Red Cell Glucose-6-Phosphate Dehydrogenase Deficiency—A Newly Recognized Cause of Neonatal Jaundice and Kernicterus in Canada

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.     

Metabolism of iodomethane in the rat

1. The LD(50) of iodomethane orally administered to rats is 76mg./kg. body wt. but repeated daily doses of 30-50mg./kg. body wt. are without effect. 2. Oral doses of iodomethane to rats are rapidly converted into S-methylglutathione in the liver, and S-methylglutathione is excreted in the bile. 3. The conjugation process has been reproduced in vitro and shown to be enzymically catalysed. 4. S-Methylglutathione is degraded to S-methylcysteine by kidney homogenates. 5. Only a small proportion of a dose of iodomethane or of S-methylcysteine is excreted as compounds related to methylmercapturic acid. 6. Paper chromatography of extracts of livers from normal and dosed rats supports the view that GSH is the predominant non-protein thiol present.