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Results for “FIBROUS DYSPLASIA OF BONE”

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At least 37 records · Page 2Linked to original sources
PubMed2006

Bisphosphonate treatment of bone fibrous dysplasia in McCune-Albright syndrome.

One of the main features of McCune-Albright syndrome is bone fibrous dysplasia (BFD) often associated with severe clinical outcomes, such as bone pain, bone deformities and pathological fractures. Medical treatment with bisphosphonates started 15 years ago. Recent trials in pediatric patients with BFD have shown encouraging results. We evaluated long-term efficacy and safety of pamidronate treatment of BFD in children and adolescents with MAS. The drug was administered at 4 month-1 year intervals according to alkaline phosphatase levels. The study included 14 patients (10 females and 4 males between the ages of 5.3 and 18.7 years) with moderate or severe BFD. Follow up lasted 1.9-9 years. Bone pain, fractures, deformities, and bone turnover markers were evaluated before every therapeutic course. The study shows the beneficial effects of long-term bisphosponate treatment on BFD lesions leading to reduced fracture rate and bone pain, and radiological evidence of long bone lesion healing.

Adolescent↗
PubMed1985

[Fibrous bone dysplasia and ossifying bone fibroma in the orbital and periorbital region with special reference to CT].

Fibro-osseous conditions affecting the craniofacial bones pose a complex diagnostic problem. Differentiation between monostotic fibrous dysplasia (FD) and ossifying fibroma (OF) is only possible by correlation of clinical, radiographical and histopathological features. CT was superior to conventional radiography/polytomography in defining exact extent and site of lesions and additional lesions, in verifying aetiology of secondary complications, as well as in depicting lesions and tandem-lesions simulating FD and OF. Density of fibro-osseous conditions was variable due to the ratio of fibrous stroma and metaplastic bone present. Density measurements in FD were 32-695 HU, in immature types of OF, consisting mainly of fibrous and osteoid tissue, 30-250 HU and could reach 690 HU in mature OF, but were definitively lower than normal bone in all our cases. Focal intrinsic nonhomogeneity was more significant in mixed types of FD and immature OF.

Adolescent↗
PubMed1996

Increased IL-6-production by cells isolated from the fibrous bone dysplasia tissues in patients with McCune-Albright syndrome.

McCune-Albright syndrome (MAS) is characterized by café-au-lait spot, multiple endocrine hyperfunction, and polyostotic fibrous dysplasia. A somatic point mutation of Gsalpha protein was reported to decrease GTPase activity, leading to increase in the GSalpha-associated hormone actions via cAMP. IL-6 is known to stimulate osteoclast formation and in the IL-6 promoter, a cAMP responsive element has been identified. In this paper, we investigated the role of IL-6 in the bone lesions of MAS, using the isolated fibrous cells from the polyostotic fibrous dysplasia tissues in bones of the two patients with MAS. Bone biopsy specimen revealed the increased osteoclast in number. In both patients, a GSalpha mutation (Arg201 -> His) was identified in the cultured fibrous cells. Intracellular cAMP content and IL-6 secretion by the patient cells were increased. Rp-8Br-cAMP significantly inhibited IL-6 production in the patient cells, while it had no effect on normal control. The addition of dibutyryl cAMP significantly increased the synthesis of IL-6 in normal control cells. In contrast, no effect of dibutyryl cAMP on IL-6 synthesis was observed in the cells from one of the MAS patients. These data suggest that IL-6 is, at least, one of the downstream effectors of cAMP and that the increased IL-6 synthesis has a pathogenic role in the bone lesions of MAS patients via increasing the number of osteoclasts. These results may provide a new strategy for the therapy of MAS patients.

Base Sequence↗
PubMedDate not supplied

[Fibrous dysplasia of bone].

At hand of 5 clinical and pathological observations, the Authors report on the main features of fibrous Bone Dysplasia. Roentgenological and histological appearances are illustred. In some polyostotic and roentgenologically atypical forms the diagnosis must be histologically confirmed. As for the surgical treatment the Authors state that the result depends considerably on the patient's age.

Adult↗
PubMedDate not supplied

[Fibrous bone dysplasia in the context of a McCune-Albright Syndrome].

Young male patient clinically and radiologically diagnosed of a tumour in IX right rib. A subsequent study with bone scintigraphy showed other areas of focal uptake in the skull, ribs and left trochanterian region. The biopsy of the costal lesion showed a fibrous dysplasia in the clinical context of a McCune-Albright syndrome. The convenience of using complementary imaging techniques is discussed to establish adequate therapeutic behavior.

Adolescent↗