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Ehlers-Danlos氏 徵候群

Ehlers-Danlos Syndrome

최신의학 1970년 13권 2호 p.88 ~ 91
朴炳文, 張世昌,
소속 상세정보
朴炳文 (  ) - 延世大學校 醫科大學 整形外科敎室
張世昌 (  ) - 延世大學校 醫科大學 整形外科敎室

Abstract


The Ehlers-Danlos syndrome is an inherited disease of the connective tissue. It belongs to the same group of disorders as the Marfan & syndrome, osteogenesis imperfecta and pseudoxanthoma elastiium (Mc Kusick, 1960).
The main clinical features are: (1) fragility of skin and of dermal blood vessels, (2) characteristic "papyraceus" scar, (3) hyperextensible skin, and (4) hypermobile joints.
The basic defect is believed to lie in the organization of collagen into a stable collagen `Wickerwork" (Jansen, 1955).
Because the initial symptoms and the manifestations of this condition vary, it is important for all physician to be aware of this entity and its multiple system involvement.
Recently, we have experienced two cases of Ehlers-Danlos syndrom. Both patients are female and their presenting symptoms and singns are hyperela & ticity and fragility of skin and hypermobility of joints associated with congenital dislocation of both hip joints in one case, and subluxation of both radiohumeral joints and congenital dislocation of left hip joint in other.

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