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Early Clinical and Radiographic Characteristics in Fibrodysplasia Ossificans ProgressivaA Report of Two Cases
Kenichi Mishima, MD1; Hiroshi Kitoh, MD1; Takenobu Katagiri, PhD2; Hiroshi Kaneko, MD1; Naoki Ishiguro, MD1
1 Department of Orthopaedic Surgery, Nagoya University School of Medicine, 65 Tsurumai-Cho, Showa-ku, Nagoya, Aichi 466-8550, Japan. E-mail address for H. Kitoh: hkitoh@med.nagoya-u.ac.jp
2 Division of Pathophysiology, Research Center for Genomic Medicine, Saitama Medical University, 1397-1 Yamane, Hidaka-shi, Saitama 350-1241, Japan
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Disclosure: In support of their research for or preparation of this work, one or more of the authors received, in any one year, outside funding or grants in excess of $10,000 from Japan Health and Labour Science Research. Neither they nor a member of their immediate families received payments or other benefits or a commitment or agreement to provide such benefits from a commercial entity.

Investigation performed at the Department of Orthopaedic Surgery, Nagoya University School of Medicine, Nagoya, Japan

Copyright © 2011 by The Journal of Bone and Joint Surgery, Inc.
J Bone Joint Surg Am, 2011 May 18;93(10):e52 1-5. doi: 10.2106/JBJS.J.01443
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Extract

Fibrodysplasia ossificans progressiva is an extremely rare and disabling genetic condition characterized by progressive heterotopic ossification of soft tissues such as muscles, ligaments, tendons, fasciae, and aponeuroses. The prevalence of fibrodysplasia ossificans progressiva is estimated to be about one in 2 million individuals1. Heterotopic ossification in fibrodysplasia ossificans progressiva usually begins in the first decade of life with the episodic development of inflammatory fibroproliferative masses in the axial skeleton. Most patients with fibrodysplasia ossificans progressiva are misdiagnosed as having soft-tissue sarcoma or aggressive juvenile fibromatosis before the definitive appearance of heterotopic ossification and undergo invasive procedures that usually lead to the acceleration of ossification2. Early correct diagnosis of fibrodysplasia ossificans progressiva is necessary to prevent additional iatrogenic harm or trauma.
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    These activities have been planned and implemented in accordance with the Essential Areas and policies of the Accreditation Council for Continuing Medical Education (ACCME) through the joint sponsorship of the American Academy of Orthopaedic Surgeons and The Journal of Bone and Joint Surgery, Inc. The American Academy of Orthopaedic Surgeons is accredited by the ACCME to provide continuing medical education for physicians.
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