Antunes, H

Fragoso, P

Gomes, CM

Oliveira, C

English

Repositório Comum

Glycogen storage disease type Ia: Assessment and preanesthetic management - Case Report Anesthesia General Balanced Anesthesia  Induction: Midazolam 1 mg + Fentanyl 100 µg + Propofol 100 mg   Airway: laryngeal mask # 2,5 Mantenaince: Sevoflurane in nitrous oxide/oxygen and air mixture Acetaminophen 500 mg iv Length of anesthesia : 55 minutes   Per-operative period without accidents or complications.  Time 9am 10am 11am 12am 13pm Glucose – venous blood (mg/dl) 128 110 84 95 83 BACKGROUND AND AIMS CASE REPORT DISCUSSION AND CONCLUSIONS  References: - Anesthesia and Co-Existing Disease 2008 5th ed: 319                     - Anaesthesia 1980; 35:699-702  Hourly blood glucose monitoring  Identification Boy, 11 years old, 29,6 Kg , ASA  III, with a diagnosis of G-Ia, since 12 months old. Presented with hepatomegaly, growth retardation, elevated liver enzymes and hyperlipidemia.  No previous surgeries and no history of allergies or atopy. Scheduled for elective circumcision.  - At 6am: last meal of corn starch;  - At 7am: admission and infusion of 5% glucose on half saline at a rate of 75cc/h;  - At 9am: hourly blood glucose determination was started ; - At 12h the surgery took place. Postoperative period At 2pm oral feeding resumed without any complications.  Fig. 1 – Schema of glycogen metabolism Glycogen Glucose Galactose  Glucose – 1 – Phosphate (P)  Glucose Glucose – 6 – P  Phosphorylase enzyme Phosphoglucomutase H2O Pi G6Pase Fructose – 6 – P Glyceraldehyde – 3 – Phosphate   Dihydroxyacetona – P   Fructose – 1 – P Fructose Pyruvate Lactato, Alanine    Celina Oliveira, Celina Gomes, Pedro Santos, Paulo Fragoso, Maria Crisóstomo, Henedina Antunes    Department of Anesthesiology, Braga Hospital, Portugal The pre-anesthetic evaluation and management of a patient with G-Ia is a challenge for the anesthesiologist, because these patients do not tolerate the fasting period required for the intervention (2). It is therefore necessary to adopt strategies to avoid hypoglycemia and lactic acidosis as well as its complications. According to the Pediatrics Department an adequate glycemic intake was selected, as well as its rate of administration; hourly blood glucose monitoring and infusion rate control were undertaken to maintain blood glucose levels above 60 mg/dl while avoiding acidosis. The use of Ringer´s lactate is not recommended by the risk of worsening lactic acidosis.  Glycogen storage disease type Ia (G-Ia; von Gierke´s disease) is a rare inherited metabolic disorder resulting from glucose-6-phosphatase deficiency, a key enzyme in the glycogen metabolism (1).  Since patients are unable to release glucose from the liver, hypoglycemia and lactic acidosis after short periods of fasting, and hepatomegaly are the most common signs. Prevention of complications by maintenance of normoglycemia, demands the ingestion of corn starch in a 3 hourly basis.  Hyperuricemia, hyperlipidemia and platelet dysfunction are also common (1).  Pre-anesthetic evaluation  

Glycogen storage disease type Ia: assessment and preanesthetic management: case report

Institutional repository of Hospital de Braga

http://comum.rcaap.pt/bitstream/10400.26/1924/1/Glycogen%20storage%20disease%20type%20Ia.pdf

Glycogen storage disease type Ia: assessment and preanesthetic management: case report

Abstract

Similar works

Full text

Available Versions

Repositório Comum

Institutional repository of Hospital de Braga