Figure - available from: BioMed Research International
This content is subject to copyright. Terms and conditions apply.
Source publication
Background:
The cardiovascular characteristics of children with Hutchinson-Gilford progeria syndrome (HGPS) remain unclear. The present study is aimed at evaluating the cardiovascular changes with ultrasound examination in children with HGPS and compared these with those in normal children and older people.
Methods:
Seven HGPS children, 21 age-m...
Similar publications
Background and Aims: Multiple studies demonstrated that non-alcoholic fatty liver disease (NAFLD) is associated with several structural and functional cardiovascular complications. The aim of this systematic review is to evaluate subclinical left ventricular (LV) systolic dysfunction in NAFLD assessed with myocardial strain measured by speckle trac...
Citations
... Interestingly, increased echobrightness was not associated with age, suggesting that extracellular matrix (ECM) remodeling may be an early alteration potentially underlying the development of the cardiac phenotype. Another recent study [37] applied three-dimensional echocardiography (speckled tracking imaging) to analyze seven HGPS patients (five children ≤ 8 years of age and two children in their teens), 21 aged-matched healthy children, and 14 older healthy volunteers (mean age: 65.7 ± 7.5). Despite the fact that most HGPS patients were in their first decade of life, the authors found one case of diastolic dysfunction and another of aortic valve calcification, severe aortic stenosis, and LV hypertrophy, in agreement with some of the findings reported by Prakash et al. [30]. ...
Hutchinson-Gilford progeria syndrome (HGPS) is a rare genetic disease that recapitulates many symptoms of physiological aging and precipitates death. Patients develop severe vascular alterations, mainly massive vascular smooth muscle cell loss, vessel stiffening, calcification, fibrosis, and generalized atherosclerosis, as well as electrical, structural, and functional anomalies in the heart. As a result, most HGPS patients die of myocardial infarction, heart failure, or stroke typically during the first or second decade of life. No cure exists for HGPS, and therefore it is of the utmost importance to define the mechanisms that control disease progression in order to develop new treatments to improve the life quality of patients and extend their lifespan. Since the discovery of the HGPS-causing mutation, several animal models have been generated to study multiple aspects of the syndrome and to analyze the contribution of different cell types to the acquisition of the HGPS-associated cardiovascular phenotype. This review discusses current knowledge about cardiovascular features in HGPS patients and animal models and the molecular and cellular mechanisms through which progerin causes cardiovascular disease.
Cardiovascular complications are the most frequent cause of death in patients with the Hutchinson – Gilford progeria syndrome. However, due to its rarity, studying the course of cardiac abnormalities has been a challenge. The cardiovascular phenotype helps to provide greater insight into the natural history of these abnormalities.
