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General Anesthesia and Fabry Disease: A Case Report
Stefan Krüger1, Albina Nowak, Torben Christoph Müller
1From the *Department of Anesthesia and Intensive Care, Kantonsspital Schaffhausen, Schaffhausen, Switzerland; and †Clinic of Medicine/Endocrinology, UniversitätsSpital Zürich.
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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