G6. PD Deficiency: Background, Pathophysiology, Epidemiology. Nkhoma ET, Poole C, Vannappagari V, Hall SA, Beutler E. The global prevalence of glucose- 6- phosphate dehydrogenase deficiency: a systematic review and meta- analysis. G6pd Deficiency Diet Hemolysis MeaningBlood Cells Mol Dis. May- Jun. 4. 2 (3): 2. Alving AS, Carson PE, Flanagan CL, Ickes CE. Enzymatic deficiency in primaquine- sensitive erythrocytes. Sep 1. 4. 1. 24 (3. G6PD deficiency is a genetic condition caused by a lack of the G6PD enzyme in the blood. Learn about G6PD deficiency symptoms, diagnosis, and treatment. G6PD Deficiency diet should focus on obtaining the necessary nutrients with as little use of precious G6PD (necessary for life) as possible. G6PD Deficiency is an inherited condition found mostly in people of African, Asian and Mediterranean descent which can cause hemolytic anemia. Methemoglobin results from. Vicia faba, also known as the broad bean, fava bean, faba bean, field bean, bell bean, English bean, horse bean, Windsor bean, pigeon bean and tic(k) bean, is a. Medscape - Indication-specific dosing for Plaquenil (hydroxychloroquine sulfate), frequency-based adverse effects, comprehensive interactions, contraindications. G6PD deficiency (Glucose 6-phosphate dehydrogenase) leads to a condition called hemolytic anemia. Causes of G6PD deficiency is an abnormal gene located in the X. Cappellini MD, Fiorelli G. Glucose- 6- phosphate dehydrogenase deficiency. Mason PJ, Bautista JM, Gilsanz F. G6. PD deficiency: the genotype- phenotype association. Kaplan M, Hammerman C. The need for neonatal glucose- 6- phosphate dehydrogenase screening: a global perspective. J Perinatol. 2. 9 Suppl 1: S4. Weng YH, Chiu YW. Clinical characteristics of G6. PD deficiency in infants with marked hyperbilirubinemia. J Pediatr Hematol Oncol. Dhillon AS, Darbyshire PJ, Williams MD, Bissenden JG. Massive acute hemolysis in neonates with glucose- 6- phosphate dehydrogenase deficiency. Arch Dis Child Fetal Neonatal Ed. F5. 34- F5. 36 doi: 1. F5. 34. Severe neonatal jaundice associated with glucose- 6- phosphate dehydrogenase deficiency: pathogenesis and global epidemiology. Acta Paediatr Suppl. Kaplan M, Hammerman C. Severe neonatal hyperbilirubinemia. A potential complication of glucose- 6- phosphate dehydrogenase deficiency. Clin Perinatol. 2. Kaplan M, Hammerman C. Glucose- 6- phosphate dehydrogenase deficiency: a hidden risk for kernicterus. Semin Perinatol. 2. American Academy of Pediatrics Subcommittee on Hyperbilirubinemia. Management of hyperbilirubinemia in the newborn infant 3. Abid S, Khan AH. Severe hemolysis and renal failure in glucose- 6- phosphate dehydrogenase deficient patients with hepatitis E. Am J Gastroenterol. Lai YK, Lai NM, Lee SW. Glucose- 6- phosphate dehydrogenase deficiency and risk of diabetes: a systematic review and meta- analysis. Ann Hematol. 9. 6 (5): 8. Rostami- Far Z, Ghadiri K, Rostami- Far M, Shaveisi- Zadeh F, Amiri A, Rahimian Zarif B. Glucose- 6- phosphate dehydrogenase deficiency (G6. PD) as a risk factor of male neonatal sepsis. Jan- Mar. Parental discussion of G6. PD deficiency and child health: implications for clinical practice. Arch Dis Child. 9. Luzzatto L, Poggi V. Glucose- 6- phosphate dehydrogenase deficiency. Orskin SH, Nathan DG, Ginsburg D, Look AT, Fisher DE, Lux SE, eds. Nathan & Oski's Hematology of Infancy and Childhood. Philadelphia PA: Saunders; 2. Vulliamy TJ, Luzzato L. Glucose- 6- phosphatase dehydrogenase deficiency and related disorders. Blood Principles and Practice of Hematology. Hemolytic anemia and anemia due to blood loss. Harrison’s Principles of Internal Medicine. Mc. Graw- Hill Professional Publishing; 2. Kuzniewicz MW, Wickremasinghe AC, Wu YW, Mc. Culloch CE, Walsh EM, Wi S, et al. Incidence, etiology, and outcomes of hazardous hyperbilirubinemia in newborns. Makarona K, Caputo VS, Costa JR, et al. Transcriptional and epigenetic basis for restoration of G6. PD enzymatic activity in human G6. PD- deficient cells. Bautista JM. Epigenetic therapy reprograms hereditary disease. Gomez- Gallego F, Garrido- Pertierra A, Bautista JM. Structural defects underlying protein dysfunction in human glucose- 6- phosphate dehydrogenase A(- ) deficiency. J Biol Chem. 2. 00. Mar 3. 1. 2. 75 (1. Oppenheim A, Jury CL, Rund D, Vulliamy TJ, Luzzatto L. G6. PD Mediterranean accounts for the high prevalence of G6. PD deficiency in Kurdish Jews. Cappellini MD, Martinez di Montemuros F, De Bellis G, Debernardi S, Dotti C, Fiorelli G. Multiple G6. PD mutations are associated with a clinical and biochemical phenotype similar to that of G6. PD Mediterranean. Martinez di Montemuros F, Dotti C, Tavazzi D, Fiorelli G, Cappellini MD. Molecular heterogeneity of glucose- 6- phosphate dehydrogenase (G6. PD) variants in Italy. Haematologica. 1. Jul- Aug. 8. 2 (4): 4. Cappellini MD, Sampietro M, Toniolo D, Carandina G, Martinez di Montemuros F, Tavazzi D, et al. G6. PD Ferrara I has the same two mutations as G6. PD A(- ) but a distinct biochemical phenotype. Pinto FM, Gonzalez AM, Hernandez M, Larruga JM, Cabrera VM. Sub- Saharan influence on the Canary Islands population deduced from G6. PD gene sequence analysis. Beutler E, Kuhl W, Vives- Corrons JL, Prchal JT. Molecular heterogeneity of glucose- 6- phosphate dehydrogenase A- . Nov 1. 5. 7. 4 (7): 2. Kurdi- Haidar B, Mason PJ, Berrebi A, Ankra- Badu G, al- Ali A, Oppenheim A, et al. Origin and spread of the glucose- 6- phosphate dehydrogenase variant (G6. PD- Mediterranean) in the Middle East. Am J Hum Genet. 4. Karimi M, Martinez di Montemuros F, Danielli MG, Farjadian S, Afrasiabi A, Fiorelli G, et al. Molecular characterization of glucose- 6- phosphate dehydrogenase deficiency in the Fars province of Iran. Haematologica. 8. Betke K, Beutler E, Brewer GJ, et al. Standardization of procedures for the study of glucose- 6- phosphate dehydrogenase: report of a WHO Scientific Group. World Health Organ Tech Rep Ser. Minucci A, Giardina B, Zuppi C, Capoluongo E. Glucose- 6- phosphate dehydrogenase laboratory assay: How, when, and why? Shah SS, Diakite SA, Traore K, Diakite M, Kwiatkowski DP, Rockett KA, et al. A novel cytofluorometric assay for the detection and quantification of glucose- 6- phosphate dehydrogenase deficiency. Domingo GJ, Satyagraha AW, Anvikar A, et al. G6. PD testing in support of treatment and elimination of malaria: recommendations for evaluation of G6. PD tests. Au WY, Ngai CW, Chan WM, Leung RY, Chan SC. Hemolysis and methemoglobinemia due to hepatitis E virus infection in patient with G6. PD deficiency. Ann Hematol. Vicia faba - Wikipedia. This article is about the broad bean plant. For the Broadbean company, see Broadbean (Company). Vicia faba, also known as the broad bean, fava bean, faba bean, field bean, bell bean, English bean, horse bean, Windsor bean, pigeon bean and tic(k) bean, is a species of flowering plant in the vetch and pea family Fabaceae. The origin of this legume is obscure, but it had been cultivated in the Middle East for 8,0. Western Europe. They probably originated in the Near East during the Neolithic Age and by the Bronze Age had spread to Northern Italy. They have been found in lakeside settlements in Switzerland and in Britain at Glastonbury. Remains are reported to have been found in Egyptian tombs. In ancient Rome, they were used in funeral rites. Initiates of the Eleusinian mysteries where studies were done on a ritual that transmogrified participants were said to have a deathlike experience after ingesting the kykeon and would then pass by the home of Kyamites, the Greek demigod of Fava beans. In parts of the English- speaking world, the name . The leaves are 1. Unlike most other vetches, the leaves do not have tendrils for climbing over other vegetation. The flowers are 1–2. Crimson- flowered broad beans also exist, which were recently saved from extinction. Each pod contains 3–8 seeds, round to oval and 5–1. Vicia faba has a diploid (2n) chromosome number of 1. Five pairs are acrocentric chromosomes and one pair is metacentric. However, the definitions of . Along with lentils, peas, and chickpeas, they are believed to have become part of the eastern Mediterranean diet around 6. BC or earlier. Fava bean grows during cool weather when other vetches and clovers are relatively dormant, but does not tolerate heat well. Unlike most legumes, the broad bean can be grown in soils with high salinity, and a wide range of p. H values (4. 5–8. However, it does prefer to grow in rich loams. Broad bean is particularly susceptible to high temperatures during the summer which makes the plants unproductive. Broad beans will grow best in a fertile, well- draining soil with a p. H between 6. 0 and 6. A second planting can be made in early autumn in areas with moderate winters. Seeds should be planted 2. An excellent nitrogen producer, follow on crops can be given half the usual rate of nitrogen. Grows well in nitrogen poor soils. Inoculation of the planting seeds is highly recommended, especially in areas where legumes have not been grown. A longpod variety producing a large harvest of delicious flavoured white seeded beans with up to 9 beans per pod.'Imperial Green Longpod': An excellent variety, plants produce a very heavy harvest with pods growing up to 1. The taste is superb and the beans freeze well. Awarded the RHS Award Of Garden Merit (AGM).'Optica': Lower growing plant, awarded the RHS Award Of Garden Merit (AGM). It is a very heavy cropper with short pods containing 5 white beans. If the pods are picked young they can be eaten whole. Dates from 1. 89. They are usually green or yellow in color, but may be pink, brown, red, or black depending on species and host plant. If aphid infestation is heavy, it may cause yellow and/or distorted leaves, necrotic spots on leaves, and/or stunted shoots. Aphids secrete a sticky, sugary substance called honeydew which encourages the growth of sooty mold on the plant. Beetles can decimate bean crops. Spraying plants with a strong jet of water can help reduce buildup of spider mite populations; if mites become problematic apply insecticidal soap and/or neem oil to plants; certain chemical insecticides may actually increase mite populations by killing off natural enemies (which are also mites) and promoting mite reproduction. Emergence of the disease is favored by warm, humid conditions; fungus overwinters on crop debris on the ground. The fungus causes dark, chocolate- coloured spots on all parts of the plant. Chocolate spot is caused by two Botrytis fungi. Botrytis fabae is the most common cause and only affects broad beans. Botrytis cinerea can cause very similar symptoms, and this fungus also causes grey mould on a very wide range of plants, which can have a severe impact on yield. Long periods of high humidity promote the switch from the non- aggressive phase to the aggressive phase; the aggressive phase of the disease favors low levels of potassium and phosphorus in the soil and overcrowded plants that limit air circulation. Damage caused by the emergence of the disease is worsened by warm, compacted soils, limited soil moisture and poor soil fertility. Xanthomonas axonopodis, a bacterium which causes water- soaked spots on leaves that enlarge and become necrotic; spots may be surrounded by a zone of yellow discoloration; lesions coalesce and give plant a burned appearance; leaves that die remain attached to plant; circular, sunken, red- brown lesion may be present on pods; pod lesions may ooze during humid conditions. Disease can be introduced by contaminates seed; bacteria overwinters in crop debris; disease emergence favored by warm temperatures; spread is greatest during humid, wet weather. Bacterium overwinters in crop residue; disease more severe when foliage is wet for extended periods. Galls on roots which can be up to 3. Glucose- 6- phosphate dehydrogenase (G6. PD) is a cytoplasmic enzyme that is distributed in all cells. G6. PD catalyzes the first step in the hexose monophosphate pathway, and it produces NADPH, which is required for reactions of various biosynthetic pathways as well as for the stability of catalase and the preservation and regeneration of the reduced form of glutathione (GSH). Because catalase and glutathione (via glutathione peroxidase) are essential for the detoxification of hydrogen peroxide, the defense of cells against this compound depends ultimately and heavily on G6. PD. This is especially true in red cells, which are exquisitely sensitive to oxidative damage and in which other NADPH- producing enzymes are lacking. The highest prevalence rates (with gene frequencies in the range of 5 to 2. Africa, in the Middle East, in tropical and subtropical Asia, in some areas of the Mediterranean, and in Papua New Guinea. The most common clinical manifestations are neonatal jaundice and acute hemolytic anemia. Hemolytic anemia occurs when red blood cells are destroyed faster than the body can replace them. The acute hemolytic anemia can be triggered by a number of drugs, by infections, or by the ingestion of fava beans. These manifestations may be life threatening, especially favism in children. The detailed mechanism of hemolysis is not fully known, but it results undoubtedly from the inability of G6. PD- deficient red cells to withstand the oxidative damage produced, directly or indirectly, by the triggering agents mentioned above. Red cell destruction in these acute hemolytic events is largely intravascular and therefore is associated with hemoglobinuria. Fortunately, apart from these episodes of hemolytic anemia, most G6. PD- deficient individuals are entirely asymptomatic. However, a rare subset of G6. PD- deficient patients has, instead, a chronic hemolytic disorder, which may be severe. The high prevalence in malaria- endemic areas of G6. PD mutants that have arisen independently corroborates this notion; indeed, it constitutes an example of convergent evolution through balanced polymorphism. Clinical data further support this notion, although it is not certain whether malaria resistance is a feature of heterozygous females only, or also of hemizygous males. In vitro culture studies have shown that the growth of malaria parasites is impaired in G6. PD- deficient red cells, and that G6. PD- deficient parasitized red cells are phagocytosed by macrophages more effectively than G6. PD normal parasitized red cells. Some epidemiological and in vitro studies suggest the hemolysis resulting from favism acts as protection from malaria, because certain species of malarial protozoa, such as Plasmodium falciparum, are very sensitive to oxidative damage due to deficiency of the glucose- 6- phosphate dehydrogenase enzyme, which would otherwise protect from oxidative damage via production of glutathione reductase. Horse beans, left to mature fully, are usually harvested in the late autumn, and are then eaten as a pulse. The immature pods are also cooked and eaten, and the young leaves of the plant can also be eaten, either raw or cooked as a pot herb (like spinach). Broad beans were a major food of old Mediterranean civilizations, particularly for the Romans and Ancient Greeks. In some cuisines, particularly in France and the United States, the beans' outer skin is removed through blanching. These are popular in China, Malaysia, Colombia, Peru (habas saladas), Guatemala (habas), Mexico (habas con chile), Gilan (North of Iran) and Thailand (where their name (. In central Mexico, mashed fava beans are a common filling for many corn flour- based antojito snacks such as tlacoyos. In Colombia, they are most often used whole in vegetable soups. Dried and salted fava beans are a popular snack in many Latin countries. Broad beans are widely cultivated in the Kech and Panjgur districts of Balochistan Province in Pakistan, and in the eastern province of Iran. They are called bakalaink in the Balochi language, and baghalee in Persian. Regional variations. Becoming somewhat rare in restaurants, Kozli. Broad beans are known instead as kouki. Dried broad beans are eaten boiled, sometimes combined with garlic sauce (skordalia). In Crete, fresh broad beans are shelled and eaten as a companion to tsikoudia, the local alcoholic drink. Favism is quite common in Greece because of malaria endemicity in previous centuries, and people afflicted by it do not eat broad beans. In Rome, Italy, fava beans are either cooked with guanciale or with globe artichokes, as side dish together with lamb or kid, or raw with Pecorino romano. Fave e Pecorino is the traditional dish for 1 May picnic. In Sicily, fava beans are the primary ingredient of Maccu soup .
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