Glucose-6-phosphate dehydrogenase deficiency in Greece.

.I T ORKERS IN ITALY,’ Israel”” and Americ&OU have shown that the V V gltmtathione ( GSH ) instability and decreased glucose-6-phosphate dehydrogenase ( C-6-PD ) activity, which are characteristic of primaquine sensitive imldividuals, are also present in persons who develop acute hemolytic anemia following ingestion of fava beans. The above authorsi ,:i, ii .12 have presented evidence that the biochemical defect in favism has a similar hereditary pattern; it is, most probably, transmnitted by a sex-linked gene of intermediate dominance. Final proof that the erythrocyte defect present in subjects sensitive to fava beans is identical to that of stibjects sensitive to primaquine was obtained by Larizza and his associates who gave primaquine in therapeutic doses to an individtial with a past history of favism and produced a hemolytic reaction. Favism is very common in Greece. Biochemical studies on Greek patients with favism were first reported by one of us (Z-M.L.) in a communication Presented at the 7th European Congress of Hematology in London (1959 ) Until then the cilaracteristic enzymatic defect had been studied only in Americans of Greek extraction.ii The present paper is an extension of this first communication and incltides the findings already reported. The main purpose of this sttidy was to investigate the biochemical (lefect in favism during tile stage of acute hemolysis. The examination of our cases (luring the acute stage was, to a certain extent, imposed tipon us; the severity of their condition made the need for tirgent treatment with blood transftisions imperative and this prevented us from studying them at any other time except that preceding treatment. On the other hand, parents would seldom cooperate and bring their children for examination two to three months after the hemolytic episode. Another reason that stimulated tis to sttidy our patients dtiring the early 1)hase of the hemolytic reaction was the lack of tinanimity in the reports of tile literature concerning the GSH levels, GSH stability and G-6-PD activity in “sensitive” individtials during that period.6 ’13”3’8 Tile recent observation that the biochemical defect is much more severe in Caucasians than it is in Negroes’”’2#{176} has been a further stimtilus to examine the cases of tile present sttidy during the cotirse of actite hemolysis. According to Marks’” young erythrocytes in “sensitive” Caucasians are almost equally