Objective: To noninvasively measure arterial nail thickness in a group
of patients with Williams syndrome (WS). Methods: High-resolution, re
al-time B-mode ultrasonography was used to examine the carotid arterie
s of 20 patients with WS (ages 7 months to 24.9 years) and 25 control
subjects Cages 2.5 years to 25.5 years). Results: The mean combined in
ternal-medial wall thickness of the patients in the WS group was 0.86
mm +/- 0.08 mm compared with a mean of 0.54 mm +/- 0.05 mm in the cont
rol subjects (p < 0.0001). Within the WS group, arterial wall thicknes
s did not vary significantly with gender, patient age, the presence or
absence of stenotic cardiac disease, or the presence or absence of hy
pertension.Conclusions: The ultrasonographic finding of increased caro
tid arterial wall thickness across a wide range of patients with WS de
monstrates the pervasive nature of the arteriopathy of this disorder.
That increased arterial trail thickness was observed in all patients s
tudied suggests that the arteriopathy of WS is related to haploinsuffi
ciency for the elastin gene.