title: Non-invasive measurements of atherosclerosis in adult cystinosis patients.
abstract: BACKGROUND
  Cystinosis is characterized by intralysosomal cystine accumulation, causing end stage renal disease around 10 years of age if not treated with cysteamine. Cystine accumulation in blood vessels might increase atheroma formation or arterial stiffness and therefore increase the risk for cardiovascular disease (CVD). This study aimed to investigate the risk for CVD by non-invasive measures of atherosclerosis (NIMA) and to evaluate the effect of cysteamine treatment.
  
  
  METHODS
  Thirteen Dutch adult cystinosis patients were included. White blood cell (WBC) cystine levels, glomerular filtration rate (GFR) and concommitant medications were obtained from medical records. NIMA included carotid intima-media thickness (cIMT, n = 13), pulse wave velocity (PWV, n = 8) and pulse wave analysis (PWA, n = 6).
  
  
  RESULTS
  GFR ranged between 4-95 mL/min/1.73 m². All but one patient were treated with cysteamine, mean WBC cystine values ranged between 0.34-1.64 nmol cystine/mg protein, 8 patients had mean WBC cystine levels <1 nmol cystine/mg protein. When compared to healthy subjects, cIMT and PWV levels were above normal values in 1 patient for each measure. PWA measurements showed high augmentation index in three patients who did not receive lipid-lowering medication. When corrected for renal function, cIMT and PWV levels were within the normal range.
  
  
  CONCLUSIONS
  Young adult cystinosis patients treated with cysteamine have no additional risk for CVD when compared to patients with chronic kidney disease of other causes.
authors.affiliation: Department of Pediatric Nephrology, University Hospitals Leuven, Leuven, Belgium.
authors.forename: Martine T P
authors.initials: MT
authors.lastname: Besouw
authors.forename: Suzanne
authors.initials: S
authors.lastname: Holewijn
authors.forename: Elena N
authors.initials: EN
authors.lastname: Levtchenko
authors.forename: Mirian C H
authors.initials: MC
authors.lastname: Janssen
mesh_terms: D000328:Adult; D050197:Atherosclerosis / Q000150:complications* / Q000175:diagnosis* / Q000473:pathology / Q000503:physiopathology; D001794:Blood Pressure; D003554:Cystinosis / Q000150:complications* / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology; D003935:Diagnostic Techniques, Cardiovascular; D005260:Female; D005919:Glomerular Filtration Rate; D006339:Heart Rate; D006801:Humans; D008297:Male; D009929:Organ Size; D017539:Tunica Intima / Q000473:pathology; D055815:Young Adult