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First International Symposium on the Ehlers-Danlos Syndrome

Posted By Donna Wicks, September 1, 2012

The first International Symposium on the Ehlers-Danlos syndrome (EDS) was held in the beautiful city of Ghent, Belgium, between 08th and 11th of September 2012.  The aim of the meeting was to bring together scientists and clinical specialists from all over the world to provide an overview of current knowledge on EDS and to be a forum to define research priorities and improve strategies for better care of patients affected by EDS.  Around 300 people attended the meeting; most of who were health professionals with special interest in heritable connective tissue conditions (geneticists, rheumatologists, dermatologists, vascular surgeons, psychologists etc). Representatives of EDS patient groups from Europe and UK were also present.

In the conference there were nine sessions, three of which concentrated on basic science related to connective tissue biology and EDS.  Two sessions covered the vascular type of EDS in humans and mice, and also EDS kyphoscoliotic type and related phenotypes.  One whole session was dedicated to hypermobility syndromes and the hypermobility type of EDS.  The presentations discussed the natural history and diagnostic criteria for this condition, issues and challenges in the management of Joint Hypermobility Syndrome (JHS).  Two very exciting studies were presented during this session discussing exercises and rehabilitation in patients with BJHS.  The first group from France described a technique called isokineticism and applied it in 24 patients.  They found that this was a relevant and safe tool for the articular stability improvement, the decrease in pain, and the restoration of function in hypermobile patients.

The second study was conducted in Australia and won a prize for the best presentation.  It described a randomised controlled trial comparing two different exercise programmes for children with joint hypermobility syndrome and knee pain.  This study involved 29 children aged 7-16 years.  The group showed that a supervised exercise programme was significantly more effective than no treatment in reducing pain, improving health- related quality of life, muscle strength and function.  It was interesting to see that self- esteem, mental health and behaviour improved more when exercises were performed into the hypermobile range.

Another very important session discussed psychological, ethical and legal aspects in patients with Ehlers-Danlos syndrome.

During the conference there was a panel discussion regarding strategies for updating the EDS criteria based on new genetic information.

In addition to very exciting oral presentations, 67 abstract posters were presented at the conference.  Few of these abstracts reported new genetic mutations in the rarer forms of EDS.  I have selected the abstracts which are most relevant in patients with benign joint hypermobility syndrome/EDS hypermobility type.

Pacey and colleagues from Australia studied the health-related quality of life in 29 children aged 7-16 with joint hypermobility syndrome and knee pain.  They reported that these children had marked reduced wellbeing compared to the general Australian paediatric population.  This reduction in quality of life was similar to those reported in children with certain types of myopathy

A group from Sweden found a significant difference in balance, pain and activity between children with JHS and children without JHS.

‘Hypermobile children with pain training – just do it?’  was the title of a very exciting poster from Sunnaas Rehabilitation Hospital in Norway.  The aim of this study was to see if children with hypermobility and pain responded positively to a muscle strengthening training programme.  The children were admitted for one week where they had various tests of motor skills and muscle strength, and learned traditional exercise strengthening programme.  Following discharge they were involved in home training for three months (daily self-training approximately 30 minutes with once weekly training under supervision by a local physiotherapist).  After three months the children were invited to go back to the rehabilitation hospital for retesting.  The results of this study were very encouraging ; all children achieved better muscle strength and function. Increased activity and less pain were reported by the children, parents and their physiotherapists.

Another group from the same hospital  conducted a similar  study in adults with BJHS.  The aim of this project was to explore the effect of a multidisciplinary rehabilitation programme in patients with JHS.  The patients were admitted for 2½ weeks and had physical training of strength , stability, posture and endurance.  They also attended lectures focussing on information about the diagnosis, energy conserving techniques and coping with pain.  Following discharge patients were involved in a home training programme for three months (five days a week for a minimum of 30 minutes, and once a week guidance from local physiotherapists).  At the end of the study patients were readmitted for four days to the Rehab Unit for retesting.

The results showed that participants reported better function in daily activities, improved muscle strength and muscle endurance, and decreased kinesiophobia(fear of movement).  There were smaller changes in self-perceived pain.

Three posters describing the gastrointestinal symptoms in patients with BJHS/EDS, hypermobility type, were presented.  Dr Asma Fikree and colleagues from Barts and The Royal London Hospital in collaboration with University College Hospital described 3 studies. The group found  that BJHS patients had significantly more upper GI symptoms, bloating and alternating bowel habits than patients with no hypermobility.  It was interesting to see that over 50% of patients with dysphasia were found to have oesophageal dysmotility using high resolution manometry (HRM) which is a relatively new technique.  In another study by the same group it was found that the majority of patients with reflux symptoms had pathological acid reflux .

The last talk was given by Professor Peter Byers from the University of Washington who was  one of the conference chairs .  He highlighted the important research which was presented during the three days of the conference.  He also discussed the importance of international future collaboration in basic science and clinical research.  He announced that the Second International Symposium on Ehlers-Danlos Syndrome would be in 2015.

 Dr Hanna KazKaz,
The Hypermobility Clinic,
Dept. of Rheumatology,
University College London Hospital,
3rd Floor,
250 Euston Road,

020 3456 7890

Published by HMSA 06.12.2012

©HMSA 2012

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