Severe neurotoxicity due to 5-fluorouracil (FUra) has previously been described in a patient with familial pyrimidinemia. We now report the biochemical basis for both the pyrimidinemia and neurotoxicity in a patient we have recently studied. After administration of a "test" dose of FUra (25 mg/m2, 600 gCi 16-3HJFUra by intravenous bolus) to a patient who had previously developed neurotoxicity a...

Glucose-6-phosphate dehydrogenase (G6PD) is expressed in all tissues, where it catalyses the first step in the pentose phosphate pathway. G6PD deficiency is prevalent throughout tropical and subtropical regions of the world because of the protection it affords during malaria infection. Although most affected individuals are asymptomatic, there is a risk of neonatal jaundice and acute haemolytic...

background and aims: glucose-6-phosphate dehydrogenase (g6pd) is the first enzyme in the route of pentose phosphate metabolism. this route fulfills an effective role in removing oxidant metabolites. deficiency of this enzyme causes decline in energy regeneration of red blood cells and hemolysis. in this study, application of fluorescence staining method in diagnosing the frequency of g6pd defic...

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is the most common human enzyme defect, being present in more than 400 million people worldwide. The global distribution of this disorder is remarkably similar to that of malaria, lending support to the so-called malaria protection hypothesis. G6PD deficiency is an X-linked, hereditary genetic defect due to mutations in the G6PD gene, which ca...

In order to study the survival mechanisms to drought stress for fruit body of Auricularia auricula, soluble carbohydrates and respiratory enzymes were investigated. Fruit bodies were exposed to sunlight and were naturally dehydrated. Samples were taken at different levels of water loss (0%, 10%, 30%, 50% and 70%) to measure the content of soluble sugars and polysaccharides. The activities of ph...

We present a patient with Burkitt's lymphoma who suffered a severe haemolytic crisis after treatment with rasburicase. This case report underlines the high incidence of glucose-6-phosphate dehydrogenase deficiency in some ethnic groups and the importance of a detailed patient and family history before starting treatment, even in case of emergency. Glucose-6-phosphate dehydrogenase is an essenti...

background: jaundice is affecting over 60-80 percent of neonates in the first week of life. glucose-6-phosphate dehydrogenase (g6pd) deficiency, which is an important cause of pathologic hyperbilirubinemia, can lead to hemolytic anemia, jaundice and kernicterus. the present study was performed to determine the prevalence of g6pd deficiency among icteric neonates in shirvan, iran. methods: this ...

in a survey carried out to establish the incidence of erythrocyte glucose­ 6-phosphate-dehydrogenase deficiency, 738 professional blood donors of tehran were examined. the fluorescent spot technique was used for screening and qualitative determination of g-6-pd in erythrocytes. this technique was compared with other methods of g-6-pd enzyme assay and proved to be very reliable. qualitative enzy...

I T HAS BECOME increasingly apparent that many forms of hemolytic disease are due to hereditary enzyme deficiencies affecting the erythrocytes. The drug-induced hemolytic anemias may be due to glucose-fl-phosphate dehydrogenase (G-6-PD ) deficiency, glutathione reductase (GSSG-R) deficiency, or to a deficiency in reduced glutathione (GSFI ). Nonspherocytic congenital hemolytic anemia may be due...