What is the prevalence of methemoglobinemia in the US?

Updated: Dec 09, 2018
  • Author: Mary Denshaw-Burke, MD, FACP; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Hereditary methemoglobinemia is rare. The most common cause of congenital methemoglobinemia is cytochrome b5 reductase deficiency (type Ib5R). This enzymatic deficiency is endemic in certain Native American tribes (Navajo and Athabaskan Alaskans).

Most cases of methemoglobinemia are acquired and result from exposure to certain drugs or toxins. One of the more common causes of acquired methemoglobinemia is exposure to topical benzocaine during medical procedures. An estimated 0.115% of patients undergoing transesophageal echocardiography (TEE) develop methemoglobinemia. [13, 51, 52]

A large retrospective cohort study found a high incidence of methemoglobinemia (up to 19.8%) in 167 pediatric patients receiving dapsone for PCP prophylaxis. [53] The median methemoglobin level was 9% (range, 3.5-22.4%). The risk of developing methemoglobinemia was increased in those patients receiving a higher dose of dapsone (≥20% above the target dosage of 2 mg/kg/day).

A retrospective study from 2 large teaching hospitals in the United States identified 138 cases of acquired methemoglobinemia over a period of 28 months. [14]


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