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Fibrodisplasia óssea e síndrome de McCune - Albright

2010-10Artigo científico Acesso aberto
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dc.contributor.authorSilva, I.
dc.contributor.authorMateus, M.
dc.contributor.authorBranco, J. C.
dc.contributor.authorBranco, Jaime
dc.contributor.institutionNOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)
dc.contributor.pblSociedade Portuguesa de Reumatologia
dc.date.accessioned2025-10-31T21:58:57Z
dc.date.available2025-10-31T21:58:57Z
dc.date.issued2010-10
dc.description.abstractFibrous 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.en
dc.description.versionpublishersversion
dc.description.versionpublished
dc.format.extent7
dc.format.extent171008
dc.identifier.issn0303-464X
dc.identifier.otherPURE: 134605279
dc.identifier.otherPURE UUID: 97526c26-b42e-472d-ac33-5831e1ef2227
dc.identifier.otherScopus: 79251526532
dc.identifier.otherPubMed: 21245817
dc.identifier.otherWOS: 000286158600010
dc.identifier.urihttp://hdl.handle.net/10362/189956
dc.identifier.urlhttps://www.scopus.com/pages/publications/79251526532
dc.identifier.urlhttps://www.arprheumatology.com/oldsite/conteudo/pdfs/13._CC_-_Fibrodisplasia_ARP2010-68CC.pdf
dc.language.isopor
dc.peerreviewedyes
dc.subjectBiphosphonates
dc.subjectFibrous dysplasia of bone
dc.subjectMcCune-Albright syndrome
dc.subjectOsteosarcoma
dc.subjectPaget bone disease
dc.subjectGeneral Medicine
dc.titleFibrodisplasia óssea e síndrome de McCune - Albrightpt
dc.title.alternativeFibrous dysplasia and McCune-Albright syndromeCase-report and differential diagnose with paget bone diseaseen
dc.title.subtitleDescrição de um caso clínico e diagnóstico diferencial com doença óssea de pagetpt
dc.typejournal article
degois.publication.firstPage497
degois.publication.issue4
degois.publication.lastPage503
degois.publication.titleActa Reumatologica Portuguesa
degois.publication.volume35
dspace.entity.typePublication
person.familyNameBranco
person.givenNameJaime
person.identifier.ciencia-idB612-E5C3-60DC
person.identifier.orcid0000-0001-7024-4375
person.identifier.scopus-author-id8417815400
rcaap.rightsopenAccess
relation.isAuthorOfPublication4caea9f3-40dd-4c95-98ec-0120509287c0
relation.isAuthorOfPublication.latestForDiscovery4caea9f3-40dd-4c95-98ec-0120509287c0

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