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Glycobiology and the Growth Plate: Current Concepts in Multiple Hereditary Exostoses

Jones, Kevin B. MD

Journal of Pediatric Orthopaedics: July/August 2011 - Volume 31 - Issue 5 - p 577–586
doi: 10.1097/BPO.0b013e31821c7738
Tumors

Background: Multiple hereditary exostoses, also termed as multiple osteochondromas, is a heritable disorder of connective tissue with primarily orthopaedic clinical manifestations. Understanding of its biological underpinnings has been advanced on a variety of fronts in recent years.

Methods: The multifaceted literature regarding osteochondromagenesis and the major clinical challenges in patients with multiple osteochondromas were reviewed.

Results: Consideration of recent advances in molecular biology, biochemistry, and animal modeling of osteochondroma pathogenesis yields a unified model.

Conclusions: Mechanistic details and therapeutic targets have yet to be elucidated, but the general biology of osteochondroma formation is increasingly clear, as well as its implications in the orthopaedic clinical setting.

Department of Orthopaedic Surgery, Center for Children's Cancer Research, Huntsman Cancer Institute, Primary Children's Medical Center, University of Utah, Salt Lake City, UT

Funding: The author is currently supported by the National Cancer Institute (National Institutes of Health) K08-CA138764 and was supported by the Orthopaedic Research and Education Foundation Resident Research Grant for Growth Factor Research 2003, related to the topic of this review.

Reprints: Kevin B. Jones, MD, 2000 Circle of Hope Drive, Room 4263, Huntsman Cancer Institute, University of Utah, Salt Lake City, UT 84112. e-mail: kevin.jones@hci.utah.edu.

Disclosures: Neither the author, nor any member of his family has any financial relationship of any kind with any corporate entity involved in medicine, orthopaedic surgery, or their related products.

Copyright © 2011 Wolters Kluwer Health, Inc. All rights reserved.