期刊
BEST PRACTICE & RESEARCH IN CLINICAL RHEUMATOLOGY
卷 14, 期 2, 页码 345-361出版社
ELSEVIER SCI LTD
DOI: 10.1053/berh.1999.0069
关键词
heritable disorders of connective tissue; Marfan syndrome; Ehlers-Danlos syndrome; osteogenesis imperfecta; benign joint hypermobility syndrome
类别
This chapter seeks to draw readers' attention to the importance of the heritable disorders of connective tissue in clinical practice. It describes the principal features of the Marfan and Ehlers-Danlos syndromes, osteogenesis imperfecta and benign joint hypermobility syndrome, their clinical and prognostic similarities and differences, and their distinguishing features. Recently revised international classifications drawing on advances in molecular genetics are described in detail. Wherever possible, patients' symptoms are explained on the basis of the altered biomechanics of genetically aberrant connective tissue matrix proteins. Finally, the chapter draws attention to the often unrecognized burden of chronic pain borne by patients with these conditions, a feature of which many rheumatologists seem unaware, and sets out a rational and holistic approach to treatment and management that is based on the best currently available evidence.
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