Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget

Translated title of the contribution: Fibrous dysplasia and McCune-Albright syndrome: case-report and differential diagnose with Paget Bone Disease

I Silva, M Mateus, Jaime Branco

Research output: Contribution to journalArticle

Abstract

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 Gs alpha, GNAS complex, results in osteoblastic differentiation defects, and bone resorption. The disease can have a monostotic or polyostotic form, or be associated with cafe-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 any bone 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.
Original languagePortuguese
Pages (from-to)497-503
Number of pages7
JournalActa reumatologica portuguesa
Volume35
Issue number4
Publication statusPublished - Oct 2010

Keywords

  • Fibrodisplasia óssea
  • Síndrome McCune-Albright
  • Bifosfonatos
  • Osteosarcoma
  • Doença Óssea de Paget
  • Fi brous Dyspla sia of Bone
  • McCu ne-Al - bright Syndro me
  • Bip hospho na tes
  • Os te o sar co ma
  • Pa get Bone Di se a se

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