title: [Glucose-6-phosphate dehydrogenase deficiency and other erythrocyte enzyme abnormalities].
abstract: Normal values of glucose-6-phosphate dehydrogenase (G6PD), 6-phosphogluconate dehydrogenase (6PGD), glutathione reductase (GR), glucosephosphate isomerase (GPI), pyruvate kinase (PK) and pyrimidine 5'-nucleotidase (P5N) have been determined in normocytes, reticulocytes, newborn cord erythrocytes, and leucocytes. Metabolic and clinical aspects of G6PD and the classification of its genetic variants are reviewed. Enzyme determinations and their variation in drug-induced haemolysis are critically presented. Extensive tables are published listing the drugs and compounds that can cause haemolysis in G6PD-deficient patients, as well as those preparations which may, probably, be administered safely. Clinical and biochemical data in patients with the inherited enzyme defects GR, GPI, PK, and P5N, as well as acquired deficiency of the last-mentioned in chronic lead intoxication, are reviewed in the light of our personal experience in this field.
mesh_terms: D015720:5'-Nucleotidase; D000743:Anemia, Hemolytic / Q000139:chemically induced / Q000201:enzymology; D000746:Anemia, Hemolytic, Congenital Nonspherocytic; D004912:Erythrocytes / Q000201:enzymology*; D005955:Glucosephosphate Dehydrogenase Deficiency / Q000378:metabolism*; D005980:Glutathione Reductase / Q000172:deficiency; D006019:Glycolysis; D006801:Humans; D007962:Leukocytes / Q000201:enzymology; D009708:Nucleotidases / Q000172:deficiency; D009711:Nucleotides / Q000378:metabolism; D010428:Pentosephosphates / Q000378:metabolism; D010734:Phosphogluconate Dehydrogenase / Q000172:deficiency; D011319:Primaquine / Q000009:adverse effects; D011770:Pyruvate Kinase / Q000172:deficiency; D012156:Reticulocytes / Q000201:enzymology
chemical_list: D009711:Nucleotides; D010428:Pentosephosphates; D010734:Phosphogluconate Dehydrogenase; D005980:Glutathione Reductase; D011770:Pyruvate Kinase; D009708:Nucleotidases; D015720:5'-Nucleotidase; D011319:Primaquine
vernacular_title: Glukose-6-Phosphatdehydrogenasemangel und andere Enzymanomalien in Erythrozyten.