Data was generously offered to the association by the late Ernest Beutler, M.D.
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Notes: (B255). - Bottini, E., Lucarelli, P., Spennati, G.F., Businco, L., and Palmarino, R. Presence in vicia faba of different substances with activity in vitro on GD (-) med red blood cell reduced glutathione. Clin.Chim.Acta 30:831-834, 1970.
- Gaetani, G., Salvidio, E., Pannacciulli, I., Ajmar, F., and Paravidino, G. Absence of haemolytic effects of L-DOPA on transfused G6PD-deficient erythrocytes. Experientia 26:785-786, 1970.
- Loiselet, J. and Chaumeil, J.C. Methode de mesure de la toxicite des extraits de vicia faba sur les globules rouges. Biochim.Biophys.Acta 52:1305-1308, 1970.
- Bottini, E., Lucarelli, P., Agostino, R., Palmarino, R., Bosinco, L., and Antognoni, G. Favism: Association with erythrocyte acid phosphatase phenotype. Science 171:409-411, 1971.
- Flohe, L., Niebch, G., and Reiber, H. Zur wirkung von divicin in menschlichen erythrocyten. Z.Klin.Chem.Klin.Biochem. 9:431-437, 1971.
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- Rattazzi, M.C., Corash, L.M., Van Zanen, G.E., Jaffe, E.R., and Piomelli, S. G6PD deficiency and chronic hemolysis: Four new mutants--relationships between clinical syndrome and enzyme kinetics. Blood 38:205-218, 1971.
- Sartori, E. On the pathogenesis of favism. J.Med.Genet. 8:462-467, 1971.
- Russo, G., Mollica, F., Pavone, L., and Schiliro, G. Hemolytic crises of favism in Sicilian females heterozygous for G-6-PD deficiency. Pediatrics 49:854-859, 1972.
- Symvoulidis, A., Voudiclaris, S., Mountokalakis, T., and Pougounias, H. Acute renal failure in g.-6-P.D. Deficiency. Lancet 2:819-820, 1972.
- Panich, V. and Na-Nakorn, S. Acute hemolysis in G-6-PD Union (Thai) report on four cases. J.Med.Assoc.Thai. 56:241-249, 1973.
- Sroczynska, M. and Sychlowy, A. Fawizm-przelom hemolityczny w przebiegu niedoboru dehydrogenazy glukozo-6-fosforanowej w krwinkach czerwonych. Pol.Tyg.Lek. 28:744-745, 1973.
- Cassimos, C.H.R., Malaka-Zafiriu, K., and Tsiures, J. Urinary d-glucaric acid excretion in normal and G-6-PD deficient children with favism. J.Pediatr. 84:871-872, 1974.
- Cutillo, S. and Meloni, T. Serum concentrations of haptoglobin and hemopexin in favism and thalassemia. Acta Haematol.(Basel) 52:65-69, 1974.
- Fiorelli, G., Podda, M., Corrias, A., and Fargion, S. The relevance of immune reactions in acute favism. Acta Haematol.(Basel) 51:211-218, 1974.
- Snyder, L.M., Edelstein, L., Fortier, N., Cariglia, N., Jacobs, J., and Cipro, D. The protective effect of L-DOPA on Heinz body formation in G6PD deficient red cells. Experientia 30:85-86, 1974.
- Daneshbod, G. Erythrocyte glucose-6-phosphate dehydrogenase in Tehran. Acta Haematol.(Basel) 53:152-157, 1975.
- Huheey, J.E. and Martin, D.L. Malaria, favism and glucose-6-phosphate dehydrogenase deficiency. Experientia 31:1145-1147, 1975.
- Mentzer, W.C. and Collier, E. Hydrops fetalis associated with erythrocyte G-6-PD deficiency and maternal ingestion of fava beans and ascorbic acid. J.Pediatr. 86:565-567, 1975.
- Cutillo, S., Costa, S., Vintuleddu, M.C., and Meloni, T. Salicylamide-glucuronide formation in children with favism and in their parents. Acta Haematol.(Basel) 55:296-299, 1976.
- Kahn, A., Marie, J., Desbois, J.-.C., and Boivin, P. Favism in a Portuguese family due to a deficient glucose-6-phosphate dehydrogenase variant of 'Canton' or 'Canton-like' type 1. Acta Haematol.(Basel) 56:58-64, 1976.
- Gahr, M., Bornhalm, D., and Schröter, W. Biochemische Eigenschaften einer neuen Variante des glucose-6-phosphatdehydrogenase (G-6-PD)-Mangels mit Favismus: G-6-PD Bielefeld. Klin.Wochenschr. 55:379-384, 1977. 62. Harley, J.D., Agar, N.S., and Yoshida, A. Glucose-6-phosphate dehydrogenase variants: Gd (+) Alexandra associated with neonatal jaundice and Gd (-) Camperdown in a young man with lamellar cataracts. J.Lab.Clin.Med. 91:295-300, 1978.
- Todd, D. Genes, beans and Marco Polo. Supplement to the Gazette 26:1-8, 1978.
- Gaetani, G.F., Mareni, C., Salvidio, E., Galiano, S., Meloni, T., and Arese, P. Favism: Erythrocyte metabolism during haemolysis and reticulocytosis. Br.J.Haematol. 43:39-48, 1979.
- Schiliro, G., Russo, A., Curreri, R., Marino, S., Sciotto, A., and Russo, G. Glucose-6-phosphate dehydrogenase deficiency in Sicily. Incidence, biochemical characteristics and clinical implications. Clin.Genet. 15:183-188, 1979.
- Vergnes, H., Riber, A., Bommelaer, G., Amadieu, J., and Brun, H. Gd(-) Muret and Gd(-) Colomiers, two new variants of glucose-6-phosphate dehydrogenase associated with favism. Hum.Genet. 57:332-334, 1981.
- Chevion, M. and Navok, T. A novel method for quantitation of Favism-inducing agents in legumes. Anal.Biochem. 128:152-158, 1983.
- Meloni, T., Forteleoni, G., Dore, A., and Cutillo, S. Favism and hemolytic anemia in glucose-6-phosphate dehydrogenase-deficient subjects in North Sardinia. Acta Haematol.(Basel) 70:83-90, 1983.
- Clark, I.A., Cowden, W.B., Hunt, N.H., Maxwell, L.E., and Mackie, E.J. Activity of divicine in plasmodium vinckei-infected mice has implications for treatment of favism and epidemiology of G-6-PD deficiency. Br.J.Haematol. 57:479-487, 1984.
- Globerman, H., Novak, T., and Chevion, M. Haemolysis in a G6PD-deficient child induced by eating unripe peaches. Scand.J.Haematol. 33:337-341, 1984.
- Mavelli, I., Ciriolo, M.R., Rossi, L., Meloni, T., Forteleoni, G., De Flora, A., Benatti, U., Morelli, A., and Rotilio, G. Favism: a hemolytic disease associated with increased superoxide dismutase and decreased glutathione peroxidase activities in red blood cells. Eur.J.Biochem. 139:13-18, 1984.
Notes: Red blood cells of favism patients with acute hemolytic crisis have markedly more superoxide dismutase (superoxide:superoxide oxidoreductase, EC 1.15.1.1) and less glutathione peroxidase (glutathione:hydrogenperoxide oxidoreductase, EC 1.11.1.9) than either normal controls, glucose-6-phosphate dehydrogenase-deficient subjects or favism patients outside hemolytic crisis. This altered value of the two enzyme activities is not due to increased reticulocyte content of blood. The electrophoretic triplet pattern of superoxide dismutase is also changed, with significant increase of the most positively charged band. Similar modifications of the two enzyme activities are observed after treatment of normal red blood cells with high concentrations of divicine and ascorbate, which are redox compounds that are contained in fava seeds. This treatment produces no hemolysis, but leads to hemolysis if the treated cells are resuspended in the homologous plasma. These results suggest a possible role of active oxygen s - De Flora, A., Benatti, U., Guida, L., Forteleoni, G., and Meloni, T. Favism: Disordered erythrocyte calcium homeostasis. Blood 66:294-297, 1985.
- Eber, S.W., Gahr, M., and Schröter, W. Glucose-6-phosphate dehydrogenase (G6PD) Iserlohn and G6PD Regensburg: Two new severe enzyme defects in German families. Blut 51:109-115, 1985. 74. Ekert, H. and Rawlinson, I. Deferoxamine and favism. N.Engl.J.Med. 312:1260, 1985. 75. Hu, X. and Du, C. Studies on the etiology and pathogenesis of favism. VII. In vitro study of the hemolytic substance in fava beans (Vicia faba). Chinese J.Pathophysiol. 1:16-23, 1985.
- Stockley, R., Dawson, A., and Slade, R. Favism in two British women. Lancet 2:1013, 1985.
- Turrini, F., Naitana, A., Mannuzzu, L., Pescarmona, G., and Arese, P. Increased red cell calcium, decreased calcium adenosine triphosphatase, and altered membrane proteins during fava bean hemolysis in glucose-6-phosphate dehydrogenase-deficient (Mediterranean variant) individuals. Blood 66:302-305, 1985.
- Golenser, J., Miller, J., Spira, D.T., Kosower, N.S., Vande Waa, J.A., and Jensen, J.B. Inhibition of the intraerythrocytic development of Plasmodium falciparum in glucose-6-phosphate dehydrogenase deficient erythrocytes is enhanced by oxidants and by crisis form factor. Trop.Med.Parasitol. 39:273-276, 1988.
Notes: In this study, we have examined P. falciparum development in untreated normal or G6PD deficient erythrocytes as well as in parasitized cells exposed to isouramil--a fava bean extract known to induce oxidant stress in G6PD deficient erythrocytes (a condition known as favism), to diamide--a thiol oxidizing agent and to crisis form factor (CFF)--a non-immunoglobulin product found in the sera of man immune to malaria. We observed a significant retardation in intraerythrocytic development of parasites cultured in the G6PD deficient cells in comparison with parasites grown in the normal ones. Plasmodia within the G6PD deficient erythrocytes were markedly more sensitive to the inhibitory activity of CFF or the oxidizing agents diamide and isouramil. Mature stages of the parasites in both normal and G6PD deficient erythrocytes were more vulnerable than young ring forms, to oxidizing agents. The overall results show that the genetic trait of the host cell and the degree of maturation of the plasmodia are both crucia - Calabr, V., Cascone, A., Malaspina, P., and Battistuzzi, G. Glucose-6-phosphate dehydrogenase (G6PD) deficiency in southern Italy: A case of G6PD A(-) associated with favism. Haematologica (Pavia) 74:71-73, 1989. 80. Wong, W.Y., Powars, D., and Williams, W.D. 'Yewdow'-induced anemia. West.J.Med. 151:459-460, 1989.
- Arese, P. and De Flora, A. Denaturation of normal and abnormal erythrocytes II. Pathophysiology of hemolysis in glucose-6-phosphate dehydrogenase deficiency. Semin.Hematol. 27:1-40, 1990.
- Galiano, S., Gaetani, G.F., Barabino, A., Cottafava, F., Zeitlin, H., Town, M., and Luzzatto, L. Favism in the African type of glucose-6-phosphate dehydrogenase deficiency (A-). BMJ 300:236, 1990.
- Dess, S., Batetta, B., Spano, O., Pulisci, D., Mulas, M.F., Muntoni, S., Armeni, M., Sanna, C., Antonucci, R., and Pani, P. Serum lipoprotein pattern as modified in G6PD-deficient children during haemolytic anaemia induced by fava bean ingestion. Int..J.Exp.Pathol. 73:157-160, 1992.
Notes: In the present study. plasma lipid concentrations were determined at different times after admission in sera from G6PD-deficient children during haemolytic crisis induced by fava bean ingestion. Reductions in total, LDL and HDL cholesterol were found in association with the maximum of bone marrow hyperplasia. A return towards normal values occurred with regression of the disease. No changes in other lipid parameters were observed. These data suggest that alterations of lipoprotein pattern, other than in experimental animals. are also present in humans with non-malignant proliferative processes. These changes appear to be a consequence of the disease, probably due to an increased utilization of cholesterol by proliferating cells. AUTHOR. - Meloni, T., Forteleoni, G., and Meloni, G.F. Marked decline of favism after neonatal glucose-6-phosphate dehydrogenase screening and health education: The northern Sardinian experience. Acta Haematol.(Basel) 87:29-31, 1992.
Notes: Favism is a potentially fatal manifestation of glucose-6-phosphate dehydrogenase (G6PD) deficiency, and it is therefore a public health problem in areas where this genetic abnormality is common. In the district of Sassari (northern Sardinia), the frequency of G6PD male hemizygotes is approximately 7.5%, and therefore all newborns since 1971 have been screened for G6PD deficiency. We have analyzed the incidence of favism in this community in two 10-year periods: (1) 1961-1970; and (2) 1981-1990. In period 1, there were 508 cases of favism, of which 76% occurred in boys. In period (2) there were 144 cases of favism, of which only 52% in boys. Thus, between the two periods there was an overall decrease in the incidence of favism of 75%, whereas the proportion of girls affected has approximately doubled. These data suggest that neonatal screening and health education programs can produce a substantial decrease in the number of cases of favism, and that the relative increase in favism in girls is possible. - Nafa, K., Reghis, A., Osmani, N., Baghli, L., Benabadji, M., Kaplan, J.-C., Vulliamy, T.J., and Luzzatto, L. G6PD Aures: A new mutation (48 Ile-->Thr) causing mild G6PD deficiency is associated with favism. Hum.Mol.Genet. 2:81-82, 1993.
- Ohga, S., Higashi, E., Nomura, A., Matsuzaki, A., Hirono, A., Miwa, S., Fujii, H., and Ueda, K. Haptoglobin therapy for acute favism: A Japanese boy with glucose-6-phosphate dehydrogenase Guadalajara. Br.J.Haematol. 89:421-423, 1995.
Notes: We report the case of a 2-year-old Japanese boy with acute favism who was treated with human haptoglobin products. He had been exhibiting chronic nonspherocytic haemolytic anaemia until the diagnosis of glucose-6-phosphate dehydrogenase (G6PD) deficiency when 14 months old. He suffered a favic crisis at 24 months of age, when the administration of haptoglobin was effective for relieving bilirubinaemia and haemoglobinuria. Serum-free Hb rapidly decreased to normal levels despite the sustained level of serum lactate dehydrogenase. His G6PD gene was G6PD Guadalajara. This is the first application of haptoglobin therapy for acute favism and the first reported case of Japanese G6PD deficiency with typical favic crisis. Haptoglobin treatment might be helpful for managing the haemolytic crisis in the disease.