| Mechanisms of premature vascular aging in children with Hutchinson-Gilford progeria syndrome. | |
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MedLine Citation:
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PMID: 22083160 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Hutchinson-Gilford progeria syndrome is a rare, segmental premature aging syndrome of accelerated atherosclerosis and early death from myocardial infarction or stroke. This study sought to establish comprehensive characterization of the fatal vasculopathy in Hutchinson-Gilford progeria syndrome and its relevance to normal aging. We performed cardiovascular assessments at a single clinical site on the largest prospectively studied cohort to date. Carotid-femoral pulse wave velocity was dramatically elevated (mean: 13.00±3.83 m/s). Carotid duplex ultrasound echobrightness, assessed in predefined tissue sites as a measure of arterial wall density, was significantly greater than age- and sex-matched controls in the intima-media (P<0.02), near adventitia (P<0.003), and deep adventitia (P<0.01), as was internal carotid artery mean flow velocity (P<0.0001). Ankle-brachial indices were abnormal in 78% of patients. Effective disease treatments may be heralded by normalizing trends of these noninvasive cardiovascular measures. The data demonstrate that, along with peripheral vascular occlusive disease, accelerated vascular stiffening is an early and pervasive mechanism of vascular disease in Hutchinson-Gilford progeria syndrome. There is considerable overlap with cardiovascular changes of normal aging, which reinforces the view that defining mechanisms of cardiovascular disease in Hutchinson-Gilford progeria syndrome provides a unique opportunity to isolate a subset of factors influencing cardiovascular disease in the general aging population. |
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Authors:
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Marie Gerhard-Herman; Leslie B Smoot; Nicole Wake; Mark W Kieran; Monica E Kleinman; David T Miller; Armin Schwartzman; Anita Giobbie-Hurder; Donna Neuberg; Leslie B Gordon |
Publication Detail:
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Type: Clinical Trial; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't Date: 2011-11-14 |
Journal Detail:
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Title: Hypertension Volume: 59 ISSN: 1524-4563 ISO Abbreviation: Hypertension Publication Date: 2012 Jan |
Date Detail:
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Created Date: 2011-12-16 Completed Date: 2012-02-07 Revised Date: 2013-05-23 |
Medline Journal Info:
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Nlm Unique ID: 7906255 Medline TA: Hypertension Country: United States |
Other Details:
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Languages: eng Pagination: 92-7 Citation Subset: IM |
Affiliation:
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Division of Cardiology, Brigham and Women’s Hospital and Harvard Medical School, Boston, MA, USA. |
| Data Bank Information | |
Bank Name/Acc. No.:
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ClinicalTrials.gov/NCT00425607 |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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Adolescent Aging / physiology* Ankle Brachial Index Blood Flow Velocity / physiology Blood Pressure / physiology Carotid Arteries / physiology Carotid Artery Diseases / epidemiology*, physiopathology*, ultrasonography Carotid Intima-Media Thickness Child Child, Preschool Female Femoral Artery / physiology Humans Male Progeria / epidemiology*, physiopathology*, therapy Prospective Studies Pulsatile Flow / physiology Vascular Stiffness / physiology |
| Grant Support | |
ID/Acronym/Agency:
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M01 RR002172-26/RR/NCRR NIH HHS; MO1-RR02172/RR/NCRR NIH HHS; R21 AG021902/AG/NIA NIH HHS; R21 AG021902-01/AG/NIA NIH HHS; UL1 RR025758/RR/NCRR NIH HHS; UL1 RR025758-01/RR/NCRR NIH HHS; UL1 RR025758-01/RR/NCRR NIH HHS |
| Comments/Corrections | |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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