Methemoglobinemia epidemiology and demographics

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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

Most common cause include different oxidant drugs, toxins or chemicals

Epidemiology and Demographics

The incidence of congenital methemoglobinemia in the United States is very low. The majority of cytochrome b5 reductase deficiency cases are found amongst some Native American tribes like Navajo [1] and Athabaskan Alaskans, and the Yakutsk people in Siberia. [2]

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. Both cytochrome b5 reductase deficiency and pyruvate kinase deficiency are autosomal recessive diseases and the Hb M has autosomal dominant pattern of inheritance. The acquired Methemoglobinemia affects men and women equally, although ([G6PD]] deficiency is X-linked, therefore the risk of acquired methemoglobinemia is greater in males. There is no racial predilection to Methemoglobinemia. 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. Howes RE1, Dewi M, Piel FB, Monteiro WM, Battle KE, Messina JP, Sakuntabhai A, Satyagraha AW, Williams TN, Baird JK, Hay SI. pmid=24228846 }}</ref> [3] [4]



Epidemiology and  Demographics


Prevalence

Prevalence is defined as the total number of cases of a disease in a given at-risk population at a specific time. Note: For diseases specific to one gender, such as prostate cancer, only male patients are considered in the equation since only male patients are the at-risk population. When using numbers with decimal points, avoid reporting more than 1 number after the decimal point. For example, report a prevalence as 10.1 instead of 10.09322. Many numbers after the decimal point may suggest a false sense of accuracy. You can use one or more of these template sentences. Worldwide, the prevalence of (insert disease state here) ranges from a low of _____ per 100,000 persons to a high of _____ per 100,000 persons with an average prevalence of _____ per 100,000 persons. Worldwide, the prevalence of (insert disease state here) is _____ per 100,000 persons. In developed countries, the prevalence of (insert disease state here) ranges from a low of _____ per 100,000 persons to a high of _____ per 100,000 persons with an average prevalence of _____ per 100,000 persons. In developed countries, the prevalence of (insert disease state here) is _____ per 100,000 persons. In developing countries/ Africa, the prevalence of (insert disease state here) ranges from a low of _____ per 100,000 persons to a high of _____ per 100,000 persons with an average prevalence of _____ per 100,000 persons. In developing countries/ Africa, the prevalence of (insert disease state here) is _____ per 100,000 persons. In ____ (insert year), the prevalence of _______ (insert disease name) was estimated to be _______ (insert number) cases per 100,000 individuals worldwide. Incidence Incidence is defined as the number of new cases per population in a given time period. The standard format to report the incidence is ___ per 100,000 individuals. When using numbers with decimal points, avoid reporting more than 1 number after the decimal point. For example, report a incidence as 10.1 instead of 10.09322. Many numbers after the decimal point may suggest a false sense of accuracy. You can pick one or more of the template sentences below for this section: Worldwide, the incidence of (insert disease state here) ranges from a low of _____ per 100,000 persons to a high of _____ per 100,000 persons with an average incidence of _____ per 100,000 persons. Worldwide, the incidence of (insert disease state here) is _____ per 100,000 persons. In developed countries, the incidence of (insert disease state here) ranges from a low of _____ per 100,000 persons to a high of _____ per 100,000 persons with an average incidence of _____ per 100,000 persons. In developed countries, the incidence of (insert disease state here) is _____ per 100,000 persons. In developing countries/ Africa, the incidence of (insert disease state here) ranges from a low of _____ per 100,000 persons to a high of _____ per 100,000 persons with an average incidence of _____ per 100,000 persons. In developing countries/ Africa, the incidence of (insert disease state here) is _____ per 100,000 persons. In ____ (insert year), the incidence of _______ (insert disease name) was estimated to be _______ (insert number) cases per 100,000 individuals worldwide. Case Fatality Rate The case fatality rate is defined the number of deaths (fatality) among patients with the disease (case). Case fatality rate should be distinguished from "Mortality Rate" (defined as the number of deaths among the at-risk population). The case fatality rate is usually described as a function of time (e.g. In 2015 / annually etc...). When using numbers with decimal points, avoid reporting more than 1 number after the decimal point. For example, report the case fatality rate as 10.1% instead of 10.09322%. Many numbers after the decimal point may suggest a false sense of accuracy. You can pick the template sentence below for this section: In ____ (year), the case fatality rate of ____ (disease name) is ____ (case fatality rate in %). The annual case fatality rate of ____ (disease name) is approximately ____ (case fatality rate in %). Age This section can describe the impact of the disease depending on the persons age, and the age-specific prevalence and incidence. The prevalence of ____ (insert disease state here) increases/decreases with age. _____ (insert acute disease) commonly affects _____ (insert age group). _____ (insert chronic disease) is usually first diagnosed among _____ (insert age group). Gender This section describe how prevalence of the disease varies by gender. When describing male to female ratios with decimal points, avoid reporting more than 1 number after the decimal point. For example, report a ratio as as 1.5 to 1 instead of 1.48294 to 1. Many numbers after the decimal point may suggest a false sense of accuracy. You can use either of the following template sentences:

______ (insert gender 1) are more commonly affected with ______ (insert disease name) than _____ (insert gender 2). The _____ (insert gender ratio e.g. male to female) ratio is approximately _____ (insert number > 1) to 1. The prevalence and incidence of (insert disease state here) does not vary by gender. Men and women are affected equally by (insert disease name here). (Insert disease state here) is more prevalent in men women. The prevalence of (insert disease state here) among men is ____ per 100,000, while it is _____ per 100,000 among women. The incidence of (insert disease state here) among men is ____ per 100,000, while it is _____ per 100,000 among women. Race This section describes how the disease differs based upon race. You can use the following template sentence for this section:

The prevalence of _____ (insert disease) does not vary by race. (Insert disease state here) is more prevalent in the ____ (inser race) race and ____ (insert other race) races. There is no racial predilection for ____ (insert disease name) _____ (insert disease) usually affects individuals of the _____ (inser race) race. _____ (insert other race) individuals are less likely to develop ______ (disease name). Developed Countries In this section you should describe the impact of the disease in developed countries. Incidence, prevalence, and geographical distribution or areas of interest can be mentioned, as well as the relevant impact the disease has on society. Developing Countries In this section you should describe the impact of the disease in developing countries. Incidence, prevalence, and geographical distribution or areas of interest can be mentioned, as well as the relevant impact the disease has on society. Preferred Template Statements IF the incidence/prevalence of the disease is known:

The incidence/prevalence of [disease name] is approximately [number range] per 100,000 individuals worldwide. In [year], the incidence/prevalence of [disease name] was estimated to be [number range] cases per 100,000 individuals worldwide. The prevalence of [disease/malignancy] is estimated to be [number] cases annually. IF the case-fatality rate is also known, you may use either of the following template statements:

In [year], the incidence of [disease name] is approximately [number range] per 100,000 individuals with a case-fatality rate of [number range]%. The case-fatality rate of [disease name] is approximately [number range]. IF details about prevalence according to age/race/sex are known:

Age: Patients of all age groups may develop [disease name]. The incidence of [disease name] increases with age; the median age at diagnosis is [#] years. [Disease name] commonly affects individuals younger than/older than [number of years] years of age. Race: There is no racial predilection to [disease name]. [Disease name] usually affects individuals of the [race 1] race. [Race 2] individuals are less likely to develop [disease name]. Sex: [Disease name] affects men and women equally. [Gender 1] are more commonly affected by [disease name] than [gender 2]. The [gender 1] to [gender 2] ratio is approximately [number > 1] to 1. IF details about prevalence by region are known:

The majority of [disease name] cases are reported in [geographical region]. If additional details are known about the patient population in which the disease is typically diagnosed, they may be included here. Supplementary template statements include:

[Disease name] is a common/rare disease that tends to affect [patient population 1] and [patient population 2]. [Chronic disease name] is usually first diagnosed among [age group]. [Acute disease name] commonly affects [age group].

References

  1. {{J Pediatr. 1964 Dec;65:928-31. HEREDITARY METHEMOGLOBINEMIA DUE TO DIAPHORASE DEFICIENCY IN NAVAJO INDIANS. BALSAMO P, HARDY WR, SCOTT EM. pmid=14244100}}
  2. {{J Pediatr Hematol Oncol. 2017 Jan;39(1):42-45. Enzymopenic Congenital Methemoglobinemia in Children of the Republic of Sakha (Yakutia). Burtseva TE1, Ammosova TN, Protopopova NN, Yakovleva SY, Slobodchikova MP. pmid=27879543 }}
  3. {{ Adv Parasitol. 2013;81:133-201. doi: 10.1016/B978-0-12-407826-0.00004-7. G6PD deficiency: global distribution, genetic variants and primaquine therapy. Howes RE1, Battle KE, Satyagraha AW, Baird JK, Hay SI.pmid=23384623}}
  4. Template:Pmid=1917622

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