Scoliosis in Fibrous Dysplasia/McCune‐Albright Syndrome: Factors Associated With Curve Progression and Effects of Bisphosphonates
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DOI: 10.1002/jbmr.3446
Abstract: Scoliosis is a complication of fibrous dysplasia/McCune‐Albright syndrome (FD/MAS); however, risk factors and long‐term outcomes are unknown. Bisphosphonates are commonly used; however, it is unknown whether their use decrease the risk of progressive scoliosis. Clinical… read more here.
Keywords: albright syndrome; dysplasia mccune; fibrous dysplasia; scoliosis ... See more keywords
Chiari I Malformation and Basilar Invagination in Fibrous Dysplasia: Prevalence, Mechanisms, and Clinical Implications
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DOI: 10.1002/jbmr.3531
Abstract: Fibrous dysplasia (FD) is a mosaic disorder of benign fibro‐osseous lesions, which may be associated with extraskeletal features as part of McCune‐Albright syndrome (MAS). Cranial base abnormalities, including Chiari I malformation (CM1), in which the… read more here.
Keywords: basilar invagination; base abnormalities; base; fibrous dysplasia ... See more keywords
Utility of Optical Coherence Tomography in the Diagnosis and Management of Optic Neuropathy in Patients with Fibrous Dysplasia
Sign Up to like & getrecommendations! Published in 2020 at "Journal of Bone and Mineral Research"
DOI: 10.1002/jbmr.4129
Abstract: Optic neuropathy (ON) is a highly disabling complication of fibrous dysplasia (FD). The optimal test for identifying and monitoring ON in FD is unknown. Optical coherence tomography (OCT) is an imaging modality that detects retinal… read more here.
Keywords: rnfl; fibrous dysplasia; rnfl thickness; optic neuropathy ... See more keywords
Identification of GNAS Variants in Circulating Cell‐Free DNA from Patients with Fibrous Dysplasia/McCune Albright Syndrome
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DOI: 10.1002/jbmr.4766
Abstract: Fibrous dysplasia/McCune‐Albright syndrome (FD/MAS) is a rare mosaic bone and endocrine disorder. Although most variants affect the GNAS R201 codon, obtaining a genetic diagnosis is difficult because not all cells harbor the variant, and an… read more here.
Keywords: dysplasia mccune; dna; mccune albright; albright syndrome ... See more keywords
Fibrous Dysplasia of Bone and McCune–Albright Syndrome: A Bench to Bedside Review
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DOI: 10.1007/s00223-019-00550-z
Abstract: Fibrous dysplasia is an uncommon mosaic disorder in which bone is replaced by structurally unsound fibro-osseous tissue. It is caused by the sporadic post-zygotic activating mutations in GNAS, resulting in dysregulated GαS-protein signaling in affected… read more here.
Keywords: bone; review; fibrous dysplasia; dysplasia ... See more keywords
Quantifying skeletal burden in fibrous dysplasia using sodium fluoride PET/CT
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DOI: 10.1007/s00259-019-04657-1
Abstract: Purpose To quantify Na 18 F-PET/CT uptake in relation to clinical and biochemical parameters of fibrous dysplasia (FD) severity and healthy bone (HB) metabolism. Secondary aims: comparing normalization for volume of distribution and determining reproducibility… read more here.
Keywords: skeletal burden; bone; burden; fibrous dysplasia ... See more keywords
Malignant transformation of fibrous dysplasia into angiosarcoma
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DOI: 10.1007/s11282-019-00404-2
Abstract: Fibrous dysplasia (FD) is generally considered to be a benign disease that affects the bones, but it has potential to become malignant over time, generally several decades after its initial diagnosis. Radiation therapy can induce… read more here.
Keywords: transformation fibrous; malignant transformation; transformation; fibrous dysplasia ... See more keywords
Improving patient outcomes in fibrous dysplasia/McCune-Albright syndrome: an international multidisciplinary workshop to inform an international partnership
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DOI: 10.1007/s11657-016-0271-6
Abstract: SummaryTo develop consensus on improving the management of patients, we convened an international workshop involving patients, clinicians, and researchers. Key findings included the diagnostic delay and variability in subsequent management with agreement to develop an… read more here.
Keywords: workshop; partnership; dysplasia mccune; fibrous dysplasia ... See more keywords
What Is the Role of Allogeneic Cortical Strut Grafts in the Treatment of Fibrous Dysplasia of the Proximal Femur?
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DOI: 10.1007/s11999-016-4806-3
Abstract: BackgroundManagement of fibrous dysplasia of the proximal femur is a progressive, often recurrent condition of bone that can cause skeletal deformity, fractures, and pain. Allogeneic cortical strut grafting to minimize the risk of fracture or… read more here.
Keywords: surgery; fracture; proximal femur; fibrous dysplasia ... See more keywords
Craniofacial fibrous dysplasia: A 10-case series.
Sign Up to like & getrecommendations! Published in 2017 at "European annals of otorhinolaryngology, head and neck diseases"
DOI: 10.1016/j.anorl.2017.02.004
Abstract: OBJECTIVES Fibrous dysplasia of bone is a rare sporadic benign congenital condition in which normal cancellous bone is replaced by fibro-osseous tissue with immature osteogenesis. Sarcomatous transformation is exceptional. Lesions may involve one bone (monostotic)… read more here.
Keywords: bone; fibrous dysplasia; dysplasia case; dysplasia ... See more keywords
Direct evidence for the age-dependent demise of GNAS-mutated cells in oral fibrous dysplasia.
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DOI: 10.1016/j.archoralbio.2018.05.018
Abstract: OBJECTIVE Fibrous dysplasia (FD) is a benign bone disease characterized by fibro-osseous lesions. FD is caused by somatic mutations in the gene, guanine nucleotide-binding protein, alpha stimulating activity polypeptide 1 (GNAS), which encodes the G… read more here.
Keywords: age dependent; evidence age; mutated cells; fibrous dysplasia ... See more keywords