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Williams-Beuren Syndrome Updated 4/2011

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BASICS

  • Description
  • Epidemiology
  • Risk Factors
  • General Prevention
  • Etiology
  • Associated Conditions

DIAGNOSIS

  • Signs and Symptoms
  • Tests
  • Differential Diagnosis

TREATMENT

  • Medication (Drugs)
  • Additional Treatment
  • Surgery

Ongoing Care

  • Follow-Up Recommendations
  • Diet
  • Patient Education
  • Prognosis
  • Complications
The following is an excerpt....
BASICS

Description
  • Williams-Beuren syndrome (WBS), also known as Williams syndrome, is a multisystem neurodevelopmental disorder characterized by subtle, distinctive facial features and intellectual disabilities, including a characteristic cognitive-behavioral profile (1).
  • Growth retardation, hoarse voice, connective tissue abnormalities, vascular stenoses, (supravalvular aortic stenosis, peripheral pulmonary stenosis, renal artery stenosis), endocrine abnormalities (hypercalcemia, diabetes, subclinical and less often clinical hypothyroidism), musculoskeletal abnormalities (limited joint movement, lordosis), bowel and bladder diverticula
  • System(s) affected: Cardiovascular; Gastrointestinal; Endocrine/Metabolic; Musculoskeletal; CNS; Renal/Urologic
  • Synonym(s): Williams syndrome; Fanconi-type idiopathic infantile hypercalcemia; Elfin facies with hypercalcemia
ALERT: Pediatric Considerations
  • Infantile hypercalcemia, colic, failure to thrive
ALERT: Pregnancy Considerations
  • Genetic evaluation and counseling; prenatal diagnosis
Epidemiology

Incidence
  • In the US, 1/10,000 ...

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See Also
Images >
1
FIGURE 4.6. A: Williams syndrome. The facial appearance in patients is remarkably uniform. This boy’s eyebrows flare medially, his nasal bridge is depressed, the epicanthal folds are prominent, and the nares are anteverted. Note prominent lips and full cheeks. (Courtesy of Prof. K. Kruse, Medizinische Universität zu Lübeck, Germany, and Dr. R. Pankau, Universitäts Kinderklinik, Kiel, Germany.) B: Williams syndrome. A stellate pattern exists in the iris. (Courtesy Dr. G. Holmström, Department of Ophthalmology, Hospital for Sick Children, Great Ormond Street, London.)Credit: NA
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