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ELECTRONIC ARTICLE: Raoul H. H. Engelbert, Ruud A. Bank, Ralph J. B. Sakkers, Paul J. M. Helders, Frits A. Beemer, and Cuno S. P. M. Uiterwaal Pediatric Generalized Joint Hypermobility With and Without Musculoskeletal Complaints: A Localized or Systemic Disorder? Pediatrics 2003; 111: e248-e254 [Abstract] [Full text] [PDF]

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Adults with joint hypermobility also have organ systems involment other than joints. involment

Jaime F. Bravo, Colorado Medical Center in Denver. USA.   (27 May 2003)

Re: Adults with joint hypermobility also have organ systems involment other than joints. invol

raoul h.h. engelbert   (2 June 2003)

Developmental Issues in Children with Generalized Joint Hypermobility

Jean E. Teasley   (3 July 2003)

Untitled

raoul h.h. engelbert   (29 July 2003)

Adults with joint hypermobility also have organ systems involment other than joints. involment 27 May 2003
Jaime F. Bravo, Physician. Rheumatologist ACR. Clínica Arauco, Santiago, Chile. I used to be Clinical Professor of Rheumatology at the Col, Colorado Medical Center in Denver. USA. Send letter to journal: Re: Adults with joint hypermobility also have organ systems involment other than joints. involment jbravos{at}ctcinternet.cl Jaime F. Bravo, et al.	I read with extreme interest your article and I am happy to see that you are describing exactly the same that we see in adults. My patients with Benign Joint Hypermobility Syndrome (BJHS) besides hypermobility of joints have Disautonomia (Hypotension, dizziness, fainting spells, cold intolerance, chronic fatigue), osteopenia/osteoporosis, abnormal skin, soft tissue rheumatism, eye signs (drooping eyelids or miopia), varicose veins or hernias or uterine/rectal prolapse. Besides this, many have Marfanoid habitus. I am interested to know if you see Marfanoid children with hypermobility, without having Marfan. In adults, this is very frequent in Chile and I think that this must be a genetic mutation related to an Hereditary Alteration of the Collagen Fibers. I do not know if asymptomatic hypermobile adults (10% of our population) do or do not have other organ involments. This would be interesting to find out. As you see in pediatrics, symptomatic joint hypermobile patients are very frequent in my private practice also, they constitute 34% of all my patients.
Re: Adults with joint hypermobility also have organ systems involment other than joints. invol 2 June 2003
raoul h.h. engelbert, pediatric physical therapist Send letter to journal: Re: Re: Adults with joint hypermobility also have organ systems involment other than joints. invol r.engelbert{at}wkz.azu.nl raoul h.h. engelbert	dear collegue, thank you for your interest in our article. We measured besides body height and weight also armspan and sitting height. Armspan divided through body height did not extend 1.07, as is a criteria for Marfan syndrome. I our population we do have children with Marfanoid habitus. Since no clear criteria regarding clinical assessment have been made to distinguish pathology from normality we do not know if these children have a mild form of Marfan syndrome or a variation of normality. This is also the case in Ehlers Danlos syndrome. The distinction between the benign hypermobility syndrome and Ehlers Danlos type III is made on discutable criteria, since no collagen defect can be found yet. Clinical assessment of hypermobility with the Beighton or Bulbena scores is also very discutable. Generalised joint hypermobilty with a hyperextensible skin is not always pathological. In order to find answers, we are developing a new clinical instrument and already gathered reference values of 500 children and young adults. We hope that based on simple but clear and reliable measurements of joint motion and skin eventually combined with collagen data will lead to an instrument by which we can distinct normality from pathology. I hope that i was able to answer all the questions, sincerely yours dr. raoul engelbert
Developmental Issues in Children with Generalized Joint Hypermobility 3 July 2003
Jean E. Teasley, physician University of Alabam School of Medicine Send letter to journal: Re: Developmental Issues in Children with Generalized Joint Hypermobility jean.teasley{at}chsys.com Jean E. Teasley	I often see children with generalized joint hypermobility for developmental delay most often for gross motor skills though at times with fine motor skill difficulties. Many of these children have been seen by physical and occupational therapists whose evaluations are notable for weakness especially in the proximal muscles. I am appreciative of the information provided in this article and wonder if any of the children evaluated in this study with generalized joint hypermobility had developmental issues.
Untitled 29 July 2003
raoul h.h. engelbert, senior pediatric physical therapist WKZ/UMCU The Netherlands Send letter to journal: Re: this article r.engelbert{at}wkz.azu.nl raoul h.h. engelbert	dear collegue, thank you for your question. We recently assessed motor development in 72 children with generalised joint hypermobility and concluded that a severe delay in motor development was found in 23 of 72 children with generalised joint hypermobility (32%). (submitted for publication). We often measure weakness of the proximal muscles of the hip-joint (isometric hand held myometry compared to reference values for children). We also found that when muscle strength is normal, after extensive exercise muscle strength decreases possibly due to a lack of coordination and muscle co contraction

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