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儿童戈谢病并发股骨颈骨折。

Femoral neck fractures complicating Gaucher disease in children.

作者信息

Goldman A B, Jacobs B

出版信息

Skeletal Radiol. 1984;12(3):162-8. doi: 10.1007/BF00361082.

Abstract

In normal children, fractures of the femoral neck are uncommon and accompany severe trauma and multiple injuries elsewhere in the skeleton [16, 17]. In children with Gaucher disease, a rare hereditary disorder of lipid metabolism, midcervical or basicervical fractures can occur with minor or no trauma and without other injury to the skeleton. Three children with Gaucher disease who developed pathologic fractures of the femoral neck are described. In all three, the fractures occurred between five and nine years of age, and the fracture lines passed through areas of abnormal bone characterized by poorly defined patches of increased and decreased density and cortical thinning along the medial femoral necks. In the affected hips, there was no evidence of avascular necrosis of the femoral heads at the time of injury. One child's fracture was preceded by multiple bone "crises" localized to the proximal femora.

摘要

在正常儿童中,股骨颈骨折并不常见,通常伴随着严重创伤以及骨骼其他部位的多发损伤[16, 17]。在患有戈谢病(一种罕见的脂质代谢遗传性疾病)的儿童中,股骨颈中部或基部骨折可能在轻微创伤或无创伤的情况下发生,且骨骼无其他损伤。本文描述了三名患有戈谢病且发生股骨颈病理性骨折的儿童。在这三名儿童中,骨折均发生在5至9岁之间,骨折线穿过异常骨区域,这些区域的特征是股骨颈内侧密度增加和减少的区域界限不清,伴有皮质变薄。在受伤时,受累髋关节没有股骨头缺血性坏死的迹象。一名儿童的骨折之前,近端股骨出现了多次局限性骨“危象”。

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