ehlers-danlos syndrome eds
Post on 23-Jan-2016
Embed Size (px)
DESCRIPTIONEhlers-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
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
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)