Carotid ultrasound examination in Williams syndrome☆,☆☆,★
Section snippets
Methods
The WS group, referred from an interdisciplinary WS clinic, included 11 male subjects and 9 female subjects ranging in age from 7 months to 24.9 years (mean age at time of examination 8.9 years). Fourteen of the 20 patients (70%) had structural cardiac disease, 9 (45%) of whom had SVAS, pulmonic stenosis, or both. Two patients (10%) had moderate to severe hypertension15 without associated aortic or renal artery stenosis. Fluorescent in situ hybridization studies disclosed an elastin gene
Results
Measurements of combined intimal-medial thickness for both the WS and control subjects (Fig. 3) disclosed no region of overlap. The mean carotid arterial wall thickness of the WS group was 0.84 ± 0.08 mm. Measurements ranged from 0.71 to 0.96 mm. The mean carotid arterial wall thickness of the control group was 0.54 ± 0.05 mm. The range within this group was 0.39 to 0.59 mm. The difference in arterial wall thickness
Discussion
The ultrasonographic finding of increased arterial wall thickness across a wide range of patients with WS demonstrates the pervasive nature of the arteriopathy of this disorder. In contrast to previous reports, which have focused on the vasculopathy of patients with WS with known cardiac disease, this study demonstrates ultrasonographic evidence of increased arterial wall thickness in patients with WS, irrespective of age or clinical status. These findings suggest that the arteriopathy of WS is
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Cited by (0)
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From the Divisions of Genetics and Cardiology, Departments of Pediatrics and Pediatric Radiology, State University of New York at Buffalo and the Children's Hospital, Buffalo.
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Reprint requests: Laurie S. Sadler, MD, Division of Genetics, Children's Hospital of Buffalo, 219 Bryant St., Buffalo, NY 14222.
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9/22/86318