PEDIATRICS Vol. 22 No. 3 September 1958, pp. 461-471
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A DEFECT OF GLUTATHIONE METABOLISM IN ERYTHROCYTES FROM PATIENTS WITH A NAPHTHALENE-INDUCED HEMOLYTIC ANEMIA

W. H. Zinkham M.D.1 and B. Childs M.D.1

1 Department of Pediatrics, The Johns Hopkins School of Medicine, and The Harriet Lane Home of The Johns Hapkins Hospital

Four patients are reported who suffered hemobytic anemia after exposure to naphthalene. These were all Negroes; three were male and one female.

All the patients were shown by determinations of concentrations of glutathione in whole blood and the glutathione stability test to possess erythrocytes with a defect of glutathione metabolism.

One of these patients was a newborn infant to whom naphthalene and its metabolites must have been delivered through the placenta, since both he and his mother had profound hemolytic anemia, but she alone ingested moth balls.

In-vitro tests are reported showing the effects of naphthalene and its metabolites on the reduced glutathione of the erythrocytes of the patients. Naphthalene itself is innocous while alpha-naphthoquinone and alpha-naphthol lowered the concentration of reduced glutathione of the erythrocytes in lower dilutions than did beta-naphthoquinone and beta-naphthol.

Repeated stability tests done with the erythrocytes of the infant and covering a period of more than 3 months gave support to the hypothesis that the defect in glutathione metabolism expresses itself only in older erythrocytes.

Submitted on April 2, 1958
Accepted on May 1, 1958