Pompe's disease and anaesthesia.

Abstract:

: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.

journal_name

Anaesthesia

journal_title

Anaesthesia

authors

McFarlane HJ,Soni N

doi

10.1111/j.1365-2044.1986.tb13007.x

subject

Has Abstract

pub_date

1986-12-01 00:00:00

pages

1219-24

issue

12

eissn

0003-2409

issn

1365-2044

journal_volume

41

pub_type

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