TY - CHAP M1 - Book, Section TI - Williams Syndrome A1 - Bissonnette, Bruno A1 - Luginbuehl, Igor A1 - Engelhardt, Thomas Y1 - 2019 N1 - T2 - Syndromes: Rapid Recognition and Perioperative Implications, 2e AB - A syndrome characterized by the presence of peculiar facial features such as broad forehead, short nose and full cheeks, giving an appearance of an “elfin (a small and delicate person in the enchanted world, typically with an attractively mischievous and/or strange charm).” Other clinical features include mild to moderate intellectual disability, very pleasant personality and ability to interact readily with strangers. It is associated with infantile hypercalcemia, cardiac defect in 80% (supravalvular aortic stenosis and peripheral pulmonary artery stenoses), and a high incidence of sudden death. Recent research has shown that the QTc interval in Williams Syndrome (WS) patients is significantly prolonged and may be associated with the sudden death. SN - PB - McGraw-Hill Education CY - New York, NY Y2 - 2024/03/29 UR - accessanesthesiology.mhmedical.com/content.aspx?aid=1164088131 ER -