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