机构:
NIDCR, Metab Bone Disorders Unit, NIH, Bethesda, MD USA
NICHD, Pediat Endocrinol Interinst Training Program, NIH, Bethesda, MD USANIDCR, Metab Bone Disorders Unit, NIH, Bethesda, MD USA
Gun, Zubeyir Hasan
[1
,2
]
Arif, Aleena
论文数: 0引用数: 0
h-index: 0
机构:
NIDCR, Metab Bone Disorders Unit, NIH, Bethesda, MD USANIDCR, Metab Bone Disorders Unit, NIH, Bethesda, MD USA
Arif, Aleena
[1
]
Boyce, Alison M.
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机构:
NIDCR, Metab Bone Disorders Unit, NIH, Bethesda, MD USANIDCR, Metab Bone Disorders Unit, NIH, Bethesda, MD USA
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
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.
机构:
Univ Castilla La Mancha, Dept Math, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, Spain
Univ Castilla La Mancha, Math Oncol Lab MOLAB, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, SpainUniv Castilla La Mancha, Dept Math, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, Spain
Beltran-Vargas, Juan C.
de Castro, Luis F.
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h-index: 0
机构:
NIH, Bldg 30,Room 209,MSC 4320, Bethesda, MD 20892 USAUniv Castilla La Mancha, Dept Math, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, Spain
de Castro, Luis F.
Calvo, Gabriel F.
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机构:
Univ Castilla La Mancha, Dept Math, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, Spain
Univ Castilla La Mancha, Math Oncol Lab MOLAB, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, SpainUniv Castilla La Mancha, Dept Math, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, Spain
Calvo, Gabriel F.
Perez-Garcia, Victor M.
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机构:
Univ Castilla La Mancha, Dept Math, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, SpainUniv Castilla La Mancha, Dept Math, Edificio Politecn,Avda Camilo Jose Cela S-N, Ciudad Real 13071, Castilla La Man, Spain
机构:
Cleveland Clin, Musculoskeletal Tumor Ctr, Main Campus Mail Code A41,9500 Euclid Ave, Cleveland, OH 44195 USACleveland Clin, Musculoskeletal Tumor Ctr, Main Campus Mail Code A41,9500 Euclid Ave, Cleveland, OH 44195 USA
Lietman, Steven A.
Levine, Michael A.
论文数: 0引用数: 0
h-index: 0
机构:
Childrens Hosp Philadelphia, Div Endocrinol & Diabet, Philadelphia, PA 19104 USA
Childrens Hosp Philadelphia, Ctr Bone Hlth, Philadelphia, PA 19104 USA
Univ Penn, Pediat & Med, Perelman Sch Med, Philadelphia, PA 19104 USACleveland Clin, Musculoskeletal Tumor Ctr, Main Campus Mail Code A41,9500 Euclid Ave, Cleveland, OH 44195 USA