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Related to congenital methemoglobinemia: methemoglobin test


The presence of methemoglobin in the blood.



the appearance of methemoglobin in the blood as a result of the toxic effect of various chemical substances (nitrates and nitrites, aniline, pyridine) that are capable of converting hemoglobin to methemoglobin (MtHb) upon entering the body through the skin, digestive tract, or lungs. With considerable methemoglobinemia, oxygen starvation, or hypoxia, may result. Methemoglobin may be excreted with the urine (methemoglobinuria); this is sometimes damaging to the kidneys. Methemoglobinemia is treated with antidotes (methylene blue, ascorbic acid) and oxygen therapy.

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As a result, a congenital methemoglobinemia attributable to HbM may be mistakenly diagnosed as carbon monoxide poisoning or toxic sulfhemoglobinemia, as happened with this patient (despite her acknowledgement of a family history of chronic methemoglobinemia at presentation).
Less severe cases of congenital methemoglobinemia may be managed with an antioxidant such as ascorbic acid.

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