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5TH NATIONAL CONFERENCE OF PEDIATRIC RHEUMATOLOGY, KOLKATA, 29TH & 30TH SEPTEMBER 2007
Kevin J. Murray
Princess Margret Hospital for Children
Perth Western Australia
It is well recognized that many children attending paediatric rheumatology clinics will have a non-inflammatory origin for their complaints or disorder. Mechanical causes are frequently postulated, and hypermobility or ligamentous laxity of joints is increasingly recognized as an aetiological factor in such presentations. Such conditions may include 'growing pains', recurrent lower-limb arthralgias, anterior knee pain syndromes, and back pain. Similarly recurrent sprain and strain disorders, chronic fatigue and pain syndromes such as fibromyalgia and reflex sympathetic dystrophy have been linked at least in part to hypermobility and soft tissue laxity. Many lessons have been learned from specific disorders with extreme laxity, such as Ehlers Danlos syndrome and Marfan's syndrome but also bony dysplasias such as Sticklers syndrome which are known to involve genetic variations and mutations of connective tissue genes such as collagen and fibrillin.
Studies of significant paediatric cohorts of so called benign hypermobility syndrome patients have now been published supporting the link of ligamentous laxity to particular symptom complexes. However, much disagreement remains as to the degree of, or precise role of hypermobility as an aetiological factor. What seems clear is that not all hypermobile individuals will be symptomatic or indeed have a major risk for specific musculoskeletal disorders in later life.
Screening tools such as the Beighton score are likely to be inadequate in many paediatric populations because of the inherent greater laxity of joints in childhood as compared with adults, and major variation in joint laxity between different ethnic groups Effort to describe a paediatric measure (The Paediatric Beighton 13 point scale) are described. Other tissue factors may be important such as, poor muscle bulk, tone and proprioception. Similarly increased skin elasticity easy bruising and poor scar formation may be present indicating the link to a more generalised tissue variation or disorder. This has led to a concept of major and minor criteria (The "Brighton" Criteria) to help in appropriate understanding and diagnosis of patients.
Along with increasing recognition of these "disorders" or physiological variants in childhood, has been the development of a multidisciplinary management approach, which usually involves predominantly allied health professionals such as podiatrists, physiotherapists and occupational therapists. An approach to sensible management of the hypermobility disorders is described. In particular the usefulness of a positive diagnosis, reducing "doctor shopping" and over investigation and treatment for spurious disorders is emphasised. The answer to complex patient management is likely to involve a clear explanation of physiological and psychosocial factors. In addition, early identification and modification of risk factors may have major implications for subsequent prevalence of many adult medical disorders such as low back pain, chronic pain syndromes and degenerative osteoarthritis. The challenge remains to interpret symptoms correctly as being related to the hypermobility and to direct patients to healthy self management.
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