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Pompe's disease and anaesthesia
Author(s) -
McFARLANE H.J.,
SONI N.
Publication year - 1986
Publication title -
anaesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.839
H-Index - 117
eISSN - 1365-2044
pISSN - 0003-2409
DOI - 10.1111/j.1365-2044.1986.tb13007.x
Subject(s) - medicine , glycogen storage disease , glycogen storage disease type ii , weakness , anesthesia , general anaesthesia , cardiomyopathy , disease , muscle disease , general anaesthetic , heart disease , local anaesthetic , muscle weakness , surgery , cardiology , heart failure , enzyme replacement therapy
Summary A case report of a child with Pompe's disease (glycogen storage disease Cori type II), who underwent two general anaesthetics, is presented. The progressive infiltration of heart and skeletal muscle with glycogen results in a severe form of cardiomyopathy and respiratory muscle weakness. Consequently, there are significant problems in the anaesthetic management of these patients. Although there are theoretical attractions for some anaesthetic drugs, the key factors in a successful outcome are attention to anaesthetic technique and close monitoring.