ABSTRACT
Favism shows a striking prevalence in the islands and coastal regions of the Mediterranean Sea and in the Middle East. Since a vast number of clinical reports as well as epidemiological, genetic, and biochemical studies have jointly contributed to the characterization of this disorder, termed "favism", and to the elucidation of its etiology and pathogenesis. Attempts to identify the toxic principle in the fava beans capable of affecting selectively glucose-6-phosphate dehydrogenase (G6PD)-deficient individuals are greatly hampered by the lack of a suitable experimental animal. The fava bean pyrimidine aglycones share with acetylphenylhydrazine and the other noxious drugs the ability to bring about irreversible oxidation of GSH in G6PD-deficient erythrocytes. The hypothesis of N. S Kosower and E. M. Kosower was modified by E. Beutler, who suggested that dopaquinone arising in vivo by oxidation of dopa, rather than dopa itself, might be the causative agent of favism.
