Generalized deficiency of the NADH-methemoglobin reductase in congenital methemoglobinemia with neurological symptoms.

Determination of the NADH-methemoglobin reductase (NADH-metHb-R) activity in red cells, lymphocytes and platelets of a two year old Polish girl with congenital methemoglobinemia and severe damage of the central nervous system, as well as in control subjects, was performed. The results permitted us to diagnose a rare case of the generalized deficiency of the NADH-metHb-R.