Abstract
Summary. Two young patients with congenital methaemoglobinaemia due to NADH‐methaemoglobin reductase deficiency associated with low activity of erythrocytic glutathione reductase (GSSG‐R) are described. Their red cells, labelled with 51Cr and transfused into normal recipients, were susceptible to haemolysis by primaquine with an abnormal pattern of destruction. The administration of riboflavin raised the level of erythrocyte GSSG‐R, but did not alter the level of methaemoglobin. The administration of methylene blue not only reduced the methaemoglobin to almost normal levels, but also increased the activity of GSSG‐R. During methylene blue therapy, their red cells, tagged with 51Cr and transfused into a normal recipient who was receiving methylene blue, were not susceptible to haemolysis by primaquine. Some possible reasons for the frequent association between NADH‐methaemoglobin reductase deficiency and low activity of GSSG‐R are discussed.
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