Fibrous dysplasia in children and its management

被引:3
|
作者
Gun, Zubeyir Hasan [1 ,2 ]
Arif, Aleena [1 ]
Boyce, Alison M. [1 ]
机构
[1] NIDCR, Metab Bone Disorders Unit, NIH, Bethesda, MD USA
[2] NICHD, Pediat Endocrinol Interinst Training Program, NIH, Bethesda, MD USA
关键词
McCune-Albright syndrome; metabolic bone disorders; pediatrics; MCCUNE-ALBRIGHT SYNDROME; DOUBLE-BLIND; BONE; DISEASE; PROGRESSION; DENOSUMAB; OUTCOMES; IL-6;
D O I
10.1097/MED.0000000000000847
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
Purpose of reviewThe purpose of this review is to provide a comprehensive overview into the diagnosis and management of fibrous dysplasia (FD) in children.Recent findingsFD is a mosaic disorder arising from somatic G alpha s variants, leading to impaired osteogenic cell differentiation. Fibro-osseous lesions expand during childhood and reach final disease burden in early adulthood. The mainstay of treatment focuses on surgical correction of skeletal deformities, physiatric care, and medical management of associated hyperfunctioning endocrinopathies. Bisphosphonates may be helpful to treat bone pain, but do not alter lesion quality or progression. Emerging evidence suggests that the RANKL inhibitor denosumab may be effective in improving lesion activity and mineralization, however further studies are needed to determine the potential utility of this and other novel therapies, particularly in children with FD.SummaryManagement of children with FD has unique challenges related to skeletal growth and age-related lesion progression. Inclusion of children in clinical research is critical to develop effective treatment strategies to treat FD lesions and prevent their development.
引用
收藏
页码:60 / 66
页数:7
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