Literature DB >> 30195254

Geleophysic dysplasia: 48 year clinical update with emphasis on cardiac care.

Janet M Legare1, Peggy Modaff1, Samuel P Strom2, Richard M Pauli1, Heather L Bartlett1,3.   

Abstract

Geleophysic dysplasia is a rare skeletal dysplasia often complicated by progressive cardiac disease. Information about long-term outcomes is limited. A clinical update of the oldest surviving patient described with geleophysic dysplasia type 1 is provided. Special note is made in relation to the cardiac disease and interventions. Genetic testing of ADAMTSL2 revealed a previously reported missense mutation as well as a novel nonsense mutation, which can be added to the list of causative mutations in geleophysic dysplasia.
© 2018 Wiley Periodicals, Inc.

Entities:  

Keywords:  ADAMTSL2; GD1; TAVR; aortic aneurysm; aortic stenosis; geleophysic dysplasia

Mesh:

Year:  2018        PMID: 30195254     DOI: 10.1002/ajmg.a.40377

Source DB:  PubMed          Journal:  Am J Med Genet A        ISSN: 1552-4825            Impact factor:   2.802


  4 in total

Review 1.  The fibrillinopathies: New insights with focus on the paradigm of opposing phenotypes for both FBN1 and FBN2.

Authors:  Silke Peeters; Pauline De Kinderen; Josephina A N Meester; Aline Verstraeten; Bart L Loeys
Journal:  Hum Mutat       Date:  2022-04-28       Impact factor: 4.700

2.  Congenital polyvalvular disease expands the cardiac phenotype of the RASopathies.

Authors:  Dena R Matalon; David A Stevenson; Elizabeth J Bhoj; Avni B Santani; Beth Keena; Meryl S Cohen; Angela E Lin; Sarah E Sheppard; Elaine H Zackai
Journal:  Am J Med Genet A       Date:  2021-03-08       Impact factor: 2.802

Review 3.  ADAMTS Proteins and Vascular Remodeling in Aortic Aneurysms.

Authors:  Zakaria Mougin; Julia Huguet Herrero; Catherine Boileau; Carine Le Goff
Journal:  Biomolecules       Date:  2021-12-22

4.  Pathogenic variants in THSD4, encoding the ADAMTS-like 6 protein, predispose to inherited thoracic aortic aneurysm.

Authors:  Sandy Elbitar; Marjolijn Renard; Catherine Boileau; Marianne Abifadel; Pauline Arnaud; Nadine Hanna; Marie-Paule Jacob; Dong-Chuan Guo; Ko Tsutsui; Marie-Sylvie Gross; Ketty Kessler; Laurent Tosolini; Vincenzo Dattilo; Sebastien Dupont; Jeremie Jonquet; Maud Langeois; Louise Benarroch; Melodie Aubart; Youmna Ghaleb; Yara Abou Khalil; Mathilde Varret; Petra El Khoury; Benoit Ho-Tin-Noé; Yves Alembik; Sébastien Gaertner; Bertrand Isidor; Laurent Gouya; Olivier Milleron; Kiyotoshi Sekiguchi; Dianna Milewicz; Julie De Backer; Carine Le Goff; Jean-Baptiste Michel; Guillaume Jondeau; Lynn Y Sakai
Journal:  Genet Med       Date:  2020-08-28       Impact factor: 8.822

  4 in total

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