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.
D000700:Analgesics; D018712:Analgesics, Non-Narcotic; D000962:Antimalarials; D058633:Antipyretics; D044466:Asian People; D001669:Biochemical Phenomena; D001671:Biochemistry; D002170:Canada; D004358:Drug Therapy; D004912:Erythrocytes; D005007:Ethnology; D005078:Exchange Transfusion, Whole Blood; D005826:Genetics, Medical; D005955:Glucosephosphate Dehydrogenase Deficiency; D005958:Glucosephosphates; D006461:Hemolysis; D006801:Humans; D006932:Hyperbilirubinemia; D007231:Infant, Newborn; D007232:Infant, Newborn, Diseases; D007565:Jaundice; D007567:Jaundice, Neonatal; D007647:Kernicterus; D008297:Male; D008706:Methemoglobin; D009249:NADP; D009281:Naphthalenes; D009581:Nitrofurans; D012723:Sex; D013223:Statistics as Topic; D000077145:Sulfanilamide; D013424:Sulfanilamides; D013449:Sulfonamides; D014116:Toxicology; D044465:White People
D000700:Analgesics; D018712:Analgesics, Non-Narcotic; D000962:Antimalarials; D058633:Antipyretics; D005958:Glucosephosphates; D009281:Naphthalenes; D009581:Nitrofurans; D013424:Sulfanilamides; D013449:Sulfonamides; D000077145:Sulfanilamide; C031721:naphthalene; D009249:NADP; D008706:Methemoglobin
ANALGESICS AND ANTIPYRETICS; ANTIMALARIALS; BIOCHEMISTRY; CANADA; CAUCASIAN RACE; DRUG THERAPY; ETHNOLOGY; GENETICS, HUMAN; GLUCOSEPHOSPHATE DEHY- DROGENASE DEFICIENCY; INFANT, NEWBORN; INFANT, NEWBORN, DISEASES; JAUNDICE, NEONATAL; KERNICTERUS; METHEMOGLOBIN; MONGOLOID RACE; NADP; NAPHTHALENES; NITROFURANS; SEX; STATISTICS; SULFONAMIDES; TOXICOLOGIC REPORT