Shop talk: Sugars, bones, and a disease called multiple hereditary exostoses

On October 29, 2009, researchers and physicians gathered at the Sheraton Four Points Hotel in Boston for 4 days to discuss a disease called multiple hereditary exostoses (MHE). MHE is an autosomal dominant disease that is associated with mutations in two enzymes that are required for heparan sulfate...

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Bibliographic Details
Published inDevelopmental dynamics Vol. 239; no. 6; pp. 1901 - 1904
Main Authors Roehl, Henry H., Pacifici, Maurizio
Format Journal Article Conference Proceeding
LanguageEnglish
Published New York Wiley‐Liss, Inc 01.06.2010
Subjects
bone
Bone and Bones - pathology
Bone Neoplasms - genetics
Bone Neoplasms - surgery
Boston
Carbohydrates - genetics
cartilage
Child
Chondrosarcoma - genetics
exostoses
Exostoses, Multiple Hereditary - genetics
Exostoses, Multiple Hereditary - pathology
Exostoses, Multiple Hereditary - surgery
heparan sulfate
Heparitin Sulfate - genetics
Humans
Mutation
osteochondroma
Osteochondroma - genetics
tumor
Boston
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Summary:On October 29, 2009, researchers and physicians gathered at the Sheraton Four Points Hotel in Boston for 4 days to discuss a disease called multiple hereditary exostoses (MHE). MHE is an autosomal dominant disease that is associated with mutations in two enzymes that are required for heparan sulfate (HS) synthesis. Children with the disease form numerous benign bone tumors (osteochondromas) and have >2% chance of developing chondrosarcoma. The aim of the meeting was to generate new ideas for the diagnoses, treatment, and cure of this disease. Discussions ranged from orthopedic surgical treatment and patients' personal experiences to fundamental questions in skeletal biology and the precise molecular role that HS plays in developmental signaling pathways. Developmental Dynamics 239:1901–1904, 2010. © 2010 Wiley‐Liss, Inc.
Bibliography:Multiple hereditary exostoses (MHE) is also known a hereditary multiple exostoses, osteochondromatosis, or diaphyseal aclasis, depending primarily upon the geographic location. It was renamed to multiple osteochondroma by the World Health Organisation in 2002; however, the renaming remains controversial, and many researchers as well as patients and their families prefer to use the original designation. This issue was discussed at the Third International MHE Research Conference, but a consensus was unfortunately not reached.
ObjectType-Article-2
content type line 25
ObjectType-Conference-1
SourceType-Conference Papers & Proceedings-1
ISSN:1058-8388
1097-0177
DOI:10.1002/dvdy.22290