| Literature DB >> 30195254 |
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.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