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Anaesthetic management of patients with Williams syndrome


Authors: P. Nosková
Authors‘ workplace: Klinika anesteziologie, resuscitace a intenzivní medicíny, 1. lékařská fakulta Univerzity Karlovy a Všeobecná fakultní nemocnice v Praze
Published in: Anest. intenziv. Med., 29, 2018, č. 3, s. 148-150
Category: Special article

Overview

Williams syndrome is caused by a genetic abnormality. The most common symptoms are heart defects and unusual facial features. Patients are known to have increased risk of adverse events during anaesthesia and difficult airway. The article concerns risk assessment and practical anaesthetic management of patients with Williams syndrome.

Keywords:

Williams syndrome – coronary artery disease – sudden death – difficult airway – anaesthetic  management


Sources

1.   Morris CA, Thomas IT, Greenberg F. Williams syn­drome: autosomal dominant inheritance. Am J Med Genet. 1993;47:478−481.

2.   Williamsův syndrom. Brožura občanského sdružení Willík. Praha, 2010.

3.   Burch TM, Mc Gowan FX, Kuussmann BD, et al. Congenital supravalvular stenosis and sudden death associated with anesthesia: what's the mystery? Anesth Analg. 2008;107:1848−1854.

4.   Committee on Genetics American Academy of Pediatrics health care supervision for children with Williams syndrome. Pediatrics. 2001;107:1192−1204.

5.   Matisoff AJ, Olivieri L, Schwartz JM, et al. Risk assessment and anesthetic management of patients with Williams syndrome: a comprehensive review. Pediatr anesth. 2015;1−9.

Labels
Anaesthesiology, Resuscitation and Inten Intensive Care Medicine
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