Publication

Non-invasive measurements of atherosclerosis in adult cystinosis patients

Besouw, M. T. P., Holewijn, S., Levtchenko, E. N. & Janssen, M. C. H., Jun-2011, In : Journal of Inherited Metabolic Disease. 34, 3, p. 811-818 8 p.

Research output: Contribution to journalArticleAcademicpeer-review

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.

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).

GFR ranged between 4-95 mL/min/1.73 mA(2). 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.

Young adult cystinosis patients treated with cysteamine have no additional risk for CVD when compared to patients with chronic kidney disease of other causes.

Original languageEnglish
Pages (from-to)811-818
Number of pages8
JournalJournal of Inherited Metabolic Disease
Volume34
Issue number3
Publication statusPublished - Jun-2011

    Keywords

  • CHRONIC KIDNEY-DISEASE, INTIMA-MEDIA THICKNESS, PULSE-WAVE VELOCITY, CARDIOVASCULAR RISK, ARTERIAL STIFFNESS, HYPERTENSION, CREATININE, PRESSURE, CHILDREN

Download statistics

No data available

ID: 90140093