Kemiğin fibröz displazisi ve McCune-Albright sendromu: bir olgu sunumu ve literatürün gözden geçirilmesi
Abstract
Özet
Kemiğin Fibröz Displazisi (FD) kemikte ağrı, deformite ve kırığa neden olabilen, genetik, kalıtsal olmayan bir hastalıktır.Nadir görülen McCune-Albright sendromunda (MAS) ise erken puberte ve diğer endokrin anormallikler, “cafe´-au-lait” diye adlandırılan kahverengi lekeler ve FD bulunur. Göğüs ağrısı, erken puberte, “cafe´-au-lait” lekeleri ve tomografide saptanan kosta destrüktif lezyonları olan 32 yaşında kadın hasta sunulmaktadır. Biyopsi FD olduğunu ortaya koydu. Hasta MAS tanısı aldı. Literatür eşliğinde olgu sunulmaktadır.
Anahtar sözcükler: Fibröz displazi, McCune-Albright sendromu, endokrinopati
Abstract
Fibrous dysplasia (FD) of bone is a genetic, non-inheritable disease that can cause bone pain, bone deformities and fracture. Early puberty, other endocrinic abnormalities, FD, and skin lesiona called "cafe-au-lait" spots are associated with rare McCune-Albright syndrome (MAS). A 32 yers-old women with chest pain, early puberty, cafe-au-lai skin lesions, dectructive lesions of the costae on tomography is raported. The biopsy revealed FD. The patient had a diagnosis of MAS. The case is presented with a literature review.
Keywords: Fibrous dysplasia, McCune-Albright syndrome, endocrinopathy
Keywords
References
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Details
Primary Language
English
Subjects
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Journal Section
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Publication Date
March 14, 2011
Submission Date
April 14, 2010
Acceptance Date
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Published in Issue
Year 1970 Volume: 33 Number: 1