ehlers-danlos syndrome eds

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Ehlers-Danlos Syndrome EDS. Hôtel-Dieu de Paris Bird meeting ASED 06 09 2012. Claude Hamonet*, Monique Vienne **. *Department of Physical Medicine and Rehabilitation (Pr. S. Poiraudeau, Dr. JY Maigne), Hôtel-Dieu Hospital, Paris **IMAP, Nice. - PowerPoint PPT Presentation

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  • Ehlers-Danlos SyndromeEDS Htel-Dieu de ParisBird meeting ASED 06 09 2012

  • Claude Hamonet*, Monique Vienne **

    *Department of Physical Medicine and Rehabilitation (Pr. S. Poiraudeau, Dr. JY Maigne), Htel-Dieu Hospital, Paris **IMAP, Nice.With support of Department of Public health and Epidemiology (Pr. Ravaud), Htel-Dieu Hospital Paris.

    Grant : Fondation de France.

  • Welcome Dr. Bird, you safe the life of our patients or you enhance their quality of life

  • Andr GrossiordThe pionner of French Rehabilitation. Garches.

  • First description : a law student. By Edward EHLERS, a Dermatologist,

    Danish Society of Dermatology Copenhaguen 12 15 1900. Published in german language

  • What is EDS today?

    A genetic disorder of connective tissue (collagen especially) autosomal dominant. Without genetic test, the diagnosis is clinical

    Which is not a "rare disease" but a frequent and an syndrome currently confused with another diagnosis

  • Most important signs for diagnosis (personnal serie of 586 patients Beighton scored 4/9 or more)PAINS (98%): (joints, muscles, belly, chest, skin, head,..) 71% very intensives ASTHENIA (97%) 69% very intensivePROPRIOCEPTIVES disorders (85%)HYPERMOBILITY (100%)HEMORRHAGES (92%) FRAGILITY OF THE SKIN (86,6%)Other family case(s) (95%).

  • Respiratory symptoms in EDS (586 patients) Pseudo Asthma : 65%

    Dyspnea : 66%

    Dysphonia : 53%

    Bronchitis : 56%

  • Strentchening of skin Popular but inconstant

  • Beighton test : the thumb sign

  • KNEES Recurvatum

  • Ecchymosis

  • EDS FOOT (Flat forward, Hollow in the middle, normal behind).

  • Pathophysiological hypothesis (proprioception) No (or bad) emission of signals from sensors rom too soft tissues.

  • Proprioceptive garments Cereplas.

  • Rigid trunk orthesis

  • Special seat adapted for a young boy with EDS

  • Light rest hand orthesis (Nokraft)

  • Functionnal hand splint

  • Murphy finger orthesis

  • Lower limb orthesis with hip device to stop hip luxation

  • Lower limbs orthesis with elastic artificial muscles (Made by JC Chignon, Orthesist)

  • She is walking,She was in a Wheelchair !

  • The discovery of Dr. Bird1980, F.M. BIRD invented Percussive intrapulmonary Ventilation ,with internal percussion of pulmonary structures by IMPULSATOR

  • Effects of ImpulsatorImprove breathing status and the new discover that (+ 02) is also in EDS a treatmentagainst fatigue and migraines !

  • A chidren using percussionnaire + oxygen at homeCarnitine is also used with successagainst the fatigue.

  • ConclusionTo believe in rehabilitation is to believe in humanityHoward Rusk (New-York)

    *

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