A carregar...
Publicação
Fibrodisplasia óssea e síndrome de McCune - Albright
| Nome: | Descrição: | Tamanho: | Formato: | |
|---|---|---|---|---|
| 167 KB | Adobe PDF |
Orientador(es)
Resumo(s)
Fibrous dysplasia of bone or Jaffe Lischtenstein's disease is a genetic, non-inheritable disease of bone development, characterized by bone pain, deformities and fracture, mainly observed in young adults. The frequency is equal between sexes. Mutations in the gene coding the GSA, GNAS complex, results in osteoblastic differentiation defects, and bone resorption. The disease can have a monostotic or polyostotic form, or be associated with café-au-lait skin spots and precocious puberty (McCune-Albright syndrome). The normal bone and bone marrow is replaced with abnormal benign intramedullary fibro-osseous tissue, and can involve anybone in the body. The vertebral involvement is rare. Radiological and pathological findings can be diagnostic. Biphosphonates and calcium, vitamin D and phosphorus supplements have been used in fibrous dysplasia treatment. Osteosarcoma is a rare, but serious malignant complication. We report the case of a 68 year old woman with a history of hypofisectomy, with a progressive low back pain, without systemic or neurological symptons. The bone scan, the radiographs and the computed tomography findings revealed polyostotic fibrous dysplasia, with vertebral and mandibular involvement. In this paper we compared fibrous dysplasia of bone with Paget bone disease.
Descrição
Palavras-chave
Biphosphonates Fibrous dysplasia of bone McCune-Albright syndrome Osteosarcoma Paget bone disease General Medicine