Disease relevance of Favism

• The biochemical events that take place during acute hemolysis of G6PD-deficient subjects in favism are far from being elucidated [1].
• In one of them favism was the first clinical sign of G6PD deficiency and chronic nonspherocytic hemolytic anemia (CNSHA) was diagnosed later [2].
• Glucose-6-phosphate: a key compound in glycogenosis I and favism leading to hyper- or hypolipidaemia [3].
• It is possible that the hydrolysis product of vicine, divicine or the analogous product from convicine, isouramil, or both, may be involved in toxicity, because these substances are known to be the cause of a hemolytic anemia in some humans that suffer from favism due to consumption of field beans [4].
• Here we have compared the biochemical abnormalities and the atherogenic risk of three different disorders of glucose metabolism including GSD-I (glucose-6-phosphatase deficiency), favism (glucose-6-phosphate dehydrogenase deficiency), and diabetes mellitus which are related to either hyper- or hypolipidaemia [3].

High impact information on Favism

• Deferoxamine and favism [5].
• In one of the commonest, G6PD Mediterranean, which is associated with favism among other clinical manifestations, a single amino acid replacement was found (serine----phenylalanine): it must be responsible for the decreased stability and the reduced catalytic efficiency of this enzyme [6].
• Association between ACP1 and favism: a possible biochemical mechanism [7].
• Active involvement of catalase during hemolytic crises of favism [8].
• To test the hypothesis that an autosomal enzyme is involved in the pathogenesis of favism, we carried out a beta-glucosidase assay in small intestine biopsies from normal subjects and G6PD deficient subjects with or without favism [9].

Chemical compound and disease context of Favism

• In vitro incubation of normal and G6PD-deficient erythrocytes with divicine, a pyrimidine aglycone present in fava beans and strongly implicated in the pathogenesis of favism, reproduces most of these events, including drop of calcium ATPase, increased intracellular calcium, and leakage of erythrocyte potassium [1].
• Lactose absorption in patients with glucose 6-phosphate dehydrogenase deficiency with and without favism [10].
• Divicine has long been thought to be a mediator of an acute hemolytic crisis, known as favism, in susceptible individuals who ingest fava beans (Vicia faba) [11].
• Our results corroborate the concept that intraerythrocytic inhibition of glutathione reductase mimicks the biochemistry of drug-sensitive glucose-6-phosphate dehydrogenase deficiency (favism), an inherited condition which confers protection from malaria [12].
• Correspondingly, the calcium permeability of erythrocytes, estimated as the fraction of intracellular calcium exchangeable with externally added 45Ca2+, was invariably enhanced in favism and returned to normal patterns after several months from the acute hemolytic crisis [13].

Biological context of Favism

• Tf, Gc and Cp phenotypes in favism and G-6-PD deficiency [14].

Anatomical context of Favism

• The endemic occurrence of favism in certain Mediterranean regions provided an investigative opportunity for testing in vivo the validity of claims as to the role of catalase in protecting human erythrocytes against peroxidative injury [8].
• Divicine, a pyrimidine aglycone strongly implicated in the pathogenesis of favism, induces calcium release from intact rat liver mitochondria [15].
• These data suggested that the reduction of SRC-rosetting lymphocytes observed during acute hemolysis of favism can be related to a modifying effect of bilirubin and hemoglobin [16].

Gene context of Favism

• Serum glutamic oxalacetic transaminase, glutamic pyruvic transaminase, gamma-glutamyl transpeptidase and glutamic dehydrogenase levels in favism [17].
• Inactivation of red cell glutathione peroxidase by divicine and its relation to the hemolysis of favism [18].
• Haptoglobin therapy for acute favism: a Japanese boy with glucose-6-phosphate dehydrogenase Guadalajara [19].
• Calpain, the micromolar Ca2+-requiring form of Ca2+-stimulated neutral proteinase purified from human red cells, is remarkably inactivated during autoxidation of divicine (2,6-diamino-4,5-dihydroxypyrimidine), an aglycone implicated in the pathogenesis of favism [20].
• Our case could be due to chronic beta2 receptor stimulation with imbalance of alpha and beta receptor, without any implication of favism [21].

Analytical, diagnostic and therapeutic context of Favism

• RESULTS: Twenty per cent of G6PD deficient subjects with a positive history of favism and 22% of G6PD deficient subjects without a positive history of favism were lactose absorbers compared with 14% lactose absorbers in the control group [10].

References