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methemoglobinemia
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blood cells,
methemoglobinemia, the iron, in the hemoglobin molecule, red blood pigment, defective, making it unable to carry oxygen effectively, tissues, methemoglobinemia inherited, congenital, inherited, 2 forms of inheritable methemoglobinemia, type 1, type 2, most hereditary cases, type 2, a deficiency, in the enzyme cytochrome b5 reductase, inheritable type, hemoglobin m disease, type 1, an autosomal dominant condition, need one affected parent to inherit it, characterized, inability to convert methemoglobin back to hemoglobin, causes, few problems, acquired methemoglobinemia exposure to certain chemicals, cause an increase, in the production of methemoglobin, chemicals include nitrites, additives, prevent spoilage of meat, anesthetics benzocaine, xylocaine, certain antibiotics, dapsone, chloroquine, benzene, acquired methemoglobinemia severe in certain cases, require treatment, in most mild cases, no treatment, than avoiding the offending medication, chemical, in patients, type 2 congenital cytochrome b5 reductase deficiency, mental retardation, developmental delay, failure to thrive, have seizures, neurologic problems, most die in infancy, in most patients, congenital methemoglobinemia, type 1, a bluish discoloration, skin, mucous membranes, in patients, acquired methemoglobinemia from chemical, drug exposure, headache, fatigue, shortness of breath, lack of energy, potentially shock, seizures, death, cyanosis, bluish skin color, a normal level of oxygen, in the blood, pa02 on blood gas, abnormal color optical spectrum of hemoglobin, incubation of blood, methylene blue, differentiate between cytochrome b5r deficiency, hemoglobin m disease, medications, treat this disorder, methylene blue, 1 - 2 grams of ascorbic acid daily, to treat type 1 or 2 b5r deficiency, methylene blue dangerous in patients, at risk, an inherited blood disease, g6pd deficiency, should not be used, cyanosis, hemoglobin m disease tends to be benign but, does not respond to treatment, cyanosis, exposure to an offending chemical is treated by avoiding that chemical, blood, exchange transfusion, patient in shock, repeated doses of iv methylene blue, in acute exposure, shock, seizures, death, family history of methemoglobinemia, develop symptoms of this disorder, emergency services immediately, severe shortness of breath, genetic counseling, prospective parents, a family history of methemoglobinemia.
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