General Anesthesia and Fabry Disease: A Case Report. Issue 10 (15th May 2017)

• Record Type: Journal Article
• Authors: Krüger, Stefan; Nowak, Albina; Müller, Torben Christoph
• Is Part Of: A & A case reports. Volume 8:Issue 10(2017:May 15)
• Publication Date: 2017-05-15
• Subjects: Anesthesiology -- Periodicals; Anesthesia -- Periodicals; Anesthesiology; Analgesia; Anesthesiology; Periodicals; 617.9605
• DOI: 10.1213/XAA.0000000000000479
• Languages: English
• ISSNs: 2325-7237

Abstract

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.