Multiple epiphyseal dysplasia: clinical and radiographic features, differential diagnosis and molecular basis.

Details

Serval ID
serval:BIB_5D81AAA67EEA
Type
Article: article from journal or magazin.
Publication sub-type
Review (review): journal as complete as possible of one specific subject, written based on exhaustive analyses from published work.
Collection
Publications
Institution
Title
Multiple epiphyseal dysplasia: clinical and radiographic features, differential diagnosis and molecular basis.
Journal
Best Practice and Research. Clinical rheumatology
Author(s)
Unger S., Bonafé L., Superti-Furga A.
ISSN
1521-6942
Publication state
Published
Issued date
03/2008
Peer-reviewed
Oui
Volume
22
Number
1
Pages
19-32
Language
english
Notes
Publication types: Journal Article ; Review
Abstract
Multiple epiphyseal dysplasia is one of the more common skeletal dysplasias but it can still be difficult to diagnose. The presenting signs are often rheumatological ('joint pain') or neurological ('myopathy') in nature, and the cardinal feature of skeletal dysplasia (short stature) may not be present. A radiographic skeletal survey is necessary to delineate the pattern of generalized delayed epiphyseal ossification and changes in epiphyseal contour. Once the diagnosis of multiple epiphyseal dysplasia has been established, careful examination of the radiographs can help to determine which genes should be analysed. Mutations in at least six different genes can cause multiple epiphyseal dysplasia, and it can be either dominant or recessive. Molecular diagnosis is important for accurate prognosis and genetic counselling.
Keywords
Anion Transport Proteins, Collagen Type IX, Extracellular Matrix Proteins, Glycoproteins, Humans, Mutation, Osteochondrodysplasias
Pubmed
Web of science
Create date
11/05/2008 8:29
Last modification date
20/08/2019 14:15
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