The Hutchinson-Gilford Progeria Syndrome: a case report
Russo-Menna, I.; Arancibias, C.
Minerva Anestesiologica 76(2): 151-154
2010
ISSN/ISBN: 0375-9393 PMID: 20150858 Document Number: 644451
The HGPS (Hutchinson Gilford Progeria Syndrome) is a rare genetic disorder with an incidence of 1 per 8 million live births. Originally described in 1886, less than 100 cases have been reported. The syndrome is characterized by premature aging with changes in many organs. The diagnosis is usually made by age 2, the mean survival age is 13.4 years and the most common cause of death is myocardial infarction. Recent genetic advances have identified the cause as a defect in the LMNA gene of chromosome 1.