Contractures in patients with Williams syndrome.
Williams syndrome is a multisystem disorder, including a characteristic facies and habitus. Intellectual, neurologic, and cardiovascular dysfunction occur; vascular disease may be progressive. Joint contractures affected 10 of a group of 20 children and young adults. In 3 of 10 patients, the contractures were severe enough to interfere with daily activities; in 3 the effect was only moderate. The contractures developed in early childhood and usually did not improve or worsen with time. Large and small joints were affected and usually were symmetrical. Their occurrence did not correlate with vascular disease. The contractures could not be attributed to a neurologic cause.
Kaplan
P
P
Department of Pediatrics, Montreal Children's Hospital and Research Institute, Quebec, Canada.
Kirschner
M
M
Watters
G
G
Costa
M T
MT
D000293:Adolescent; D000328:Adult; D001024:Aortic Valve Stenosis / Q000150:complications; D002648:Child; D002675:Child, Preschool; D003286:Contracture / Q000150:complications*; D005145:Face / Q000002:abnormalities*; D005260:Female; D006801:Humans; D006934:Hypercalcemia / Q000150:complications; D008607:Intellectual Disability / Q000150:complications*; D008297:Male; D013577:Syndrome