Literature DB >> 24039088

More than meets the eye: The evolving phenotype of Weill-Marchesani syndrome-diagnostic confusion with geleophysic dysplasia.

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.
© 2013 Wiley Periodicals, Inc.

Entities:  

Keywords:  Weill-Marchesani syndrome; evolving phenotype; geleophysic dysplasia

Mesh:

Substances:

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


  4 in total

1.  Extended-depth spectral-domain optical coherence tomography imaging of the crystalline lens in Weill-Marchesani-like syndrome.

Authors:  Florence Cabot; Marco Ruggeri; Hady Saheb; Jean-Marie Parel; Richard K Parrish
Journal:  JCRS Online Case Rep       Date:  2014-10

2.  Three novel mutations of the FBN1 gene in Chinese children with acromelic dysplasia.

Authors:  Yu Wang; Huiwen Zhang; Jun Ye; Lianshu Han; Xuefan Gu
Journal:  J Hum Genet       Date:  2014-08-21       Impact factor: 3.172

3.  ADAMTS10 inhibits aggressiveness via JAK/STAT/c-MYC pathway and reprograms macrophage to create an anti-malignant microenvironment in gastric cancer.

Authors:  Junyi Zhou; Tuoyang Li; Hao Chen; Yingming Jiang; Yandong Zhao; Jintuan Huang; Zijian Chen; Xiaocheng Tang; Zhenze Huang; Zuli Yang
Journal:  Gastric Cancer       Date:  2022-08-04       Impact factor: 7.701

4.  Case report: A homozygous ADAMTSL2 missense variant causes geleophysic dysplasia with high similarity to Weill-Marchesani syndrome.

Authors:  Mojiang Li; Yingshu Li; Huixing Liu; Haiyan Zhou; Wanqin Xie; Qinghua Peng
Journal:  Front Genet       Date:  2022-09-28       Impact factor: 4.772

  4 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.