The Protective Role of Anise Oil in Oxidative Stress and Genotoxicity Produced in Favism.

The metabolic disease favism is an acute hemolytic anemia. Anise oil was obtained from Pimpinella anisum L. seeds (family Apiaceae). The objective of this study was to establish the protective effect of anise oil in favism disorders. Forty-eight male albino rats were divided into six groups: group 1 orally administrated 1 mL distilled water, group 2 orally received 300 mg/kg anise oil, and group 3 orally administrated 100 mg/kg anethole over a seven-day period, group 4 favism-induced rats, group 5 orally administrated 300 mg/kg anise oil and group 6 orally administrated 100 mg/kg anethole once a day over a seven-day period prior to favism induction. The result obtained revealed that oral administration of either anise oil or anethole into normal rats over a seven-day period did not induce any change. Following favism induction, hemoglobin, hematocrit, red and white blood cell counts, serum glucose, blood glutathione, glucose-6-phosphate dehydrogenase, total protein, globulin, alanine and aspartate aminotransferases levels were significantly decreased, while serum alkaline phosphatase and bilirubin showed significant increase. Pretreatment with either anise oil or anethole into favism-induced rats prevented these changes. Favism also induced deoxyribonucleic acid (DNA) damage and prior treatment of anise oil maintained liver DNA content. These results were supported by histopathological evaluation. In conclusion, anise oil pretreatment into favism-induced rats decreased the favism disorders, and this effect was related to the anethole ingredient of the oil.

Ictericia secundaria a una deficiencia de glucosa 6-fosfato deshidrogenasa / Secondary jaundice to deficiency of glucose-6-phosphate dehydrogenase

Presentamos el caso de un varón de 7 años de edad, remitido a nuestra consulta para su valoración por un cuadro de ictericia mucocutánea. Las analíticas orientan hacia una anemia hemolítica y, tras interrogar a la familia, se concreta que el cuadro había comenzado a las 24 horas de la ingesta de habas, por lo que se orienta el cuadro como un probable déficit de glucosa 6-fosfato deshidrogenasa (D-G6PD), confirmándose posteriormente. Se inicia tratamiento de soporte. Las cifras más bajas de hemoglobina y hematocrito se detectan al cuarto día tras la ingesta del agente desencadenante, permaneciendo el paciente prácticamente asintomático. Las cifras analíticas se normalizan completamente a los 14 días del inicio del cuadro. En este caso se detectó la coexistencia de D-G6PD con otra eritropatía, G6PD/HbS (anemia de células falciformes), asociación que ya ha sido descrita en otros trabajos anteriormente. Al alta hospitalaria se proporcionó al paciente una lista de fármacos y alimentos con efecto oxidante, así como asesoramiento genético. Asimismo, nos parece importante resaltar la importancia de excluir la coexistencia de D-G6PD con otras eritropatía (AU)

We describe the case of a seven year old male patient, who was sent to study because of recient jaundice, with final diagnosis hemolitic anemia, beginning up to 24 hours to have consumed beans, and that’s why the initial diagnosis was glucose 6 phosphate dehydrogenase deficiency (G6PD), being confirmed later. Lower numbers of haemoglobin and hematocrito appears on fourth day, remaining practically asymptomatic. Analytical normalized to 14 days. We diagnose also coexistence of G6PD with other erythropathy G6PG/HbS. The patient received the list of food and drugs potentially harmful and genetic counselling. We want to stand out the importance of reject the coexistence with another erythropathy (AU)

Favismo en un niño de 2 años y medio de edad / Favism in a two-and-a-half-year-old boy

El déficit de glucosa 6-fosfato deshidrogenasa (G6PD) es la enfermedad más importante de la vía de derivación de las pentosas y puede ser responsable de una anemia hemolítica inducida por fármacos oxidantes o por ciertas infecciones. El déficit de G6PD es frecuente en grupos étnicos afroamericanos y de la cuenca del mediterráneo. Presentamos el caso de un niño de 2 años y medio de edad de origen sirio que tras haber comido habas, presentó un cuadro de anemia hemolítica grave con 2,6 g de hemoglobina y hematócrito del 6,8 por ciento, que precisó tratamiento con un concentrado de hematíes (AU)

The role of reduced glutathione during the course of acute haemolysis in glucose-6-phosphate dehydrogenase deficient patients: clinical and pharmacodynamic aspects.

Tissue hypoperfusion leads to cellular oxidative and peroxidative damage due to biochemical disorders in the oxygen and substrate metabolism. The metabolic turnover of glutathione (GSH) represents one the main cytoprotective systems against the peroxide attack and the depletion or defect in resynthesis of this compound is accompanied by pathological consequences. In the present study the clinical effects of glutathione depletion were investigated in conditions of acute tissue hypoxia due to marked haemolysis in glucose-6-phosphate dehydrogenase deficient patients (favism syndrome). In these subjects a significant marker of the tissue oxidative damage was represented by the uric acid blood levels, presumably linked to xanthine-hypoxanthine altered metabolism. To antagonize the effects of oxyradical pathology, reduced glutathione was administered to a group of patients and the results confirmed the cytoprotective role played by the GSH supplementation. The GSH action was evident on the tissue metabolism and this supports the opinion that reduced glutathione could represent a new and interesting therapeutic approach in marked and acute hypoxic conditions.

Activity of divicine in Plasmodium vinckei-infected mice has implications for treatment of favism and epidemiology of G-6-PD deficiency.

Intravenous injection of divicine into mice infected with Plasmodium vinckei rapidly killed the parasites and caused haemolysis. Degenerating parasites were observed frequently inside intact circulating erythrocytes, implying that parasite death was not a passive consequence of haemolysis. Both parasite death and haemolysis were prevented by the iron chelator desferrioxamine. In vitro, divicine caused the accumulation of malonyldialdehyde and the depletion of reduced glutathione in normal mouse erythrocytes. Desferrioxamine inhibited the former event, but not the latter. These observations support the hypothesis advanced by Huheey & Martin (Experientia, 31, 1145, 1975) to explain the patchy geographical distribution of glucose-6-phosphate dehydrogenase deficiency in historic malarial areas and also suggest that desferrioxamine, a drug already in clinical use, is a potential treatment for favism and other examples of oxidative haemolysis.