Favism
Glucosephosphate Dehydrogenase Deficiency
Pyrimidinones
Vicia faba
Glucosephosphate Dehydrogenase
Favism: divicine hemotoxicity in the rat. (1/26)
Favism is an acute hemolytic anemia known to occur in susceptible individuals who ingest fava beans. Susceptibility to favism is conferred by a genetic deficiency in erythrocytic glucose-6-phosphate dehydrogenase (G6PD) activity. Although the fava bean pyrimidine aglycones, divicine and isouramil, have been implicated in the onset of favism in humans, the lack of a well-defined experimental animal model for favism has hampered progress in elucidating the mechanism underlying hemotoxicity. We have examined whether a favic-like response could be provoked in G6PD-normal rats treated with synthetic divicine. Intraperitoneal administration of divicine to rats preloaded with 51Cr-tagged erythrocytes resulted in a severe, dose-dependent decrease in blood radioactivity (TD50 approximately 0.5 mmol/kg) within 24 h. The increased rate of removal of blood radioactivity was accompanied by a rapid decline in reduced glutathione levels in the blood, decreased hematocrits, marked hemoglobinuria, splenic enlargement, and reticulocytosis. In vitro exposure of 51Cr-tagged red cells to divicine before their re-administration to isologous rats also resulted in a sharp, concentration-dependent decrease in erythrocyte survival in vivo (TC50 approximately 1.5 mM), and these divicine-damaged red cells were removed from the circulation by the spleen. These data demonstrate that a favic response can be induced in G6PD-normal rats treated with divicine, and that hemolytic activity can be reproduced in isolated red cells under conditions that will allow a direct examination of the mechanism underlying this hemotoxicity. (+info)Favism: effect of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate shunt activity, morphology, and membrane skeletal proteins. (2/26)
Favism is an acute anemic crisis that can occur in susceptible individuals who ingest fava beans. The fava bean pyrimidine aglycone divicine has been identified as a hemotoxic constituent; however, its mechanism of toxicity remains unknown. We have shown recently that divicine can induce a favic-like response in rats and that divicine is directly toxic to rat red cells. In the present study, we have examined the effect of hemotoxic concentrations of divicine on rat erythrocyte sulfhydryl status, hexose monophosphate (HMP) shunt activity, morphology, and membrane skeletal proteins. In vitro exposure of rat red cells to divicine markedly stimulated HMP shunt activity and resulted in depletion of reduced glutathione with concomitant formation of glutathione-protein mixed-disulfides. Examination of divicine-treated red cells by scanning electron microscopy revealed transformation of the cells to an extreme echinocytic morphology. SDS-PAGE and immunoblotting analysis of the membrane skeletal proteins indicated that hemotoxicity was associated with the apparent loss of skeletal protein bands 2.1, 3, and 4.2, and the appearance of membrane-bound hemoglobin. Treatment of divicine-damaged red cells with dithiothreitol reversed the protein changes, which indicated that the observed alterations were due primarily to the formation of disulfide-linked hemoglobin-skeletal protein adducts. The data suggest that oxidative modification of hemoglobin and membrane skeletal proteins by divicine may be key events in the mechanism underlying favism. (+info)Acid phosphatases. (3/26)
Acid phosphatases (APs) are a family of enzymes that are widespread in nature, and can be found in many animal and plant species. Mystery surrounds the precise functional role of these molecular facilitators, despite much research. Yet, paradoxically, human APs have had considerable impact as tools of clinical investigation and intervention. One particular example is tartrate resistant acid phosphatase, which is detected in the serum in raised amounts accompanying pathological bone resorption. This article seeks to explore the identity and diversity of APs, and to demonstrate the relation between APs, human disease, and clinical diagnosis. (+info)Serum lipoprotein pattern as modified in G6PD-deficient children during haemolytic anaemia induced by fava bean ingestion. (4/26)
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. (+info)An Ashkenazi Jewish woman presenting with favism. (5/26)
The case of a 44 year old Ashkenazi Jewish woman of Russian origin who presented with a typical clinical and haematological picture of favism is reported. There was initial difficulty in confirming glucose-6-phosphate dehydrogenase (G6PD) deficiency because the enzyme concentrations were normal at presentation, but later fell to a concentration compatible with heterozygosity for the Mediterranean type of G6PD deficiency. The diagnosis was also later confirmed by gene analysis. The reasons for the difficulties in the initial confirmation of the diagnosis and the normal G6PD enzyme activity at presentation are discussed. (+info)Acute massive haemolysis in children with glucose-6-phosphate dehydrogenase deficiency. (6/26)
We report seven consecutive episodes of acute massive haemolysis accompanied by symptomatic anaemia and gross haemoglobinuria in six boys with glucose-6-phosphate dehydrogenase deficiency seen in a regional hospital during a 12-year period. They presented at a mean age of 5.5 years (range, 1.5-11.3 years) with trough haemoglobin levels between 35 and 84 g/L. Two children developed transient renal impairment. Five children required erythrocyte transfusion, of whom one underwent exchange transfusion during the oliguric phase. Three patients required intensive care but all recovered from the haemolysis. The probable precipitating factors included consumption of fava beans (n=2), exposure to mothballs (n=1), treatment with herbal medicine or intramuscular injection of unknown nature (n=3), and upper respiratory tract infection (n=1). Although uncommon, acute massive haemolysis remains a life-threatening complication in children with glucose-6-phosphate dehydrogenase deficiency. Improvement in patient education and public health measures is suggested. (+info)Molecular basis of glutathione reductase deficiency in human blood cells. (7/26)
Hereditary glutathione reductase (GR) deficiency was found in only 2 cases when testing more than 15 000 blood samples. We have investigated the blood cells of 2 patients (1a and 1b) in a previously described family suffering from favism and cataract and of a novel patient (2) presenting with severe neonatal jaundice. Red blood cells and leukocytes of the patients in family 1 did not contain any GR activity, and the GR protein was undetectable by Western blotting. Owing to a 2246-bp deletion in the patients' DNA, translated GR is expected to lack almost the complete dimerization domain, which results in unstable and inactive enzyme. The red blood cells from patient 2 did not exhibit GR activity either, but the patient's leukocytes contained some residual activity that correlated with a weak protein expression. Patient 2 was found to be a compound heterozygote, with a premature stop codon on one allele and a substitution of glycine 330, a highly conserved residue in the superfamily of NAD(P)H-dependent disulfide reductases, into alanine on the other allele. Studies on recombinant GR G330A revealed a drastically impaired thermostability of the protein. This is the first identification of mutations in the GR gene causing clinical GR deficiency. (+info)Association between ACP(1) genetic polymorphism and favism. (8/26)
(+info)Favism is a genetic disorder that results in a sensitivity to broad beans (Vicia faba) and related plants. It is most commonly found in populations from the Mediterranean, Middle East, and Asia. The disorder is caused by a deficiency of the enzyme glucose-6-phosphate dehydrogenase (G6PD), which is necessary for protecting red blood cells from damage.
When individuals with favism eat broad beans or inhale their pollen, the beans' metabolites can cause the release of harmful oxidative agents that destroy red blood cells, leading to hemolytic anemia. Symptoms of favism can include weakness, fatigue, abdominal pain, dark urine, and jaundice. In severe cases, it can lead to kidney failure, seizures, or even death.
Avoiding broad beans and related plants is the primary treatment for favism. In some cases, blood transfusions or medications that boost red blood cell production may be necessary to manage symptoms. It's important to note that not all people with G6PD deficiency will develop favism, and not all people with favism have G6PD deficiency.
Glucose-6-Phosphate Dehydrogenase (G6PD) deficiency is a genetic disorder that affects the normal functioning of an enzyme called G6PD. This enzyme is found in red blood cells and plays a crucial role in protecting them from damage.
In people with G6PD deficiency, the enzyme's activity is reduced or absent, making their red blood cells more susceptible to damage and destruction, particularly when they are exposed to certain triggers such as certain medications, infections, or foods. This can lead to a condition called hemolysis, where the red blood cells break down prematurely, leading to anemia, jaundice, and in severe cases, kidney failure.
G6PD deficiency is typically inherited from one's parents in an X-linked recessive pattern, meaning that males are more likely to be affected than females. While there is no cure for G6PD deficiency, avoiding triggers and managing symptoms can help prevent complications.
Pyrimidinones are a class of heterocyclic organic compounds that contain a pyrimidine ring fused with a ketone group. The basic structure of a pyrimidinone consists of two nitrogen atoms and four carbon atoms in a six-membered ring, with a carbonyl (C=O) group attached to one of the carbon atoms.
In a medical context, pyrimidinones are important because many naturally occurring and synthetic compounds that contain this structure have been found to have biological activity. For example, some pyrimidinones have antiviral, antibacterial, or anticancer properties, making them useful in the development of new drugs for various medical conditions.
One well-known drug that contains a pyrimidinone ring is the antiviral medication Ribavirin, which is used to treat hepatitis C and certain viral hemorrhagic fevers. Other pyrimidinones are being studied for their potential therapeutic benefits in areas such as cancer therapy, neuroprotection, and inflammation.
'Vicia faba' is the scientific name for the fava bean plant, which belongs to the legume family (Fabaceae). It is also known as broad bean or horse bean. The plant is widely cultivated as a vegetable crop, and its seeds, pods, and young leaves are all edible. Fava beans are rich in proteins, dietary fiber, vitamins, and minerals, making them an essential component of many diets around the world. However, some people may have an adverse reaction to fava beans due to a genetic disorder called favism, which can cause hemolytic anemia.
Glyceraldehyde-3-phosphate dehydrogenase (GAPDH), also known as Glucosephosphate Dehydrogenase, is an enzyme that plays a crucial role in cellular metabolism, particularly in the glycolytic pathway. It catalyzes the conversion of glyceraldehyde 3-phosphate (G3P) to 1,3-bisphosphoglycerate (1,3-BPG), while also converting nicotinamide adenine dinucleotide (NAD+) to its reduced form NADH. This reaction is essential for the production of energy in the form of adenosine triphosphate (ATP) during cellular respiration. GAPDH has been widely used as a housekeeping gene in molecular biology research due to its consistent expression across various tissues and cells, although recent studies have shown that its expression can vary under certain conditions.
Vicine
X-linked recessive inheritance
Pea
Bean
Divicine
Marcello Siniscalco
Habibollah Hedayat
Methylene blue
Vicia bithynica
Human genetic resistance to malaria
4-Aminosalicylic acid
Glucose-6-phosphate dehydrogenase deficiency
Pharmacogenomics
Lidia Mannuzzu
Vicia faba
Aharon Razin
Food intolerance
Food and drink prohibitions
Primaquine
Elimination diet
Lethal allele
Glutathione reductase
Nitrogen dioxide poisoning
Vicia
List of MeSH codes (C16)
List of MeSH codes (C15)
James E. Bowman
Sardinian people
List of MeSH codes (C21)
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G6PD6
- Although I initially stumbled across favism while researching my first book, Living with Food Intolerance , some twenty years ago, I hadn't realised it could pose such a risk to those those with acute G6PD deficiency. (allergy-insight.com)
- Risk factors for G6PD deficiency or favism must be assessed. (nih.gov)
- Hemolytic reactions (moderate to severe) may occur in individuals with G6PD deficiency and in individuals with a family or personal history of favism. (nih.gov)
- In humans with the G6PD genetic deficiency, these compounds lead to favism, a type of anemia. (saskpulse.com)
- Clinical manifestations of GR deficiency are very similar to that of glucose-6-phosphate dehydrogenase (G6PD) deficiency (favism and severe neonatal jaundice). (enerca.org)
- have a rare blood problem called glucose-6-phosphate dehydrogenase (G6PD) deficiency or favism. (krystexxa.com)
Glucose-6-Phosphat2
Fava beans3
- Congenital GR deficiency is associated with acute haemolytic crisis after oxidant drugs of fava beans ingestion (favism). (enerca.org)
- Many Italian cities have launched ordinances banning the cultivation of fava beans inside tons of of meters of colleges or the properties of the weak, as some favism victims say a mere whiff of the insidious fava pollen can set off an assault. (thebusinessnext.com)
- Favism is a severe reaction to fava beans and or their pollen. (seeds-gallery.com)
Deficiency3
- But one of the most sensible explanations is the fact that in some people (those suffering from the genetic deficiency known as favism), eating raw broad beans can provoke a serious allergic reaction (severe haemolytic anaemia) and could lead to death. (cooksister.com)
- Metabolic food reactions are due to inborn or acquired errors of metabolism of nutrients, such as in lactase deficiency, phenylketonuria and favism. (olsreview.com)
- Vicine and convicine are anti-nutritional compounds that when present in high levels can give rise to favism in humans (those that have a deficiency in the activity of a particular enzyme) and can give performance issues when fed in animal feeds. (farmersguide.co.uk)
Vicia1
- favism, Vicia faba for karyotype FigureB65. (forexsignal300.com)
Haemolytic1
- It causes haemolytic anaemia, called favism. (wikipedia.org)
Allergies1
- The importance of plants as agents of illness and disease (including allergies and favism) will also be discussed. (laurentian.ca)
Broad2
- Favism is not a food allergy, neither is it an autoimmune condition, and it can only be considered a food intolerance in that those affected by favism cannot 'tolerate' broad beans. (allergy-insight.com)
- Important Info : A small percentage of people suffer from 'favism' an allergy to the 'fava' or 'broad' bean, which can be fatal. (backyardgardener.com)
People1
- That can cause favism in people who have a variant glucose-6-phosphate dehydrogenase. (seeds-gallery.com)
Hemolytic anemia1
- Favism is a genetic disorder which causes hemolytic anemia. (sustainewe.org.nz)
G6PD Deficiency4
- G6PD Deficiency: Favism [Internet]. (medlineplus.gov)
- Risk factors for G6PD deficiency or favism must be assessed. (nih.gov)
- Hemolytic reactions (moderate to severe) may occur in individuals with G6PD deficiency and in individuals with a family or personal history of favism. (nih.gov)
- have a rare blood problem called glucose-6-phosphate dehydrogenase (G6PD) deficiency or favism. (krystexxa.com)
Haemolytic anaemia1
- It causes haemolytic anaemia, called favism. (wikipedia.org)
Hereditary2
- There is a hereditary condition, Favism, which causes an allergic-like reaction to fava or broad beans . (foodreference.com)
- Favism, named after the fava bean, is a hereditary disease that can cause significant levels of hemolysis. (perfusiontheory.com)
Disorder1
- In this later case, the disorder is known as favism . (bvsalud.org)
Jaundice2
- Indications of such a sudden attack of favism are dark urine, pallor, jaundice, abdominal pain and in most cases fever. (wikipedia.org)
- People with favism react to external factors that trigger jaundice during babyhood the most. (gurmebebek.com)
Infants1
- In a review of 67 cases of favism over a 3-year period by a physician in Cyprus, 2 of the cases were breastfed infants, aged 5 and 9 months. (nih.gov)
Genetic1
- What is Favism?It is a genetic disease. (oxxo.life)
Dehydrogenase1
- Favism by proxy in nursing glucose-6-phosphate dehydrogenase-deficient neonates. (nih.gov)
Anemia1
- Favism can cause anemia by destroying red blood cells ( 15 , 16 , 17 ). (healthline.com)
Severe1
- Favism via breastmilk can be quite severe. (nih.gov)
Cases1
- It should not be used in cases with favism disease, widespread infection, thyroid dysfunction, and pregnant women. (profdrokanbalcioglu.com)
Individuals1
- Such an association is likely coincidental because favism is common among individuals from the family's place of origin. (medscape.com)
High1
- An estimated, 30,000 to 40,000 black male G6PDD babies, a high risk group for favism, are born annually in the United States (2). (perfusiontheory.com)
Background1
- The purpose of this site is to offer the reader a background information on favism. (g6pd.org)
Content1
- While all effort has been made to ensure that the information offered as part of this site is correct and up-to-date, neither the authors of included content, nor the Associazione Italiana Favism - Deficit di G6PD, which created the site, nor the volunteers who are now administering it, and third party information providers warrant that the information is accurate and complete. (g6pd.org)