ABSTRACT
Pompe disease (Glycogen storage disease type II) leads to abnormal glycogen deposition in various vital organs resulting in multiple systemic sequelae. We present the anaesthetic management for caesarean section of a 31-year-old parturient with known Pompe disease. The parturient had symptoms and signs of respiratory dysfunction and the pregnancy was complicated by preeclampsia. She underwent urgent caesarean section under regional anaesthesia resulting in the birth of a healthy baby girl. To our knowledge, this is the first reported case of both spinal anaesthesia for caesarean section and successful live birth in a patient with Pompe disease.
Subject(s)
Anesthesia, Epidural , Anesthesia, Obstetrical , Anesthesia, Spinal , Cesarean Section, Repeat , Glycogen Storage Disease Type II/complications , Pre-Eclampsia/drug therapy , Pregnancy Complications , Adult , Anesthesia, Obstetrical/methods , Female , Humans , Postoperative Care/methods , Pregnancy , Prenatal Care/methods , Spirometry , Treatment OutcomeABSTRACT
Alfentanil requirements were compared in thirty-six Asian and forty-three European patients during general anaesthesia with muscle relaxants. Alfentanil infusion at 5 micrograms/kg/min was started immediately after induction with thiopentone and alcuronium. The infusion rate was reduced to 0.5 microgram/kg/min after ten minutes. An incremental dose of 5 micrograms/kg/min for five minutes was given on each occasion when anaesthesia was clinically judged to be inadequate. Recovery parameters were recorded. Pharmacokinetics were also studied in five Europeans, four Chinese and four Nepalese. The dosage of alfentanil required was comparable in both Asian and European patients, but recovery was slower in the Asian patients. The elimination half-life in the Chinese and the Nepalese were both significantly shorter than that of the Europeans (P less than 0.05), but at the time of recovery of spontaneous ventilation, the mean plasma concentrations were not significantly different.