Chan Brendan, Zacharin Margaret
Department of Endocrinology and Diabetes, Royal Children's Hospital, Parkville, Victoria, Australia.
J Pediatr Endocrinol Metab. 2006 Jan;19(1):75-80. doi: 10.1515/jpem.2006.19.1.75.
To examine outcomes of pamidronate treatment on fibrous dysplasia of bone in three children with McCune-Albright syndrome (MAS).
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.
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.
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.
研究帕米膦酸治疗三名患有McCune-Albright综合征(MAS)儿童骨纤维发育不良的疗效。
回顾了三名MAS儿童的骨纤维发育不良进展的放射学证据,这些儿童从2.5至5岁开始接受帕米膦酸治疗,为期8至10.5年。
尽管长骨疼痛轻微且骨折率低,但除存在严重畸形的部位外,长骨中存在的所有发育异常病变仍继续不受控制地扩大。相比之下,面部形态没有重大新变化,蝶骨翼病变在临床上没有明显扩大,任何患者均未出现视神经孔受压或视野受限。
尽管之前有报道称成人和儿童骨纤维发育不良病变的大小受到限制或缩小,但我们的经验是,双膦酸盐治疗MAS儿童的多骨型骨纤维发育不良并不能阻止这些病变的扩大。