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Methemoglobinemia

Alternative Names

Hemoglobin M disease; Erythrocyte reductase deficiency; Generalized reductase deficiency

Treatment

A medicine called methylene blue is used to treat persons with severe methemoglobinemia. Note: Methylene blue may be dangerous in patients who have or may be at risk for a blood disease called G6PD deficiency, and should not be used. If you or your child have G6PD deficiency, always tell the health care provider before treatment is given.

Ascorbic acid may also be used to reduce the level of methemoglobin.

Alternative treatments include hyperbaric oxygen therapy and exchange transfusions.

In most cases of mild acquired methemoglobinemia, no treatment is required, other than avoiding the medicine or chemical that caused the problem. However, treatment (such as a transfusion) may be needed in severe cases.

Outlook (Prognosis)

Persons with Type 1 methemoglobinemia and hemoglobin M disease usually do well. Type 2 methemoglobinemia is much more serious, and usually causes death within the first few years of life.

Persons with acquired methemoglobinemia usually do very well once the drug, food, or chemical that caused the problem is avoided.

Possible Complications

  • Shock
  • Seizures
  • Death

When to Contact a Medical Professional

Call your health care provider if there is a family history of methemoglobinemia and you develop symptoms of this disorder.

Call your health care provider or emergency services immediately if there is severe shortness of breath.

References

Jaffe ER, Hultquist DE. Cytochrome b5 reductase deficiency and enzymopenic hereditary methemoglobinemia. In: Scriver CR, Beaudet AL, Sly WS, et al, eds. The Metabolic and Molecular Basis of Inherited Disease. 7th ed. New York, NY: McGraw-Hill; 1995:2267-2280.

Review Date: 6/24/2007
Reviewed By: Neal Sondheimer, M.D., Ph.D., Division of Genetics and Metabolism, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network.

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