Evaluation of aortic elasticity properties in mucopolysaccharidosis patients; effect of enzyme replacement therapy (ERT) on aortic stiffness.
Kerem ErtaşOzlem GulAyse Ergul BozaciHuseyin BilginPublished in: Journal of pediatric endocrinology & metabolism : JPEM (2024)
In the MPS group, aortic elasticity properties were impaired, and aortic stiffness increased. ERT has positive effects on cardiac function, aortic diameter, and aortic stiffness in MPS patients. An increased LVmass-i and impaired ventricular geometric structure in patients with MPS may be associated with increased aortic stiffness.
Keyphrases
- aortic valve
- left ventricular
- pulmonary artery
- end stage renal disease
- replacement therapy
- aortic dissection
- newly diagnosed
- ejection fraction
- chronic kidney disease
- heart failure
- peritoneal dialysis
- coronary artery
- prognostic factors
- smoking cessation
- patient reported outcomes
- atrial fibrillation
- pulmonary arterial hypertension
- patient reported