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

Détails

ID Serval
serval:BIB_5D81AAA67EEA
Type
Article: article d'un périodique ou d'un magazine.
Sous-type
Synthèse (review): revue aussi complète que possible des connaissances sur un sujet, rédigée à partir de l'analyse exhaustive des travaux publiés.
Collection
Publications
Institution
Titre
Multiple epiphyseal dysplasia: clinical and radiographic features, differential diagnosis and molecular basis.
Périodique
Best Practice and Research. Clinical rheumatology
Auteur⸱e⸱s
Unger S., Bonafé L., Superti-Furga A.
ISSN
1521-6942
Statut éditorial
Publié
Date de publication
03/2008
Peer-reviewed
Oui
Volume
22
Numéro
1
Pages
19-32
Langue
anglais
Notes
Publication types: Journal Article ; Review
Résumé
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.
Mots-clé
Anion Transport Proteins, Collagen Type IX, Extracellular Matrix Proteins, Glycoproteins, Humans, Mutation, Osteochondrodysplasias
Pubmed
Web of science
Création de la notice
11/05/2008 9:29
Dernière modification de la notice
20/08/2019 15:15
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