| Literature DB >> 24039088 |
Allen L Pimienta1, William R Wilcox, Eyal Reinstein.
Abstract
The criteria for diagnosing and distinguishing between Weill-Marchesani syndrome (WMS) and geleophysic dysplasia (GD) are inexact and often overlap. We report the clinical findings and evolving phenotype for a period of 18 years in a patient whose diagnosis, and distinguishing characteristics, transformed from GD to WMS. Molecular testing demonstrated novel mutations in the ADAMTS10 gene confirming a diagnosis of autosomal recessive WMS in the proposita. We further report on phenotypic features not classically linked to WMS. These findings indicate that the Weill-Marchesani phenotype may be developed and is not always apparent in early childhood.Entities:
Keywords: Weill-Marchesani syndrome; evolving phenotype; geleophysic dysplasia
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Year: 2013 PMID: 24039088 DOI: 10.1002/ajmg.a.36161
Source DB: PubMed Journal: Am J Med Genet A ISSN: 1552-4825 Impact factor: 2.802