CONGENITAL IDIOPATHIC METHEMOGLOBINEMIA

Abstract

The purpose of this report is to describe the observations in a case of congenital idiopathic methemoglobinemia and the favorable response of the patient to ascorbic acid therapy. Methemoglobinemia of a congenital and spontaneous origin is an unusual condition, infrequently reported in the literature. The occurrence of cyanosis because of a high blood level of methemoglobin in two or more members of the same family is referred to as familial idiopathic methemoglobinemia. Among the cases of this type appearing in the literature, the cyanosis has usually been present since the patient's birth but in a few instances has not been observed until later in childhood. This should not be confused with methemoglobinemia of enterogenic origin, such as is described by Stokvis, 1 Talma, 2 van den Bergh, 3 Gibson and Douglas, 4 Lichtenbelt 5 and Wynter, 6 in which the elevated methemoglobin concentration is associated with autointoxication produced by chronic persistent diarrhea or