Camurati-Engelmann Disease: A Late And Rare Sporadic Case With Vertebral And Pelvic Involvement

Ahmet Mete; Çağatay Andıç; Ayhan Özkur; Eda Parlak

doi:10.58600/eurjther.2009-15-1-1269-arch

Authors

Ahmet Mete Gaziantep University School Of Medicine Department Of Radiology
Çağatay Andıç Gaziantep University School Of Medicine Department Of Radiology
Ayhan Özkur Gaziantep University School Of Medicine Department Of Radiology
Eda Parlak Gaziantep University School Of Medicine Department Of Radiology

DOI:

https://doi.org/10.58600/eurjther.2009-15-1-1269-arch

Keywords:

Camurati-Engelmann disease, Bone dysplasia, Hyperostosis

Abstract

Camurati-Engelmann hastalığı (KED) veya ilerleyici diyafiz displazisi, nadir, otozomal dominant kalıtsal bir kemik hastalığıdır. Ağırlıklı olarak uzun kemiklerin diyafizlerini etkileyen ilerleyici genişleme ve skleroz bu hastalığın karakteristik özellikleridir. Vertebral ve göğüs kafesi tutulumu sadece en ağır vakalarda görülür. Bu olgu, vertebral cisimlerin, kaburgaların ve pelvisin aynı anda belirgin şekilde tutulması, şiddetli formu ve sporadik prezentasyonu nedeniyle ilgi çekicidir. Ayrıca literatürde bu kadar yaygın bir katılım içeren bir rapor bulamadık.

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