vitamin-k-semiquinone-radical and Glucosephosphate-Dehydrogenase-Deficiency

Topics: Anemia, Hemolytic; Aniline Compounds; Animals; Antimalarials; Catalase; Dihydroxyphenylalanine; Erythrocytes; Female; Genes; Glucosephosphate Dehydrogenase Deficiency; Glutathione; Heterozygote; Humans; Hydrogen Peroxide; Mice; Peroxidases; Phenylhydrazines; Primaquine; Quinolines; Radiation Effects; Sex Chromosomes; Triazoles; Ultraviolet Rays; Vitamin K

Individuals with celiac disease (CD) are predisposed to a number of haematological abnormalities including anaemia secondary to malabsorption of iron, vitamin B12 or folate; anaemia of chronic disease and coagulopathy secondary to vitamin K deficiency. Correction of coagulopathy with vitamin K is necessary before endoscopic biopsy in patients with suspected CD. However, vitamin K causes haemolysis in glucose-6 phosphate-dehydrogenase deficiency.. When vitamin K administration becomes necessary for correction of coagulopathy in patients with CD; glucose-6 phosphate-dehydrogenase deficiency should be considered.

Topics: Celiac Disease; Contraindications; Diet, Gluten-Free; Endoscopy, Gastrointestinal; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Hemolysis; Humans; Infant; Intestinal Mucosa; Male; Vitamin K; Vitamin K Deficiency

Topics: Adult; Glucosephosphate Dehydrogenase Deficiency; Hemolysis; Humans; Male; Textbooks as Topic; Vitamin K

Topics: Animals; Erythrocytes; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Humans; Male; Phenylhydrazines; Plasmodium falciparum; Vitamin K

Hemoglobin in glucose-6-phosphate dehydrogenase (G6PD)-deficient erythrocytes is abnormally vulnerable to oxidative denaturation, which may release ferriheme, a known cytolytic agent. We found 13.3 nmol of ferriheme in G6PD-deficient erythrocyte membranes (per gram of total erythrocyte hemoglobin) using a spectrophotometric assay, as compared to 9.8 in normal membranes (P less than .05). After incubation of erythrocytes with 250 mumol/L menadione, an oxidant drug, the values increased by 37.4 nmol in G6PD-deficient membranes and by 26 in normal membranes (P less than .005), indicating increased hemoglobin denaturation. To verify that hemoglobin denaturation in G6PD-deficient erythrocytes releases ferriheme in a form available to interact with other ligands, [14C]-chloroquine binding to intact erythrocytes was measured. With an initial concentration of 5 mumol/L chloroquine in a medium containing no menadione, an excess of 14.8 nmol of chloroquine was bound in G6PD-deficient erythrocytes (per gram of hemoglobin) as compared to normal erythrocytes (P less than .005). In the presence of 250 mumol/L menadione, chloroquine binding increased by 17.9 nmol in G6PD-deficient and by 7.2 in normal erythrocytes (P less than .005). These results indicate that ferriheme becomes available to interact with endogenous ligands and, thus, to mediate menadione-induced hemolysis in patients with G6PD deficiency. Furthermore, the increase in ferriheme may mediate the selective toxicity of menadione for Plasmodium falciparum parasites growing in G6PD-deficient erythrocytes. Ferriheme release in response to the intraerythrocytic oxidant stress introduced by malaria parasites also may account for the resistance to malaria afforded by G6PD deficiency. This is a US government work. There are no restrictions on its use.

Topics: Chloroquine; Erythrocyte Membrane; Ferric Compounds; Glucosephosphate Dehydrogenase Deficiency; Heme; Hemolysis; Humans; Malaria; Male; Vitamin K

Topics: Adult; Cells, Cultured; Erythrocytes; Fetal Hemoglobin; Glucosephosphate Dehydrogenase Deficiency; Glutathione; Humans; Malaria; Oxidation-Reduction; Oxygen; Plasmodium falciparum; Riboflavin; Thalassemia; Vitamin K

Topics: Antimalarials; Biochemical Phenomena; Biochemistry; Environment; Genetics; Glucosephosphate Dehydrogenase Deficiency; Humans; Neomycin; Nutritional Physiological Phenomena; Pharmacology; Vitamin K

Topics: Adenosine Triphosphate; Animals; Child, Preschool; Erythrocytes; Glucose; Glucosephosphate Dehydrogenase Deficiency; Hemolysis; Humans; Kinetics; Phospholipases; Snakes; Venoms; Vitamin K

Topics: Anemia, Hemolytic, Congenital; Anemia, Neonatal; Blood Coagulation Factors; Blood Transfusion; Chlorothiazide; Dicumarol; Female; Glucosephosphate Dehydrogenase Deficiency; Hemoglobinopathies; Hemophilia A; Hemorrhage; Humans; Infant, Newborn; Iron; Liver; Pregnancy; Thrombocytopenia; Vitamin K; Vitamin K Deficiency Bleeding

Topics: Chromium Isotopes; Drug Hypersensitivity; Erythrocytes; Favism; Female; Glucosephosphate Dehydrogenase Deficiency; Hemolysis; Heterozygote; Humans; Immunogenetics; Mosaicism; Primaquine; Sex Chromosomes; Vitamin K

Topics: Adult; Catalase; Chromium Isotopes; Erythrocytes; Female; Genetics, Population; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Humans; Male; Middle Aged; Sex Chromatin; Vitamin K

Topics: Aniline Compounds; Animals; Antimalarials; Catalase; Enzyme Inhibitors; Erythrocytes; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Hemolysis; Hemolytic Agents; Herbicides; Hydrogen Peroxide; Mice; Pharmacology; Phenylhydrazines; Primaquine; Research; Toxicology; Vitamin K

Topics: Anemia; Anemia, Hemolytic; Erythrocytes; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Humans; Infant; Infant, Newborn; Infant, Newborn, Diseases; Naphthalenes; Poisoning; Toxicology; Vitamin K

Topics: Analgesics; Analgesics, Non-Narcotic; Anemia; Anemia, Hemolytic; Antimalarials; Antipyretics; Erythroblastosis, Fetal; Female; Fetal Diseases; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Humans; Hyperbilirubinemia; Infant; Infant, Newborn; Infant, Newborn, Diseases; Jaundice; Jaundice, Neonatal; Maternal-Fetal Exchange; North Carolina; Novobiocin; Pregnancy; Salicylates; Sulfisoxazole; Toxicology; Vitamin K

Topics: Antimalarials; Catalase; Chloroquine; Erythrocytes; Ethanol; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Hemolysis; Hemolytic Agents; Hydrogen Peroxide; Hydroquinones; Pharmacology; Phenols; Phenylhydrazines; Primaquine; Research; Triazoles; Vitamin K

Topics: Anemia; Anemia, Hemolytic; Blood Chemical Analysis; Drug Therapy; Exchange Transfusion, Whole Blood; Genetics, Medical; Glucose; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Hemoglobinometry; Humans; Infant Mortality; Infant, Newborn; Jaundice; Jaundice, Neonatal; Kernicterus; Naphthalenes; Oxidoreductases; Phosphates; Toxicology; Vitamin K

Topics: Antifibrinolytic Agents; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Humans; Infant, Newborn; Jaundice; Jaundice, Neonatal; Naphthoquinones; Nigeria; Vitamin K

Topics: Anemia, Hemolytic; Antimalarials; Brain Diseases; Cerebral Palsy; Chloramphenicol; Genetics, Medical; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Humans; Hyperbilirubinemia; Infant; Infant, Newborn; Jaundice; Jaundice, Neonatal; Naphthalenes; Nitrofurantoin; Phenylhydrazines; Pregnancy; Quinidine; Sulfonamides; Thailand; Toxicology; Vitamin K