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General Anesthesia and Fabry Disease


Krüger, Stefan; Nowak, Albina; Müller, Torben Christoph (2017). General Anesthesia and Fabry Disease. A & A Case Reports, 8(10):247-249.

Abstract

Fabry disease is an inherited X-linked disorder characterized by the absence (in men) or deficiency (in women) in α-galactosidase A activity that causes a progressive accumulation of glycosphingolipids within lysosomes of cells of all the major organ systems. The subsequent organ damage that manifests in childhood and early adulthood presents a widely variable clinical picture of pain, hypertension, and cardiac, renal, nervous system, and lung dysfunction. We present 2 female patients with Fabry disease who required general anesthesia twice for gynecological and trauma surgery, respectively, and discuss their perioperative management based on new information in the medical literature.

Abstract

Fabry disease is an inherited X-linked disorder characterized by the absence (in men) or deficiency (in women) in α-galactosidase A activity that causes a progressive accumulation of glycosphingolipids within lysosomes of cells of all the major organ systems. The subsequent organ damage that manifests in childhood and early adulthood presents a widely variable clinical picture of pain, hypertension, and cardiac, renal, nervous system, and lung dysfunction. We present 2 female patients with Fabry disease who required general anesthesia twice for gynecological and trauma surgery, respectively, and discuss their perioperative management based on new information in the medical literature.

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Additional indexing

Contributors:Department of Anesthesia and Intensive Care, Kantonsspital Schaffhausen
Item Type:Journal Article, refereed, original work
Communities & Collections:04 Faculty of Medicine > University Hospital Zurich > Clinic and Policlinic for Internal Medicine
Dewey Decimal Classification:610 Medicine & health
Language:English
Date:2017
Deposited On:19 Oct 2017 10:38
Last Modified:15 May 2018 00:01
Publisher:Lippincott Williams & Wilkins
ISSN:2325-7237
OA Status:Green
Publisher DOI:https://doi.org/10.1213/XAA.0000000000000479
PubMed ID:28079663

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