sodium-ethylxanthate and Glucosephosphate-Dehydrogenase-Deficiency

Topics: Anemia, Sickle Cell; Blood Protein Electrophoresis; Child, Preschool; Female; Glucosephosphate Dehydrogenase Deficiency; Hemoglobins, Abnormal; Humans; Infant; Malaria; Male; Nigeria; Sex

Topics: Color Vision Defects; Ethnicity; Genetics, Medical; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Humans; Karyometry; Netherlands; Sex; White People

Seven male newborns of Chinese, Greek and Italian origin presented with severe hemolytic jaundice due to red cell glucose-6-phosphate dehydrogenase (G-6-PD) deficiency. In five, the hemolysis was precipitated by inhalation of mothball vapours in the home. Kernicterus was evident upon admission in six infants and was fatal in four of these.G-6-PD deficiency should be suspected as a cause of jaundice in all full-term male infants of these ethnic groups. The diagnosis can be confirmed in any hospital by the methemoglobin reduction test. In areas similar to Toronto, Canada, where these high-risk ethnic groups prevail, the following measures are recommended: (1) detection of G-6-PD deficient newborns by screening cord bloods of all infants of these ethnic groups; (2) protection of affected infants from potentially hemolytic agents such as naphthalene, certain vitamin K preparations, and sulfonamides; and (3) observation of serum bilirubin levels to assess the need for exchange transfusion for hyperbilirubinemia.

Topics: Analgesics; Analgesics, Non-Narcotic; Antimalarials; Antipyretics; Asian People; Biochemical Phenomena; Biochemistry; Canada; Drug Therapy; Erythrocytes; Ethnology; Exchange Transfusion, Whole Blood; Genetics, Medical; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Hemolysis; Humans; Hyperbilirubinemia; Infant, Newborn; Infant, Newborn, Diseases; Jaundice; Jaundice, Neonatal; Kernicterus; Male; Methemoglobin; NADP; Naphthalenes; Nitrofurans; Sex; Statistics as Topic; Sulfanilamide; Sulfanilamides; Sulfonamides; Toxicology; White People

Topics: Angiomatosis; Chromosome Aberrations; Chromosome Disorders; Color Vision Defects; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Hemophilia A; Humans; Sex; X Chromosome

Topics: Agammaglobulinemia; Albinism; Anemia; Anemia, Hypochromic; Anemia, Sickle Cell; Blood Group Antigens; Choroid; Color Vision Defects; Ectodermal Dysplasia; Eye Diseases; Gene Silencing; Genetics, Medical; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Humans; Muscular Dystrophies; Sex; Sex Chromatin

Topics: Electrophoresis; Ethnology; Genetics, Medical; Geography; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Sex

Topics: Biomarkers; Brazil; Genetic Testing; Genetics, Medical; Genetics, Population; Glucosephosphate Dehydrogenase; Glucosephosphate Dehydrogenase Deficiency; Glucosephosphates; Humans; Sex; Statistics as Topic