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Methemoglobinemia mostly affects infants under 6 months of age (particularly those under 4 months) due to low hepatic production of methemoglobin reductase. [ 23 ] [ 24 ] The most at-risk populations are those with water sources high in nitrates , such as wells and other water that is not monitored or treated by a water treatment facility.
A methemoglobin level > 1.5 g/dL causes cyanosis. The most common congenital cause is a deficiency in the enzyme cytochrome b5 reductase which reduces methemoglobin in the blood. [22] However, in infants the most common cause of methemoglobinemia is acquired through the ingestion of nitrates (NO − 3) through well water or foods.
The structure of cytochrome b5 reductase, the enzyme that converts methemoglobin to hemoglobin. [1]Methemoglobin (British: methaemoglobin, shortened MetHb) (pronounced "met-hemoglobin") is a hemoglobin in the form of metalloprotein, in which the iron in the heme group is in the Fe 3+ state, not the Fe 2+ of normal hemoglobin.
The most common and successful treatment used to treat patients with high levels of methemoglobinemia is the antidote methylene blue. Methylene blue is already recognized as a product of the reversible reaction fueled by NAPHD methemoglobin reductase, catalyzed by leukcomethylene, to reduce methemoglobin to hemoglobin.
The disorder can cause heart abnormalities and seizures if the amount of methemoglobin in the blood exceeds 20 percent, but at levels between 10 and 20 percent it can cause blue skin without other symptoms. Most of the Fugates lived long and healthy lives. The "bluest" of the blue Fugates, Luna Stacy, had 13 children and lived to age 84. [6]
A mom who was told her daughter had the flu discovered she had something far more dire — after looking online when her toddler’s health continued to deteriorate.
Infants younger than 4 months are at greater risk given that they drink more water per body weight, they have a lower NADH-cytochrome b5 reductase activity, and they have a higher level of fetal hemoglobin which converts more easily to methemoglobin. Additionally, infants are at an increased risk after an episode of gastroenteritis due to the ...
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