Skip to main content Accessibility help
×
Hostname: page-component-cd9895bd7-lnqnp Total loading time: 0 Render date: 2024-12-23T18:12:08.926Z Has data issue: false hasContentIssue false

23 - PROGERIA

from PART II: - HEREDITARY AND GENETIC CONDITIONS AND MALFORMATIONS

Published online by Cambridge University Press:  06 January 2010

Louis R. Caplan
Affiliation:
Beth Israel Deaconess Medical Center, Boston
Julien Bogousslavsky
Affiliation:
Valmont Clinique, Glion, Switzerland
Get access

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.
Type
Chapter
Information
Publisher: Cambridge University Press
Print publication year: 2008

Access options

Get access to the full version of this content by using one of the access options below. (Log in options will check for institutional or personal access. Content may require purchase if you do not have access.)

Save book to Kindle

To save this book to your Kindle, first ensure [email protected] is added to your Approved Personal Document E-mail List under your Personal Document Settings on the Manage Your Content and Devices page of your Amazon account. Then enter the ‘name’ part of your Kindle email address below. Find out more about saving to your Kindle.

Note you can select to save to either the @free.kindle.com or @kindle.com variations. ‘@free.kindle.com’ emails are free but can only be saved to your device when it is connected to wi-fi. ‘@kindle.com’ emails can be delivered even when you are not connected to wi-fi, but note that service fees apply.

Find out more about the Kindle Personal Document Service.

Available formats
×

Save book to Dropbox

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Dropbox.

Available formats
×

Save book to Google Drive

To save content items to your account, please confirm that you agree to abide by our usage policies. If this is the first time you use this feature, you will be asked to authorise Cambridge Core to connect with your account. Find out more about saving content to Google Drive.

Available formats
×