LABORATORY AND CLINICAL STUDIES IN CONGENITAL METHEMOGLOBINEMIA

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Abstract
Congenital idiopathic methemoglobinemia in a 12 year old boy was corrected by the administration of either ascorbic acid or methylene blue, the latter being more efficient. The oxygen dissociation curve was normal but as would be expected the oxygen-carrying capacity of the blood was increased after methylene blue was given. Arterial oxygen saturation was determined by an oximeter to demonstrate the increased oxygen-carrying capacity after methylene blue. Para-amino propriophenone administration caused an increase in methemoglobin level but not to the maximum value for this patient. Sodium nitrite by mouth did give the maximum level. Enzyme studies revealed no difference in oxygen uptake of hexosediphosphate either with or without methylene blue in normal or methemoglobinemic cells in a medium which contained glucose. No "cytochrome-reductase like" activity was demonstrated in the methemoglobinemic cell. No definitive evidence exists to support the belief that reduced co-enzyme I reacts directly with methemoglobin in normal cells.