The authors report on their experience in nearly 10 years follow-up of four families with hereditary enzymopenic methemoglobinemia. In patients a dangerous elevation of methemoglobin level was never observed, what is probably due to the information of the patients and the family doctor about the nature of the patient's constitution. All gene--carriers were practically healthy. In one family 10 children died from convulsions as newborns. Mental retardation was not present in these four families.

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