title: Endothelial Function and Vascular Properties in Children with Sickle Cell Disease.
abstract: BACKGROUND
  Sickle cell disease (SCD) is an inherited disorder characterized by recurrent painful crises with ischemia resulting from vascular occlusion. Adults with SCD have increased arterial stiffness and reduced flow-mediated dilation (FMD), due to impaired release of substances such as nitric oxide.
  
  
  OBJECTIVE
  We aimed to assess the vascular properties of carotid and brachial arteries in children with SCD compared with a control group without cardiovascular risk factors.
  
  
  METHODS
  Thirty patients with SCD, mean age 12.3 ± 4.5 years, were prospectively enrolled. A control group was made up of 30 age- and gender-matched healthy subjects. Carotid intima-media thickness (IMT), cross-sectional compliance (CSC), cross-sectional distensibility (CSD), diastolic wall stress (DWS), incremental elastic modulus (Einc), and FMD were determined in both groups.
  
  
  RESULTS
  There was no significant difference in FMD between the two groups (8.2 ± 5.0% in the SCD group vs. 9.3 ± 4.2% in the control group, P = 0.15). There was no significant correlation between FMD and age, hemoglobin, LDH level, or transcranial Doppler findings. CSD was significantly elevated in the SCD group (0.96 ± 0.44 vs. 0.59 ± 0.21, P = 0.0002), whereas DWS and Einc were significantly lower in the SCD group. CSC did not differ significantly between the two groups.
  
  
  CONCLUSIONS
  Children with SCD have no marked endothelial dysfunction or change in arterial stiffness. These manifestations may be related to disease severity and duration. Changes may become evident later in life as the disease progresses.
authors.affiliation: Department of Pediatric Cardiology, Children's Hospital, Toulouse, France.
authors.forename: Khaled
authors.initials: K
authors.lastname: Hadeed
authors.affiliation: Department of Pediatric Cardiology, Children's Hospital, Toulouse, France.
authors.forename: Sébastien
authors.initials: S
authors.lastname: Hascoet
authors.affiliation: Department of Pediatric Hematology, Children's Hospital, Toulouse, France.
authors.forename: Marie-Pierre
authors.initials: MP
authors.lastname: Castex
authors.affiliation: Department of Pediatric Clinical Research, Children's Hospital, Toulouse, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Munzer
authors.affiliation: Department of Pediatric Cardiology, Children's Hospital, Toulouse, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Acar
authors.affiliation: Department of Pediatric Cardiology, Children's Hospital, Toulouse, France.
authors.forename: Yves
authors.initials: Y
authors.lastname: Dulac
mesh_terms: D000755:Anemia, Sickle Cell / Q000150:complications* / Q000000981:diagnostic imaging / Q000503:physiopathology*; D001916:Brachial Artery / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002339:Carotid Arteries / Q000000981:diagnostic imaging / Q000503:physiopathology*; D002648:Child; D055119:Elastic Modulus; D005260:Female; D006801:Humans; D008297:Male; D058729:Peripheral Arterial Disease / Q000000981:diagnostic imaging / Q000209:etiology* / Q000503:physiopathology*; D015203:Reproducibility of Results; D012680:Sensitivity and Specificity; D033081:Shear Strength; D014463:Ultrasonography; D014655:Vascular Resistance
keywords: endothelial function; flow-mediated dilation; sickle cell disease; vascular elastic properties