Methemoglobinemia epidemiology and demographics

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

Overview

Congenital (Hereditary) Methemoglobinemia

1. Cytochrome b5 reductase deficiency and pyruvate kinase deficiency

2. G6PD deficiency

3. Presence of abnormal hemoglobin (Hb M)


Acquired or Acute Methemoglobinemia

Epidemiology and Demographics

The incidence of congenital methemoglobinemia in the United States is very low.

Age

  • Patients of all age groups may develop methemoglobinemia.
  • The acquired methemoglobinemia is a rare disease that tends to affect infants and people exposed to local anesthetics during medical procedures.
  • Infants, particularly those younger than 4 months are most susceptible to methemoglobinemia. This is due to the fact that the NADH methemoglobin reductase activity and concentration (the main protective enzyme against oxidative stress) is not fully mature in infants.

Gender

Developed Countries

  • There is no racial predilection to methemoglobinemia.
  • In developed countries, the incidence of acquired methemoglobinemia is higher in developing countries when people are exposed to local anesthetics during various medical procedures.
  • The majority of cytochrome b5 reductase deficiency cases are found among some Native American tribes like Navajo [1] and Athabaskan Alaskans, and the Yakutsk people in Siberia. [2]

Developing Countries

  • The highest prevalence of G6PD deficiency is observed in the malaria-endemic regions: Sub-Saharan Afria, West Asia and Arabian Peninsula, as well as in people of Mediterranean descent. [3] [4] [5]

References

  1. BALSAMO P, HARDY WR, SCOTT EM (1964). "HEREDITARY METHEMOGLOBINEMIA DUE TO DIAPHORASE DEFICIENCY IN NAVAJO INDIANS". J Pediatr. 65: 928–31. PMID 14244100.
  2. Burtseva TE, Ammosova TN, Protopopova NN, Yakovleva SY, Slobodchikova MP (2017). "Enzymopenic Congenital Methemoglobinemia in Children of the Republic of Sakha (Yakutia)". J Pediatr Hematol Oncol. 39 (1): 42–45. doi:10.1097/MPH.0000000000000705. PMID 27879543.
  3. Howes RE, Dewi M, Piel FB, Monteiro WM, Battle KE, Messina JP; et al. (2013). "Spatial distribution of G6PD deficiency variants across malaria-endemic regions". Malar J. 12: 418. doi:10.1186/1475-2875-12-418. PMC 3835423. PMID 24228846.
  4. Howes RE, Battle KE, Satyagraha AW, Baird JK, Hay SI (2013). "G6PD deficiency: global distribution, genetic variants and primaquine therapy". Adv Parasitol. 81: 133–201. doi:10.1016/B978-0-12-407826-0.00004-7. PMID 23384623.
  5. Lawton CA, Won M, Pilepich MV, Asbell SO, Shipley WU, Hanks GE; et al. (1991). "Long-term treatment sequelae following external beam irradiation for adenocarcinoma of the prostate: analysis of RTOG studies 7506 and 7706". Int J Radiat Oncol Biol Phys. 21 (4): 935–9. PMID 1917622.

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