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X-linked recessive deficiency in glucose-6-phosphate dehydrogenase enzyme leading to RBC hemolysis:

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  • Abnormally low levels of metabolic enzyme involved in pentose phosphate pathway
  • Important in RBC metabolism
  • Causes nonimmune hemolytic anemia (direct Coombs negative)
  • Neonatal jaundice with kernicteris
  • Hemolytic crisis in response to triggers
  • More frequent in patients with African, southern European, Middle Eastern, southeast Asian, or central and southern Pacific Island descent

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Information to Elicit in H & P in Patient with G6P Deficiency
HistoryPhysical exam
  • Previous crisis with treatment
  • Medication for G6P deficiency
  • Recent history of fatigue
  • History of dark urine
  • Complaint of lumbar/substernal pain
  • Review medication history (especially recent initiation of any offending agent)
  • Cyanosis
  • Jaundice
  • Dark urine
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  • Thorough H & P
  • Avoid triggers:
    • Illness:
      • Infection (hyperthermia)
      • Stress
      • Acidosis
      • Hyperglycemia (thus DKA can trigger hemolysis)
    • Foods: broad beans (also known as fava beans)
    • Drugs:
      • Antimalarial drugs: primaquine, pamaquine, chloroquine
      • Sulfonamides: sulfanilamide, sulfamethoxazole mafenide, thiazolesulfone, dapsone
      • Methylene blue, toluidine blue
      • Drugs causing methemoglobinemia: benzocaine, lidocaine, articaine, prilocaine
      • Certain analgesics: aspirin, phenazopyridine acetanilide
      • Nalidixic acid, nitrofurantoin
      • TNT
      • Naphthalene (a chemical compound used for industrial strength removal of grease) should also be avoided by people with G6PD deficiency
  • Administer benzodiazepine to prevent/treat anxiety

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Monitors

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Temperature probe:

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  • Avoid hyperthermia

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At least two IVs in case transfusion necessary.

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Foley: monitor urine closely for signs of hemolytic anemia (dark urine).

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Induction

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  • Routine anesthetic care with no restrictions on GA versus MAC versus regional
  • Avoid local anesthetics that can lead to methemoglobinemia (prilocaine, benzocaine, lidocaine [rare])

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Maintenance

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  • Continue to avoid triggering agents
  • Antibiotics, strict aseptic precautions to prevent infection
  • Opioids or nerve block to provide adequate analgesia (as stress is a trigger)

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  • Adequate analgesia
  • Clinical signs (fatigue, cyanosis) will arise 24–72 hours after initiation of offending agent
  • Labs:
    • Peripheral blood smear: “schistocytes” and reticulocytes
    • Denatured hemoglobin inclusions within RBCs = Heinz bodies
    • LDH elevated
    • Unconjugated bilirubin elevated
    • Haptoglobin levels decreased
    • Direct Coombs result should be negative (as hemolysis is not immune)
    • Hemosiderin and urobilinogen in the urine indicate chronic intravascular hemolysis
  • Patients being discharged home should be counseled to seek medical attention if signs and symptoms arise
  • Treat acute hemolytic anemia by discontinuing the offending agent and maintaining adequate urine output with fluid replacement and diuretics
  • Blood transfusions in more severe cases

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Signs of hemolytic anemia from G6PD deficiency difficult to notice during GA: monitor for signs and symptoms closely postoperatively.

1. Elyassi AR, Rowshan HH. Perioperative management of the glucose-6-phosphate dehydrogenase deficient patient: a review of literature. Anesth Prog. 2009 Autumn;56(3):86–91.   [PubMed: 19769422]
2. Cappellini MD, Fiorelli G. Glucose-6-phosphate dehydrogenase deficiency. Lancet. 2008 Jan;371(9606):64–74.   [PubMed: 18177777]

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