Methemoglobinemia risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Template:Aksiniya K. Stevasarova, M.D.


Overview

Congenital (Hereditary) Methemoglobinemia

There are three main congenital conditions that lead to methemoglobinemia:

1. Cytochrome b5 reductase deficiency and pyruvate kinase deficiency

2. G6PD deficiency

3. Presence of abnormal hemoglobin (Hb M)


Acquired or Acute Methemoglobinemia

Some of the most common causes include different oxidant drugs, toxins and chemicals

Risk Factors

[1] [2]

Congenital (Hereditary) Methemoglobinemia

There are three main congenital conditions that lead to methemoglobinemia:

1. Cytochrome b5 reductase deficiency and pyruvate kinase deficiency

2. G6PD deficiency

3. Presence of abnormal hemoglobin (Hb M)


Both cytochrome b5 reductase deficiency and pyruvate kinase deficiency can lead to NADH deficiency which in turn will lead to decreased ability to remove MetHb from the blood. Cytochrome b5 reductase deficiency is an autosomal recessive disorder with at least 2 forms that we know of. The most common form, is the Ib5R deficiency, where cyt b5 reductase is absent only in RBCs, and the levels of MetHb are around 10% to 35%.

The second type, which is much less common, is the IIb5R, where MetHb varies between 10% and 15% and the cyt b5 reductase is absent in all cells. This form is associated with mental retardation, microcephaly, and other neurologic problems. The lifespan of the affected individuals is greatly affected and patients usually die very young.

Congenital deficiency in G6PD can lead to decreased levels of NADPH and thus compromising the function of the diaphorase II enzyme system.

Abnormal hemoglobins like Hb M, including Hb Ms, Hb MIwate, Hb MBoston, Hb MHyde Park, and Hb MSaskatoon, an autosomal dominant condition, can also lead to methemoglobinemia. In case of amino acid substitution in the alpha-chain of hemoglobin, we observe cyanosis at birth, and infants with beta chain amino acid substitution will present with cyanosis later around 4-6 months of age.


Acquired methemoglobinemia

The acquired methemoglobinemia is significantly more common than the congenital one. It is associated with exposure to or ingestion of oxidant drugs, toxins or chemicals, that cause acute increment in methemoglobin levels, by overwhelming the normal physiologic protective enzyme mechanisms. The most common agents are anesthetics like benzocaine, lidocaine, prilocaine, used locally or topically, antibiotics like dapsone (used for the treatment of Brown Recluse spider bites, Leprosy, PCP prophylaxis, ecc) trimethoprim, sulfonamides, nitrates([[amynitrate])), nitroglycerin (NG), [[aniline dyes)], metoclopramide, chlorates and bromates.

Drug Induced

• Anesthetics like benzocaine, lidocaine, prilocaine

Methylene blue

Nitric oxide

Amilnitrate

Nitroglycerin

• Antimalarial drugs like Primaquine phosphate (in nicotinamide adenine dinucleotide (NADH) methemoglobin reductase deficient individuals)

Rasburicase

Sulfasalazine

Dapsone

Trimethoprim

Sulfonamides

Aniline dyes

Metoclopramide

Chlorates and Bromates and others


Contaminated well water (in premature infants and infants younger than 4 months)

Solid foods (not well cooked vegetables high in nitrates in premature infants and infants younger than 4 months)


  1. Template:Med Toxicol. 1986 Jul-Aug;1(4):253-60. Drug- and chemical-induced methaemoglobinaemia. Clinical features and management. Hall AH, Kulig KW, Rumack BH.pmid=PMID: 3537620
  2. {{Rev Bras Anestesiol. 2008 Nov-Dec;58(6):651-64. Methemoglobinemia: from diagnosis to treatment. [Article in English, Portuguese] do Nascimento TS1, Pereira RO, de Mello HL, Costa J. pmid=19082413}}

References

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