1/2. Anaesthesia for diagnostic muscle biopsy in an infant with Pompe's disease.The anaesthetic management of children with glycogen-storage disease type IIa (Pompe's disease) presents a variety of challenges. A modification of a femoral nerve block, the inguinal paravascular block, as described by Winnie, was used in conjunction with intravenous ketamine to provide anaesthesia for a diagnostic muscle biopsy in a 5.5-month-old infant with Pompe's disease. A peripheral nerve stimulator was used to locate the femoral nerve in lieu of eliciting a paraesthesia.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/2. Pompe's disease and anaesthesia.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.- - - - - - - - - - ranking = 4keywords = anaesthesia (Clic here for more details about this article) |