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...

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Main Authors: Nilufer Ozdemir Kutbay, Banu Sarer Yurekli, Emine Kartal Baykan, Serap Baydur Sahin, Fusun Saygili
Format: Article
Language:English
Published: Wiley 2015-01-01
Series:Case Reports in Endocrinology
Online Access:http://dx.doi.org/10.1155/2015/670809
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author Nilufer Ozdemir Kutbay
Banu Sarer Yurekli
Emine Kartal Baykan
Serap Baydur Sahin
Fusun Saygili
author_facet Nilufer Ozdemir Kutbay
Banu Sarer Yurekli
Emine Kartal Baykan
Serap Baydur Sahin
Fusun Saygili
author_sort Nilufer Ozdemir Kutbay
collection DOAJ
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.
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institution Kabale University
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series Case Reports in Endocrinology
spelling doaj-art-435c99df01824b0fb226e0529c2ad92a2025-02-03T01:24:20ZengWileyCase Reports in Endocrinology2090-65012090-651X2015-01-01201510.1155/2015/670809670809Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the LiteratureNilufer Ozdemir Kutbay0Banu Sarer Yurekli1Emine Kartal Baykan2Serap Baydur Sahin3Fusun Saygili4Endocrinology Department, Ege University Faculty of Medicine, Izmir, TurkeyEndocrinology Department, Ege University Faculty of Medicine, Izmir, TurkeyEndocrinology Department, Ege University Faculty of Medicine, Izmir, TurkeyEndocrinology Department, Recep Tayyip Erdogan University Faculty of Medicine, Rize, TurkeyEndocrinology Department, Ege University Faculty of Medicine, Izmir, TurkeyAim. 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.http://dx.doi.org/10.1155/2015/670809
spellingShingle Nilufer Ozdemir Kutbay
Banu Sarer Yurekli
Emine Kartal Baykan
Serap Baydur Sahin
Fusun Saygili
Characteristics and Treatment Results of 5 Patients with Fibrous Dysplasia and Review of the Literature
Case Reports in Endocrinology
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
url http://dx.doi.org/10.1155/2015/670809
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