先天股脱を合併した Ehlers-Danlos 症候群の1例

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  • A Case Report of Congenital Dislocations of Both Hip Associated with Ehlers-Danlos Syndrome

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The patient was a two years and ten months old girl who had been treated her congenital dislocations of hips since four month old. Recently she was received the operation of varus-detorsion osteotomy to the right femur.<br>The patient had not only the Ronchese's triad for Ehlers-Danlos Syndrome; dermatorrhexis, dermatochalasis, and arthrochalasis, but also blue sclera and funnel chest. According her skin pathology, there found the thinning of collagen fibers and increased elastic fibers. In the laboratory work-ups, slight elongation of bleeding time and marked positive Rumpel-Leede test were found, although normal CPK level, Thorn test, blood coagulation time, urine hydroxyproline, and urine creatinine level.

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