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Methemoglobinemia

 

 

Background

  • MetHb = oxidized form of Hb (heme iron configuration changed from ferrous (Fe2+) to ferric (Fe3+) state)

    • doesn't bind O2 --> can't deliver O2 to tissues

    • causes left shift of Hb O2 dissociation curve --> further ↓ in O2 delivery

  • Can be congenital or acquired

    • Congenital - ↓ enzymatic reduction of MetHb back to Hb

      • may appear cyanotic but generally asymptomatic

    • Acquired - from drugs that can oxidize Hb to MetHb

      • ↑ risk in infants & heterozygous CYB5R3 (cytochrome b5 reductase) mutations, G6PD deficiency

      • can be fatal

  • Drugs that can cause MetHb:

    • local anesthetics: prilocaine, lidocaine, benzocaine

    • NTG, sodium nitroprusside

    • Inhaled nitric oxide

    • Phenytoin

    • Sulfonamides

    • Metoclopramide

  • Diagnosis

    • symptoms of hypoxia that don't improve w/ O2

    • blood may have "chocolate brown" colour

    • [MetHb] on blood gas (co-oximetry)

      • Nl [MetHb] <1%

      • >10%: cyanosis

      • <20%: asymptomatic or headache, fatigue, lethargy

      • >20%: resp depression, altered LOC, seizures

      • >40%: life threatening

      • PaO2 usually normal or high

    • SpO2 may read 85% w/ no improvement w/ O2

  

Management

  • D/C precipitating agents

  • Rule out other causes of cyanosis

  • Supportive care - IV fluids, intubation/ventilation prn, antiseizure Rx prn

  • Methylene blue if concerning symptoms and/or [MetHb] >20-30%

    • accelerates reduction of MetHb

    • fast-acting; resolution w/in 20-60 mins

    • Contraindications:

      • G6PD deficiency --> can precipitate hemolysis

      • Caution if pt on serotonergic Rx --> can precipitate serotonin syndrome

  • Ascorbic acid if methylene blue contraindicated

  • Consider blood or exchange transfusion & hyperbaric in severe/refractory dz

References

  • Ludlow JT, Wilkerson RG, Nappe TM. Methemoglobinemia. [Updated 2022 Aug 29]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK537317/ 

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