Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Jaundice and bilirubin levels varied widely in 85 children with favism. Low bilirubin levels (less than 2 mg/100 ml) with clinically undetectable jaundice were seen in 34 (40%), mild jaundice in 32 (38%), moderate in 16 (19%), and severe (greater than 8 mg/100 ml) in 3 (4%). Bilirubin levels were unrelated to the severity of anaemia or to reticulocytosis. The absence of bilirubinaemia and jaundice in a high proportion of the patients was attributed to the ability of the liver to conjugate large amounts of bilirubin. The extreme bilirubinaemia and jaundice observed in a minority of cases was attributed to the existence of an additional hereditary factor affecting the liver.
 ...
PMID:Jaundice and bilirubin levels in Greek children with favism. 98 25

A child suffering from G6PD deficiency developed a severe haemolytic crisis without an apparent trigger. The possible pathogenetic role of the ingestion of unripe peaches was studied biochemically in this anaemia. We show that an extract from the unripe peach exerts an oxidative challenge on normal as well as on asymptomatic G6PD-deficient erythrocytes. This effect is analogous to that of the favism-inducing agents. The effect of the extract on the patient's red blood cells was more pronounced than on other asymptomatic G6PD-deficient erythrocytes, particularly during his haemolytic crisis. The chemical nature of the deleterious component was not identified. It is suggested that unripe peaches be added to the list of hazards for G6PD-deficient subjects in combination with other factors.
 ...
PMID:Haemolysis in a G6PD-deficient child induced by eating unripe peaches. 650 33

Acute haemolysis due to Glucose-6-Phosphate-Dehydrogenase deficiency is a common disorder in American and African Blacks, in Mediterranean people and among Orientals. The erythrocytes in affected individuals have insufficient reducing power against toxic peroxydes and free radicals generated during metabolism. Normally, affected individuals are without signs of disease, but under the influence of oxydants severe intravascular haemolysis may occur. One of the most important oxydants is the fava bean which, when ingested, may cause acute favism, a condition which has a 10% mortality if not treated properly. We describe a 35 year-old man from Iraq who developed serious haemolytic anemia with a fall in haemoglobin to 6.5 g/100 ml three days after ingestion of fava beans. He was treated with intravenous fluids and blood transfusions. He recovered and was discharged from hospital after nine days. This is the first described case of favism in Norway.
 ...
PMID:[Favism. Acute hemolysis after intake of fava beans]. 949 26

Glukose-6-phosphate dehydrogenase (G6PD) is essential in protecting the red cell from oxidative damage. We report a case of acute haemolysis in a child with G6PD deficiency. Because of the severity of the anaemia, the patient was treated with blood transfusions and recovered fully. There are two main variants of G6PD deficiency (the Mediterranean variant and variant A) with different clinical profiles. Acute haemolytic attacks are induced by certain drugs, by infections or, in the Mediterranean variant, by ingestion of fava beans (favism). Increased awareness of this condition is necessary in Denmark because of increased immigration.
 ...
PMID:[Acute hemolytic anemia caused by glucose-6-phosphate dehydrogenase deficiency]. 1077 18

In a study conducted at Songklanagarind Hospital in the south of Thailand, the subjects were 225 patients (210 boys and 15 girls) with glucose-6-phosphate dehydrogenase (G6PD) deficiency. Favism was found in 3.6% of the G6PD-deficient children. Approximately one half of the G6PD-deficient patients with favism were younger than 2 years. Sudden onset of anemia was found within 1 to 3 days after ingestion of dried fava beans. The classic features of favism, which are pallor, hemoglobinuria, and jaundice, were detected in all cases. To characterize the known G6PD mutations in Thai children, molecular analysis was performed for 8 G6PD-deficient children with favism by a combination of polymerase chain reaction-restriction fragment length polymorphism analysis and amplification refractory mutation system analysis. The G6PD variants in these children were G6PD Kaiping 1388,G-->A; G6PD Mahidol 487,G-->A; G6PD Viangchan 871,G-->A; and uncharacterized mutation with silent mutation 1311,C-->T.
 ...
PMID:Glucose-6-phosphate dehydrogenase variants associated with favism in Thai children. 1651 31

Glucose 6 phosphate dehydrogenase (G6PD) deficiency is the most common disease producing enzymopathy. People with G6PD deficiency cannot cope with oxidative stressors. These patients are asymptomatic until they develop a haemolytic crisis which presents as anaemia and jaundice. The agents known to cause haemolysis in these patients are: oxidant drugs, (primaquine, chloroquine and other anti-malarials), antibiotics, (chloramphenicol, nitrofurantoin, sulphonamides, and all quinolone antibiotics). Chemicals to be avoided are moth balls (napththalene), aniline dyes, and some Chinese herbal medicine (San Chi, Chuan Lian). The classic presentation of exposure to Fava beans (legumes), causing oxidative haemolysis, has led G6PD deficiency to be also known as Favism. The patient we present here had no exposure to any of the known causes of oxidative haemolysis. Instead his precipitating cause strongly suggests raw fenugreek (T foenum-graecum L), a legume used as a herbal treatment for diabetes and previously not known to be a precipitant of haemolysis in G6PD deficiency.
 ...
PMID:Possible fenugreek induced haemolysis in a patient with previously unknown G6PD deficiency. 2178 55

Glucose-6-phosphate dehydrogenase (G6PD) deficiency is a common genetic abnormality known to predispose to acute hemolytic anemia (AHA), which can be triggered by certain drugs or infection. However, the commonest trigger is fava beans (Vicia faba) ingestion, causing AHA (favism), which may be life-threatening especially in children. G6PD deficiency is genetically highly heterogeneous, as nearly 200 different mutations have been observed. We have investigated the hematological features of acute favism in the Palestinian Gaza community that is characterized by the polymorphic coexistence of three different G6PD deficiency genes (G6PD A-, G6PD Cairo, G6PD Med). We have found by comparison to the general population (485 adults and 466 newborns) that children with favism, in terms of relative frequency, G6PD A- was under-represented, whereas G6PD Med was over-represented. We also found that the severity of anemia was significantly greater with G6PD Med and G6PD Cairo than with G6PD A-; and with G6PD Cairo, compared to the other two variants, there was greater hyperbilirubinemia, as well as persistence of mild anemia and reticulocytosis for as long as 4months after recovery from favism. This is the first report determining a differential impact of different G6PD mutations on the clinical features of favism in the same population and the same environment.
 ...
PMID:Favism, the commonest form of severe hemolytic anemia in Palestinian children, varies in severity with three different variants of G6PD deficiency within the same community. 2751 46