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Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature
Aim. Fibrous dysplasia is a rare bone disease caused by missense mutation leading to abnormal fibroblast and osteoblast proliferation and increased bone resorption. FD can present in monostotic or polyostotic forms. About 3% of FD could be in association with McCune-Albright syndrome (MAS). Because...
Autores principales: | , , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Hindawi Publishing Corporation
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4480246/ https://www.ncbi.nlm.nih.gov/pubmed/26171261 http://dx.doi.org/10.1155/2015/670809 |
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author | Ozdemir Kutbay, Nilufer Sarer Yurekli, Banu Kartal Baykan, Emine Baydur Sahin, Serap Saygili, Fusun |
author_facet | Ozdemir Kutbay, Nilufer Sarer Yurekli, Banu Kartal Baykan, Emine Baydur Sahin, Serap Saygili, Fusun |
author_sort | Ozdemir Kutbay, Nilufer |
collection | PubMed |
description | Aim. Fibrous dysplasia is a rare bone disease caused by missense mutation leading to abnormal fibroblast and osteoblast proliferation and increased bone resorption. FD can present in monostotic or polyostotic forms. About 3% of FD could be in association with McCune-Albright syndrome (MAS). Because FD is a rare disease, there is limited data in the literature about characteristics of disease and response to treatment. Methods. We present our five cases of FD with general properties and their responses to medical treatment. Results. Two of our patients had polyostotic and three had monostotic FD. One of the polyostotic patients had MAS. One of our patients had surgery for femur fractures, facial asymmetry, and findings of compression. Four patients were given pamidronate; one was given zoledronic acid as bisphosphonate treatment. Bone pain was relieved in all patients with medical treatment. Conclusion. There was a decrease in bone turnover markers to some degree with medical treatment but no radiological improvement was observed. |
format | Online Article Text |
id | pubmed-4480246 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Hindawi Publishing Corporation |
record_format | MEDLINE/PubMed |
spelling | pubmed-44802462015-07-13 Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature Ozdemir Kutbay, Nilufer Sarer Yurekli, Banu Kartal Baykan, Emine Baydur Sahin, Serap Saygili, Fusun Case Rep Endocrinol Case Report Aim. Fibrous dysplasia is a rare bone disease caused by missense mutation leading to abnormal fibroblast and osteoblast proliferation and increased bone resorption. FD can present in monostotic or polyostotic forms. About 3% of FD could be in association with McCune-Albright syndrome (MAS). Because FD is a rare disease, there is limited data in the literature about characteristics of disease and response to treatment. Methods. We present our five cases of FD with general properties and their responses to medical treatment. Results. Two of our patients had polyostotic and three had monostotic FD. One of the polyostotic patients had MAS. One of our patients had surgery for femur fractures, facial asymmetry, and findings of compression. Four patients were given pamidronate; one was given zoledronic acid as bisphosphonate treatment. Bone pain was relieved in all patients with medical treatment. Conclusion. There was a decrease in bone turnover markers to some degree with medical treatment but no radiological improvement was observed. Hindawi Publishing Corporation 2015 2015-06-14 /pmc/articles/PMC4480246/ /pubmed/26171261 http://dx.doi.org/10.1155/2015/670809 Text en Copyright © 2015 Nilufer Ozdemir Kutbay et al. https://creativecommons.org/licenses/by/3.0/ This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. |
spellingShingle | Case Report Ozdemir Kutbay, Nilufer Sarer Yurekli, Banu Kartal Baykan, Emine Baydur Sahin, Serap Saygili, Fusun Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature |
title | Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature |
title_full | Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature |
title_fullStr | Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature |
title_full_unstemmed | Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature |
title_short | Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature |
title_sort | characteristics and treatment results of 5 patients with fibrous dysplasia and review of the literature |
topic | Case Report |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4480246/ https://www.ncbi.nlm.nih.gov/pubmed/26171261 http://dx.doi.org/10.1155/2015/670809 |
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