An instrument to measure skeletal burden and predict functional outcome in fibrous dysplasia of bone

被引:98
作者
Collins, MT
Kushner, H
Reynolds, JC
Chebli, C
Kelly, MH
Gupta, A
Brillante, B
Leet, AI
Riminucci, M
Robey, PG
Bianco, P
Wientroub, S
Chen, CC
机构
[1] NIDCR, Craniofacial & Skeletal Dis Branch, NIH, Dept Hlth & Human Serv, Bethesda, MD 20892 USA
[2] Biomed Comp Res Inst, Philadelphia, PA USA
[3] NIH, Dept Nucl Med, Dept Hlth & Human Serv, Bethesda, MD USA
[4] Univ Maryland, Dept Orthopaed Surg, Baltimore, MD 21201 USA
[5] NIH, Warren G Magnuson Clin Ctr, Dept Nursing Serv, Dept Hlth & Human Serv, Bethesda, MD 20892 USA
[6] Johns Hopkins Univ, Dept Orthoped, Div Pediat Orthoped, Baltimore, MD USA
[7] Sperimentale Univ Laquila, Dipartimento Med, Laquila, Italy
[8] Parco Sci Biomed San Raffaele, Rome, Italy
[9] Univ Roma La Sapienza, Dipartimento Med Sperimentale & Patol, Rome, Italy
[10] Tel Aviv Univ, Sackler Fac Med, Tel Aviv Med Ctr, Dana Childrens Hosp,Dept Pediat Orthopaed Surg, IL-69978 Tel Aviv, Israel
关键词
fibrous dysplasia; outcomes(s); instrument; disease burden; McCune-Albright syndrome; GNAS;
D O I
10.1359/JBMR.041111
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
An instrument to measure skeletal burden in fibrous dysplasia was developed. Biological and clinical relevance was shown by correlating skeletal burden scores with bone markers, quality of life, and ambulatory status. Childhood scores predict adult ambulatory status, and scores were unaffected when bone markers decreased with bisphosphonate treatment or aging.
引用
收藏
页码:219 / 226
页数:8
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