title: [Pompe disease : anesthesiological special features].
abstract: Pompe disease is a very rare disorder of glycogen metabolism. Due to a deficiency of the enzyme glucosidase glycogen accumulates inside the lysosomes. The clinical picture varies widely as a consequence of varying participation of skeletal and heart muscle. In adults respiratory insufficiency can occur which must be taken into consideration during anesthesiology procedures for affected patients. This case report describes a 60-year-old patient scheduled for punch biopsy of the prostate.
authors.affiliation: Klinik für Anästhesie, operative Intensivmedizin und Schmerztherapie, Sozialstiftung Bamberg - Klinikum am Bruderwald, Deutschland. judith.lehberger@sozialstiftung-bamberg.de
authors.forename: J
authors.initials: J
authors.lastname: Lehberger
authors.forename: R
authors.initials: R
authors.lastname: Roth
authors.forename: K
authors.initials: K
authors.lastname: Weingärtner
authors.forename: M
authors.initials: M
authors.lastname: Heesen
mesh_terms: D000758:Anesthesia; D000771:Anesthesia, Intravenous; D001706:Biopsy; D005959:Glucosidases / Q000172:deficiency / Q000378:metabolism; D006003:Glycogen / Q000378:metabolism; D006009:Glycogen Storage Disease Type II / Q000150:complications / Q000175:diagnosis / Q000503:physiopathology*; D006801:Humans; D008247:Lysosomes / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000378:metabolism; D009206:Myocardium / Q000378:metabolism; D010993:Plethysmography, Whole Body; D011467:Prostate / Q000473:pathology; D012129:Respiratory Function Tests; D013147:Spirometry
chemical_list: D006003:Glycogen; D005959:Glucosidases