• Paediatric anaesthesia · May 2019

    Review

    Williams syndrome.

    • Mark D Twite, Scott Stenquist, and Richard J Ing.
    • Department of Anesthesiology, University of Colorado Anschutz Medical Campus & Children's Hospital Colorado, Aurora, Colorado.
    • Paediatr Anaesth. 2019 May 1; 29 (5): 483-490.

    AbstractWilliams syndrome affects approximately one in 10 000 people and is caused by the deletion of genes on chromosome 7q11.23 which code for elastin. The phenotypic appearance of people with Williams syndrome is well characterized, but there continues to be new genetic and therapeutic discoveries. Patients with Williams syndrome have increased morbidity and mortality under sedation and anesthesia, largely as a result of cardiovascular abnormalities. This review article focuses on new information about Williams syndrome and outlines a structured approach to patients with Williams syndrome in the perioperative period.© 2019 John Wiley & Sons Ltd.

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