23 - PROGERIA

Summary

Progeria is a rare condition characterized by premature aging beginning in very early life and invariably ending in premature death. Progeria is caused by a mutation of the LMNA gene on chromosome1q. Based on clinical descriptions and, more recently, genetic analyses, several progeroid syndromes have been defined in addition to progeria itself. The manifestations of these conditions vary, but each has some clinical features that resemble physiologic aging and a variable risk of stroke. Most children with progeria develop premature severe vascular disease. Coronary artery disease and myocardial infarction are common, and heart disease is the leading cause of death. The Wiedemann-Rautenstrauch syndrome, sometimes called neonatal progeria, typically manifests from birth and features delayed development, poor growth, alopecia, and lipoatrophy. Given the severity of large artery pathology in patients with progeroid syndromes, it is probably reasonable to use antiplatelet agents.