Cardiovascular Pathology in Hutchinson-Gilford Progeria: Correlation With the Vascular Pathology of Aging

摘要: Objective— Children with Hutchinson-Gilford progeria syndrome (HGPS) exhibit dramatically accelerated cardiovascular disease (CVD), causing death from myocardial infarction or stroke between the ages of 7 and 20 years. We undertook first histological comparative evaluation genetically confirmed HGPS CVD aging. Methods Results— present structural immunohistological analysis tissues 2 children who died infarction. Both had features classically associated atherosclerosis aging, as well arteriolosclerosis small vessels. In addition, vessels exhibited prominent adventitial fibrosis, a previously undescribed feature HGPS. Importantly, although progerin was detected at higher rates in coronary arteries, it also non-HGPS individuals. Between 1 month 97 years, staining increased an average 3.34% per year ( P Conclusion— find concordance among many aspects pathology both geriatric patients. generates more fibrosis than typical CVD. Vascular generation young individuals, which significantly increases throughout life, strongly suggests that has role aging general population.