Williams-Beuren Syndrome - Wobble

Williams-Beuren syndrome

(wil′yămz-būr′ĕn) [J.C.P. Williams; A.J. Beuren, Ger. cardiologist, 1919–1984] Williams syndrome.

Williamsia

(wĭl-yăm′zē-ă) A genus of mycolic acid containing actinomycetes. Most members of the genus are thought to be nonpathogenic bacteria found in a variety of natural environments. They have rarely been identified in culture specimens as a cause of human disease.

Williams syndrome

(wil′yămz) [J.C.P. Williams, 20th-cent. New Zealand cardiologist] ABBR: WS. A rare congenital disorder caused by a deletion of part of chromosome 7 characterized by impaired growth, heart disease, hypercalcemia, mental retardation, sensitivity to loud sounds, and "elfin" facial features, among other anomalies. Some children with WS are described as excessively sociable, hyperactive, and musically talented, but these behavioral markers are not universally present. SYN: Williams-Beuren syndrome.

Willis, Thomas

(wil′is) Brit. anatomist, 1621–1675.

 circle of W. An arterial anastomosis that encircles the optic chiasm and hypophysis, from which the principal arteries supplying the brain are derived. It receives blood from the two internal carotid arteries and the basilar artery formed by union of the two vertebral arteries. SYN: cerebral arterial circle. SEE: illus.

CIRCLE OF WILLIS

Inferior view of brain

willow bark

(wil′ō) The bark of many species of the genus Salix, the willow tree. This bark is the source of salicylic acid, the active principle in aspirin.

Wilms tumor

(vilmz) [Max Wilms, Ger. surgeon, 1867–1918] A rapidly developing tumor of the kidney that usually occurs in children. It is the most common renal tumor of childhood. It is associated with chromosomal deletions, esp. from chromosomes 11 and 16. In the past, the mortality from this type of cancer was extremely high; however, newer approaches to therapy have been very effective in controlling the tumor in about 90% of patients. SYN: embryonal carcinosarcoma; nephroblastoma.

Wilson disease

(wil′sŏn) [Samuel Alexander Wilson, Brit. internist, 1877–1937] An hereditary syndrome in which faulty metabolism of copper allows the metal to accumulate in toxic concentrations in the liver, and eventually, in the bones, joints, brain, cornea, kidney, and skin.

INCIDENCE: The disease is rare. It is present in about 1 in 40,000 people and usually becomes clinically apparent in the first four decades of life.

CAUSES: The disease is inherited as an autosomal recessive trait.

SYMPTOMS AND SIGNS: The disease is characterized by elevated liver enzymes, hemolytic anemia, degenerative changes in the brain, cirrhosis of the liver, splenomegaly, tremor, progressive weakness, dystonia, muscular rigidity, involuntary movements, spastic contractures, and psychatric disturbances.

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