Pamidronate Treatment of Polyostotic Fibrous Dysplasia: Failure to Prevent Expansion of Dysplastic Lesions During Childhood
Pamidronate Treatment of Polyostotic Fibrous Dysplasia: Failure to Prevent Expansion of Dysplastic Lesions During Childhood
复制标题
帕米膦酸钠治疗多发性纤维性发育不良：未能预防儿童期发育不良病变的扩大
DOI：
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发表时间：
2006
期刊：
影响因子：
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通讯作者：
M. Zacharin
中科院分区：
文献类型：
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作者：
B. Chan;M. Zacharin
旨在
探讨帕米膦酸二钠治疗3例McCune-Albright综合征（MAS）儿童骨纤维异常增殖症的疗效。
方法
对3名MAS儿童的骨纤维异常增殖症进展的影像学证据进行了审查，这些儿童在2.5-5岁时接受了帕米膦酸盐治疗，持续8-10.5年。
结果
尽管长骨的疼痛最小，骨折率低，但除了存在严重畸形的地方，长骨中存在的所有发育不良病变继续经历不受控制的扩张。相比之下，在任何患者中，面部结构没有重大的新变化，蝶翼病变没有临床上明显的扩大，没有侵犯视神经孔或视野限制。
结论
尽管之前有报道称成人和儿童的骨纤维异常增殖症病变大小受限或缩小，但我们的经验是，双膦酸盐治疗MAS儿童的多骨纤维异常增殖症并不能阻止这些病变的扩大。
AIMS
To examine outcomes of pamidronate treatment on fibrous dysplasia of bone in three children with McCune-Albright syndrome (MAS).
METHODS
Radiological evidence of fibrous dysplasia progress was reviewed for three children with MAS who were treated with pamidronate from age 2.5-5 years, for 8-10.5 years.
RESULTS
Despite minimal pain and a low fracture rate in long bones, except where gross deformity exists, all dysplastic lesions present in long bones continued to undergo uncontrolled expansion. In contrast, there were no major new changes in facial configuration, no clinically obvious expansion of sphenoid wing lesions and no encroachment on optic foramina or visual field restriction in any patient.
CONCLUSIONS
Despite previous reports of limitation or reduction in size of fibrous dysplasia lesions in adults and children, it is our experience that bisphosphonate treatment of polyostotic fibrous dysplasia in children with MAS does not arrest the expanding nature of these lesions.
麦库恩-奥尔布赖特综合症。
DOI：
10.1530/acta.0.112s207
发表时间：
1986
期刊：
Acta endocrinologica. Supplementum
影响因子：
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作者：
Mauras,N;Blizzard,RM
通讯作者：
Blizzard,RM