Case report of sudden death in a child with Williams syndrome following administration of anaesthesia

Southern African Journal of Anaesthesia and Analgesia

 
 
Field Value
 
Title Case report of sudden death in a child with Williams syndrome following administration of anaesthesia
 
Creator du Toit-Prinsloo, Lorraine Dippenaar, Johannes M. Honey, Engela M.
 
Subject — deletion of genes on chromosone 7q11.23; intra-operative deaths; sudden death; supravalvular aortic stenosis; Williams syndrome
Description Williams syndrome is a neurodevelopmental disorder characterized by distinctive personality traits, facial features (so called “elfin face”) and cardiac abnormalities, of which supravalvular aortic stenosis is the most common lesion found. The cause is a deletion of a group of genes on chromosome 7q11.23. Administration of anaesthesia to these patients carries a higher risk for sudden death due to the cardiac defects. The purpose of this case study is to demonstrate the entity in the South African population, to review the literature, to put emphasis on the multi-disciplinary approach in the pre-operative management, and to review the medico-legal investigation of intra-operative deaths. Furthermore, administration of anaesthesia in the remote location and to syndromic children will also be discussed.
 
Publisher AOSIS
 
Contributor
Date 2015-01-01
 
Type info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Peer-reviewed Article —
Format application/pdf
Identifier 10.1080/22201181.2014.992158
 
Source Southern African Journal of Anaesthesia and Analgesia; Vol 21, No 1 (2015); 75-78 2220-1173 2220-1181
 
Language eng
 
Relation
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https://sajaa.co.za/index.php/sajaa/article/view/808/802 https://sajaa.co.za/index.php/sajaa/article/downloadSuppFile/808/419 https://sajaa.co.za/index.php/sajaa/article/downloadSuppFile/808/420 https://sajaa.co.za/index.php/sajaa/article/downloadSuppFile/808/421 https://sajaa.co.za/index.php/sajaa/article/downloadSuppFile/808/422
 
Coverage — — —
Rights Copyright (c) 2015 Lorraine du Toit-Prinsloo, Johannes Marthinus Dippenaar, Engela M Honey https://creativecommons.org/licenses/by/4.0
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