| Literature DB >> 8533820 |
E M Rosser1, A R Wilkinson, J A Hurst, J M McGaughran, D Donnai.
Abstract
Geleophysic dysplasia is characterized by short stature with short limbs and brachydactyly, a "happy" facial appearance, and joint contractures. Infiltration of heart valves and liver with a mucopolysaccharide-like substance has been demonstrated in some patients. A metabolic pathogenesis is suspected, but has not yet been identified. We report on 3 boys with the condition, 2 of whom are brothers. Serial ultrasound scans were performed on 2 of the cases during pregnancy, but short limbs did not become obvious until after 28 weeks of gestation, making it an uninformative procedure for prenatal diagnosis.Entities:
Mesh:
Year: 1995 PMID: 8533820 DOI: 10.1002/ajmg.1320580304
Source DB: PubMed Journal: Am J Med Genet ISSN: 0148-7299