Carotid ultrasound examination in Williams syndrome.
OBJECTIVE
To noninvasively measure arterial wall thickness in a group of patients with Williams syndrome (WS).


METHODS
High-resolution, real-time B-mode ultrasonography was used to examine the carotid arteries of 20 patients with WS (ages 7 months to 24.9 years) and 25 control subjects (ages 2.5 years to 25.5 years).


RESULTS
The mean combined intimal-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 control subjects (p < 0.0001). Within the WS group, arterial wall thickness did not vary significantly with gender, patient age, the presence or absence of stenotic cardiac disease, or the presence or absence of hypertension.


CONCLUSIONS
The ultrasonographic finding of increased carotid arterial wall thickness across a wide range of patients with WS demonstrates the pervasive nature of the arteriopathy of this disorder. That increased arterial wall thickness was observed in all patients studied suggests that the arteriopathy of WS is related to haploinsufficiency for the elastin gene.
Sadler
L S
LS
Department of Pediatrics, State University of New York at Buffalo and the Children's Hospital, 14222, USA.
Gingell
R
R
Martin
D J
DJ
D000293:Adolescent; D000328:Adult; D002339:Carotid Arteries / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D017539:Tunica Intima / Q000000981:diagnostic imaging; D014463:Ultrasonography; D018980:Williams Syndrome / Q000000981:diagnostic imaging*