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Anästhesie und Ahornsirupkrankheit : Fallbericht und perioperatives anästhesiologisches Management

Haberstich, P. and Kindler, C. H. and Schürch, M.. (2010) Anästhesie und Ahornsirupkrankheit : Fallbericht und perioperatives anästhesiologisches Management. Der Anästhesist, Vol. 59, H. 10. pp. 914-197.

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Official URL: http://edoc.unibas.ch/dok/A6007410

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Abstract

Maple syrup urine disease is a rare autosomal-recessive metabolic disorder caused by a deficit of oxidative decarboxylation of branched-chain amino acids. First symptoms appear in the neonatal period. Without treatment the disease is characterized by rapid progression of neurological symptoms. During stressful situations, such as infection or surgery, patients may experience severe ketoacidosis, rapid neurological deterioration and hypoglycemia. The perioperative management of a 26-year-old man with maple syrup urine disease is described, a review of the disease is given and anaesthesia-related implications are discussed.
Faculties and Departments:03 Faculty of Medicine > Bereich Querschnittsfächer (Klinik) > Anästhesiologie
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Querschnittsfächer (Klinik) > Anästhesiologie
UniBasel Contributors:Kindler, Christoph
Item Type:Article, refereed
Article Subtype:Further Journal Contribution
Bibsysno:Link to catalogue
Publisher:Springer-Verlag
ISSN:0003-2417
Note:Note: Englischer Titel: Anaesthesia in patients with maple syrup urine disease. Case report and perioperative anaesthetic management -- Publication type according to Uni Basel Research Database: Journal item
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Last Modified:12 Sep 2014 08:02
Deposited On:12 Sep 2014 08:02

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