Congenital methemoglobinemia

Category Baby Diseases | August 12, 2017 17:58

disease known as methemoglobinemia.This is the innate biochemical defect of red blood cells.High levels of intracellular hemoglobin is in the form of methemoglobin.Due to the progressive oxidation of iron in hemoglobin of ferro (Fe ++) goes into ferric (Fe + + +) (ferric state).Metgemoglobinovoe iron can not carry oxygen.In normal erythrocytes methemoglobin not greater than 0.4% of total hemoglobin, as it is reduced again in the hemoglobin under the influence of enzymatic chain reaction nearly as fast as it is formed.Under normal conditions, the reduction of methemoglobin faster flavoprotein - koenzimny factor.In congenital methemoglobinemia there is insufficient koenzimnogo factor in red blood cells accumulate up to 40% of methemoglobin.The oxygen in the hemoglobin bound so strongly that this portion of hemoglobin is not involved in the oxidation processes.

clinical picture .The main symptom is cyanosis.The degree of cyanosis depends on the absolute content of methemoglobin in the blood of children.Cy
anosis and diffuse most strongly expressed on the lips, the mouth, the fingers and toes.Cyanosis appears immediately after birth.Having drum your fingers are not marked.Characteristically for cyanosis with methemoglobinemia, which is the skin and mucous membranes it has pale grayish hue.Despite the diffuse cyanosis, shortness of breath is absent or weakly expressed.Develops compensatory polycythemia, as well as reticulocytosis.At a high content of methemoglobin venous blood acquires the color of chocolate.When stirring in the air red color it can not be restored.The presence of methemoglobin in the blood proved spectroscopically.

With a small content of methemoglobin in the blood characteristic ab-sorption bands may be absent.In such cases, the positive results obtained in a quantitative spectrophotometric examination.

Treatment .Assign high doses of vitamin C - 200-500 mg per day.Very good results are obtained by intravenous administration of 1.2 mg per kg body weight of 1% methylene blue solution.When intravenous methylene blue methemoglobin cyanosis and disappear quickly.He turns ferric methemoglobin to ferrous iron in hemoglobin.

Methylene blue is methemoglobin reduction by exposing the enzyme system.To maintain this state of the baby without cyanosis, and may have a 1% solution of methylene blue and calculation of the mouth of 5.3 mg per kg body weight.After stopping treatment methemoglobin level again increases slowly as endogenous cause is not eliminated.

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