Cargando…

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...

Descripción completa

Detalles Bibliográficos
Autores principales: Ozdemir Kutbay, Nilufer, Sarer Yurekli, Banu, Kartal Baykan, Emine, Baydur Sahin, Serap, Saygili, Fusun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Hindawi Publishing Corporation 2015
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
_version_ 1782378125652393984
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
work_keys_str_mv AT ozdemirkutbaynilufer characteristicsandtreatmentresultsof5patientswithfibrousdysplasiaandreviewoftheliterature
AT sareryureklibanu characteristicsandtreatmentresultsof5patientswithfibrousdysplasiaandreviewoftheliterature
AT kartalbaykanemine characteristicsandtreatmentresultsof5patientswithfibrousdysplasiaandreviewoftheliterature
AT baydursahinserap characteristicsandtreatmentresultsof5patientswithfibrousdysplasiaandreviewoftheliterature
AT saygilifusun characteristicsandtreatmentresultsof5patientswithfibrousdysplasiaandreviewoftheliterature