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By David Bennett Pediatric Orthopaedic Surgeon in the United States
Julianne Huegel, Federica Sgariglia, Motomi Enomoto-Iwamoto, Eiki Koyama, John P Dormans, Maurizio Pacifici
Heparan sulfate (HS) is an essential component of cell surface and matrix-associated proteoglycans. Due to their sulfation patterns, the HS chains interact with numerous signaling proteins and regulate their distribution and activity on target cells. Many of these proteins, including bone morphogenetic protein family members, are expressed in the growth plate of developing skeletal elements, and several skeletal phenotypes are caused by mutations in those proteins as well as in HS-synthesizing and modifying enzymes...
September 2013: Developmental Dynamics: An Official Publication of the American Association of Anatomists
Martin Rupprecht, Alexander S Spiro, Carsten Schlickewei, Sandra Breyer, Karsten Ridderbusch, Ralf Stücker
BACKGROUND: Temporary screw epiphysiodesis of the distal tibia is employed to correct ankle valgus deformity in patients with a wide spectrum of underlying etiologies. For patients with hereditary multiple exostosis it is unclear whether a rebound phenomenon may play a role after screw removal (SR) and successful management of ankle valgus deformity. METHODS: From January 2002 to July 2013, 10 boys and 2 girls with HME and an ankle valgus deformity were included in this study...
January 2015: Journal of Pediatric Orthopedics
Saravanaraja Muthusamy, Sheila A Conway, H Thomas Temple
General orthopedic surgeons frequently encounter patients with conditions affecting multiple bones. It is important to recognize common polyostotic diseases. This article describes five polyostotic conditions: Multipe Enchondromatosis (Ollier Disease and Maffucci syndrome), Multiple Hereditary Exostosis (Diaphyseal Aclasis), Fibrous Dysplasia (McCune-Albright syndrome and Mazabraud syndrome), Paget's Disease of bone (Osteitis Deformans), and Skeletal Metastases. This is a survey of the clinical, pathologic and radiographic features that assist in diagnosing these conditions...
July 2014: Orthopedic Clinics of North America
N D Clement, D E Porter
BACKGROUND: There is a high rate of forearm deformity in patients with hereditary multiple exostoses, with many patients developing radial head dislocation associated with ulnar shortening. METHODS: One hundred and six patients with hereditary multiple exostoses who were fifteen years of age or older were identified with use of a previously compiled database. An independent observer measured flexion and extension of the elbow and wrist as well as supination and pronation of the forearm and recorded the number of exostoses affecting the forearm...
September 4, 2013: Journal of Bone and Joint Surgery. American Volume
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