Fibrous dysplasia

被引:0
作者
Wirth, T. [1 ]
机构
[1] Klinikum Stuttgart, Olgahosp, Orthopad Klin, D-70176 Stuttgart, Germany
来源
ORTHOPADE | 2012年 / 41卷 / 12期
关键词
Fibrous dysplasia of bone; McCune-Albright syndrome; GNAS-gene; Coxa vara; Intramedullary nailing; MCCUNE-ALBRIGHT-SYNDROME; PRECOCIOUS PUBERTY; ALPHA-SUBUNIT; G-PROTEIN; BONE; PIGMENTATION; GENE; HYPERTHYROIDISM; DYSFUNCTION; MUTATIONS;
D O I
10.1007/s00132-012-1996-6
中图分类号
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学(修复外科学)];
学科分类号
摘要
Fibrous dysplasia is a benign intraosseous tumor disease which can be found in a monostotic or polyostotic form. In combination with dermatological and endocrine features it is known as McCune-Albright disease. Fibrous dysplasia originates from a genetic defect that has been identified as a postzygotic mutation of the GNAS gene leading to a malregulation of osteogenesis of the affected part of the bone. The weakening of the bone may result in isolated local pain, pathological fractures or severe deformation of the bones, which in the latter case results in loss of walking ability. Orthopedic treatment offers suitable methods to set and stabilize fractures, to strengthen weak areas of bone and to straighten out and stabilize deformed long bones. The treatment can help many patients to return to a high level of pain-free mobility or help the most affected patients lead a better life with a restricted but individual mobility.
引用
收藏
页码:993 / 1003
页数:11
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