Onset, progression, and plateau of skeletal lesions in fibrous dysplasia and the relationship to functional outcome

被引:120
作者
Hart, Elizabeth S.
Kelly, Marilyn H.
Brillante, Beth
Chen, Clara C.
Ziran, Navid
Lee, Janice S.
Feuillan, Penelope
Leet, Arabella I.
Kushner, Harvey
Robey, Pamela G.
Collins, Michael T.
机构
[1] NIDCR, SCSU, CSDB, NIH, Bethesda, MD 20892 USA
[2] NIH, Hatfield Clin Res Ctr, Dept Nucl Med, Bethesda, MD 20892 USA
[3] Univ Rochester, Dept Orthoped, Rochester, NY USA
[4] Univ Calif San Francisco, San Francisco, CA 94143 USA
[5] NHGRI, NIH, Dept Hlth & Human Serv, Bethesda, MD 20892 USA
[6] Johns Hopkins Univ, Dept Orthoped, Baltimore, MD USA
[7] Biomed Comp Res Inst, Philadelphia, PA USA
关键词
fibrous dysplasia; bone; outcomes; McCune-Albright syndrome; GNAS; G(s)alpha; MCCUNE-ALBRIGHT-SYNDROME; STIMULATORY G-PROTEIN; MARROW STROMAL CELLS; BONE-MARROW; DYSFUNCTION; GENE; ABNORMALITIES; PIGMENTATION; MUTATIONS;
D O I
10.1359/JBMR.070511
中图分类号
R5 [内科学];
学科分类号
100201 [内科学];
摘要
Most lesions in FD and their attendant functional disability occur within the first decade; 90% of lesions are present by 15 years, and the median age when assistive devices are needed is 7 years. These findings have implications for prognosis and determining the timing and type of therapy.
引用
收藏
页码:1468 / 1474
页数:7
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