Abnormally increased carotid intima media-thickness and elasticity in patients with Morquio A disease.
Raymond Yu-Jeang WangKyle D RudserDonald R DengelNicholas EvanoffJulia SteinbergerNina MovsesyanRobert GarrettKatherine ChristensenDeborah BoylanStephen R BraddockMarwan ShinawiQi GanAdriana M MontañoPublished in: Orphanet journal of rare diseases (2020)
Increased carotid elasticity in Morquio A patients is an unexpected contrast to the reduced elasticity observed in other MPS. These Morquio A cIMT findings corroborate MPS IVA arterial post-mortem reports and are consistent with cIMT of other MPS. Aortic root dilatation in Morquio A indicates arterial elastin dysfunction, but their carotid hyperelasticity indicates other vascular intima/media components, such as proteoglycans, may also influence artery function. Studying MPS I and IVA model systems may uniquely illuminate the function of glycosaminoglycan-bearing proteoglycans in arterial health.
Keyphrases
- end stage renal disease
- healthcare
- ejection fraction
- public health
- newly diagnosed
- chronic kidney disease
- magnetic resonance
- cardiovascular risk factors
- prognostic factors
- peritoneal dialysis
- aortic valve
- left ventricular
- heart failure
- health information
- cardiovascular disease
- emergency department
- magnetic resonance imaging
- optical coherence tomography
- metabolic syndrome
- pulmonary artery
- climate change
- coronary artery
- contrast enhanced
- electronic health record