Anaesthetic management of a patient with glycogen storage disease type 1b

Ze'ev Shenkman, Yackov Golub, Shimon Meretyk, Yoram Shir, Daniel Landau, Ezekiel H. Landau

    Research output: Contribution to journalArticlepeer-review

    12 Scopus citations

    Abstract

    Purpose: A rare case of a ten-year old patient with type 1b glycogen storage disease (GSD), scheduled for extracorporeal shockwave lithotripsy (ESWL), is described. Clinical features: Patients with type 1b GSD manifest a range of clinical symptoms, including mental retardation, hepatosplenomegaly, renal enlargement, stomatitis, hypoglycaemic convulsions, bleeding diathesis, lactic acidosis and leukopaenia, thus creating a challenge for the anaesthetist. Following preanaesthetic administration of glucose-containing fluids, general anaesthesia was induced and the patient was mechanically ventilated. Except for mild hypoglycaemia after induction of anaesthesia, and moderate intraoperative metabolic acidosis, which was attributed to the underlying disorder, anaesthesia was uneventful. No postoperative complications occurred and the patient was discharged home three days after lithotripsy. Clinical features of this rare inborn error of metabolism are reviewed and the approach for the anaesthetic management is discussed. Conclusions: A skillful perioperative management of patients with type 1b GSD can be achieved by cautious attention to the metabolic and homeostatic derangements that occur with the disease.

    Original languageEnglish
    Pages (from-to)467-470
    Number of pages4
    JournalCanadian Journal of Anaesthesia
    Volume43
    Issue number5
    DOIs
    StatePublished - 1 Jan 1996

    Keywords

    • Acid-base equilibrium: acidosis, lactic
    • Anaesthesia: paediatric
    • Metabolism: glycogen storage disease

    ASJC Scopus subject areas

    • Anesthesiology and Pain Medicine

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