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-matched healthy children, and 14 older healthy volunteers were evaluated by three-dimensional echocardiography (including strain analysis) and carotid elasticity examination with the echo-tracking technique.
Results: Children with HGPS had higher left ventricular ejection fraction (LVEF) and global longitudinal strain, when compared to older healthy volunteers ( < 0.05). However, these parameters were not significantly different, when compared to those in healthy children. Furthermore, children with HGPS had lower average peak times in the left ventricle, when compared with the other two groups. For the structure of the carotid artery detected by ultrasound, the abnormality rates were similar between children with HGPS and older healthy volunteers (83.3% 71.4%). The elastic parameters, elastic modulus, stiffness parameter, and pulsed wave transmittal velocity of children with HGPS were lower, when compared to those in older healthy volunteers ( < 0.05), while they were higher with arterial compliance ( > 0.05). Furthermore, no significant difference existed among the vascular elastic parameters between HGPS and normal children.
Conclusion: HGPS children had impaired left ventricular (LV) synchrony, when compared to normal children, although the difference in LVEF was not statistically significant. Furthermore, the structural abnormality of the carotid artery in HGPS children was similar to that in older people, although the index of elasticity appears to be more favorable. These results suggest that the cardiovascular system in HGPS children differs from natural aging.
Download full-text PDF |
Source |
---|---|
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7180498 | PMC |
http://dx.doi.org/10.1155/2020/9631851 | DOI Listing |
Br J Hosp Med (Lond)
November 2024
Department of Hearing Center, Children's Hospital of Zhejiang University School of Medicine, Clinical Research Center for Child Health, Hangzhou, Zhejiang, China.
Few hearing loss studies have been conducted in patients with progeria, and only the possibility of low-frequency conductive hearing loss has been mentioned. The primary objective of this study is to perform a comprehensive analysis of the clinical audiological characteristics of children with Hutchinson-Gilford progeria syndrome (HGPS), and the secondary objective is to analyse the causes of their hearing loss and what can be done to enable them to hear as well as possible. Ten children with HGPS underwent impedance audiometry (tympanogram), otoacoustic emissions, and pure-tone audiometry tests.
View Article and Find Full Text PDFFront Physiol
November 2024
Musculoskeletal Translational Innovation Initiative, Carl J. Shapiro Department of Orthopaedic Surgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, United States.
Introduction: Hutchinson-Gilford Progeria Syndrome (HGPS) is a rare genetic condition characterized by premature aging, impacting multiple organ systems, including cardiovascular, musculoskeletal, and integumentary. Significant abnormalities in a transgenic mouse model (homozygous G608G mutation), specifically targeting the development of skull and facial bone indices through high-resolution CT scanning and cephalometric analysis.
Methods: Key measurements include bone thickness, skull volume, and cranial suture integrity.
Aging Cell
October 2024
Department of Cell Biology and Molecular Genetics, University of Maryland, College Park, Maryland, USA.
Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disorder in children caused by a point mutation in the lamin A gene, resulting in a toxic form of lamin A called progerin. Accelerated atherosclerosis leading to heart attack and stroke are the major causes of death in these patients. Endothelial cell (EC) dysfunction contributes to the pathogenesis of HGPS related cardiovascular diseases (CVD).
View Article and Find Full Text PDFProc Natl Acad Sci U S A
October 2024
The Key Laboratory of Cell Proliferation and Differentiation of the Ministry of Education, College of Life Sciences, Peking University, Beijing 100871, China.
A A Pract
August 2024
From the Departments of Anesthesiology, Critical Care and Pain Medicine.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!