Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget
Autor(a) principal: | |
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Data de Publicação: | 2010 |
Outros Autores: | , |
Tipo de documento: | Artigo |
Idioma: | por |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | http://hdl.handle.net/10362/23189 |
Resumo: | 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. |
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Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de pagetFibrous dysplasia and McCune-Albright syndrome: case-report and differential diagnose with Paget Bone DiseaseFibrousDysplasiaDiseaselesionsspineSyndromeBiphosphonatesPagetOsteosarcomaMcCune-AlbrightofcystBoneFibrodisplasia ósseaSíndrome McCune-AlbrightBifosfonatosOsteosarcomaDoença Óssea de PagetFi brous Dyspla sia of BoneMcCu ne-Al - bright Syndro meBip hospho na tesOs te o sar co maPa get Bone Di se a seFibrous 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.NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM)RUNSilva, IMateus, MBranco, Jaime2017-09-12T22:01:15Z2010-102010-10-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/article7application/pdfhttp://hdl.handle.net/10362/23189por0303-464XPURE: 458629info:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2024-03-11T04:11:24Zoai:run.unl.pt:10362/23189Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-20T03:27:41.600691Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget Fibrous dysplasia and McCune-Albright syndrome: case-report and differential diagnose with Paget Bone Disease |
title |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget |
spellingShingle |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget Silva, I Fibrous Dysplasia Disease lesions spine Syndrome Biphosphonates Paget Osteosarcoma McCune-Albright of cyst Bone 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 |
title_short |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget |
title_full |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget |
title_fullStr |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget |
title_full_unstemmed |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget |
title_sort |
Fibrodisplasia óssea e síndrome de Mccune-albright: Descrição de um caso clínico e diagnóstico diferencial com doença óssea de paget |
author |
Silva, I |
author_facet |
Silva, I Mateus, M Branco, Jaime |
author_role |
author |
author2 |
Mateus, M Branco, Jaime |
author2_role |
author author |
dc.contributor.none.fl_str_mv |
NOVA Medical School|Faculdade de Ciências Médicas (NMS|FCM) RUN |
dc.contributor.author.fl_str_mv |
Silva, I Mateus, M Branco, Jaime |
dc.subject.por.fl_str_mv |
Fibrous Dysplasia Disease lesions spine Syndrome Biphosphonates Paget Osteosarcoma McCune-Albright of cyst Bone 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 |
topic |
Fibrous Dysplasia Disease lesions spine Syndrome Biphosphonates Paget Osteosarcoma McCune-Albright of cyst Bone 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 |
description |
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. |
publishDate |
2010 |
dc.date.none.fl_str_mv |
2010-10 2010-10-01T00:00:00Z 2017-09-12T22:01:15Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
http://hdl.handle.net/10362/23189 |
url |
http://hdl.handle.net/10362/23189 |
dc.language.iso.fl_str_mv |
por |
language |
por |
dc.relation.none.fl_str_mv |
0303-464X PURE: 458629 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
7 application/pdf |
dc.source.none.fl_str_mv |
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Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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RCAAP |
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RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
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Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799137904390307840 |