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Minerva Anestesiologica 2010 February;76(2):151-4

Copyright © 2010 EDIZIONI MINERVA MEDICA

lingua: Inglese

The Hutchinson-Gilford Progeria Syndrome: a case report

Russo-Menna I., Arancibias C.

Department of Anesthesiology, Division of Pediatric Anesthesiology, Virginia Commonwealth University, Richmond,


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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.

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