Williams syndrome

Summary Williams 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 discoverie...

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Bibliographic Details
Published in:Pediatric anesthesia 2019-05, Vol.29 (5), p.483-490
Main Authors: Twite, Mark D., Stenquist, Scott, Ing, Richard J.
Format: Article
Language:English
Subjects:
Anesthesia
Child
Child, Preschool
Humans
Infant
Infant, Newborn
prolonged QTc
pulmonary artery stenosis
sudden death
supravalvar aortic stenosis
Williams syndrome
Williams Syndrome - complications
Williams Syndrome - physiopathology
Williams‐Beuren syndrome
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Summary:Summary Williams 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.
ISSN:1155-5645
1460-9592
DOI:10.1111/pan.13620