordance with the criteria of Brighton (Grahame) and Villefranche (Beighton). They are now 2350. The observed clinical manifestations, more severe in females, are the expression of a syndrome of tissue fragility and proprioceptive disorders. Hereditary non-mendelian inheritance affect the tissue collagen impairs the strength and responsiveness of the entire connective system of the human body. From this observation result of the pathophysiological consequences to better interpret clinical disease that <em>sixth sense</em>: that of perception of her own body and guide treatment. These aim to restore proprioception, especially using orthosis and particularly compression garments. Oxygen therapy appears to be the best treatment for fatigue. Surgery has very few indications and can cause significant aggravations. The contribution of psychology is important.</p></div>","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Pages 32-37"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jrm.2015.11.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86612559","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Syndrome d’Ehlers-Danlos et odontologie","authors":"M. Delarue","doi":"10.1016/j.jrm.2015.10.006","DOIUrl":"10.1016/j.jrm.2015.10.006","url":null,"abstract":"<div><p>Les odontologistes connaissent mieux l’ostéogenèse imparfaite et la maladie de Marfan, deux autres pathologies héréditaire du collagène différentes du syndrome d’Ehlers-Danlos qui est pourtant, de très loin, plus fréquent. Les manifestations bucco-dentaires sont nombreuses, souvent handicapantes sur le plan fonctionnel. Elles contribuent au diagnostic et l’odontologiste peut être la praticien qui évoque le premier la maladie très rarement diagnostiquée ? Les anomalies de forme sont nombreuses : les racines sont souvent plus courtes, il est fréquent de retrouver des hypoplasies amélaires. La fragilité muqueuse se retrouve chez les trois quarts des patients atteints d’un SED. L’atteinte des articulations temporo-mandibulaires (ATM) est présente chez 47 % d’entre eux pouvant être à l’origine de troubles posturaux. La résistance aux anesthésiques locaux est habituelle. Il n’y a aucun risque d’infection oslérienne même en présence de modifications des valves cardiaques. Les gestes chirurgicaux et orthodontiques doivent être atraumatiques du fait de la grande fragilité de tous les tissus. Une adaptation au cas par cas est nécessaire.</p></div><div><p>Odontologists are more familiar with osteogenesis imperfecta and Marfan's disease, two other inherited diseases of collagen than Ehlers-Danlos syndrome is yet, by far more, common. The oral manifestations are numerous, often disabling functionally. They contribute to the diagnosis and the dentist may be the first practitioner that evokes very rarely diagnosed disease? Shaped anomalies are numerous: the roots are often shorter, it is common to find the enamel hypoplasia. Mucosal fragility is found in three quarters of patients with EDS. The achievement of the temporo-mandibular joints is present in 47% of which may cause postural disorders. The resistance to local anesthetics is usual. There is no risk of oslerian infection even in the presence of cardiac valves modifications. Surgical and orthodontic actions must be atraumatic due to the fragility of all the tissues. An adaptation in each case is required.</p></div>","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Pages 85-88"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jrm.2015.10.006","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"84950285","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
J. Bravo , M. Castori , R. Graham , C. Hamonet , D. Manicourt
{"title":"Déclaration commune sur le syndrome d’Ehlers-Danlos (SED), en particulier sur sa forme « hypermobile »","authors":"J. Bravo , M. Castori , R. Graham , C. Hamonet , D. Manicourt","doi":"10.1016/j.jrm.2015.09.005","DOIUrl":"10.1016/j.jrm.2015.09.005","url":null,"abstract":"","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Pages 89-90"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jrm.2015.09.005","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91273767","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"CV2 - Editorial Board","authors":"","doi":"10.1016/S0242-648X(16)00005-0","DOIUrl":"https://doi.org/10.1016/S0242-648X(16)00005-0","url":null,"abstract":"","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Page IFC"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0242-648X(16)00005-0","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"136853349","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Syndrome d’Ehlers-Danlos (SED) : de l’identification aux traitements d’une maladie au destin contrarié","authors":"C. Hamonet","doi":"10.1016/j.jrm.2015.12.001","DOIUrl":"10.1016/j.jrm.2015.12.001","url":null,"abstract":"","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"36 1","pages":"Pages 5-6"},"PeriodicalIF":0.0,"publicationDate":"2016-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/j.jrm.2015.12.001","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"85724753","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Apparence, odontologie et situations de handicap","authors":"S. Moussa-Badran","doi":"10.1016/J.JRM.2015.08.002","DOIUrl":"https://doi.org/10.1016/J.JRM.2015.08.002","url":null,"abstract":"","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"2011 1","pages":"195-196"},"PeriodicalIF":0.0,"publicationDate":"2015-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"86318287","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"CV2 - Editorial Board","authors":"","doi":"10.1016/S0242-648X(15)00143-7","DOIUrl":"https://doi.org/10.1016/S0242-648X(15)00143-7","url":null,"abstract":"","PeriodicalId":100745,"journal":{"name":"Journal de Réadaptation Médicale : Pratique et Formation en Médecine Physique et de Réadaptation","volume":"35 4","pages":"Page IFC"},"PeriodicalIF":0.0,"publicationDate":"2015-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.1016/S0242-648X(15)00143-7","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"91680615","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}