Glucose-6-phosphate dehydrogenase deficiency and carboxyhemoglobin concentrations associated with bilirubin-related morbidity and death in Nigerian infants

• Published in: The Journal of pediatrics Vol. 126; no. 1; pp. 102 - 108
• Main Authors: Slusher, Tina M., Vreman, Hendrik J., McLaren, Donald W., Lewison, Laura J., Brown, Audrey K., Stevenson, David K.
• Format: Journal Article
• Language: English
• Published: New York, NY Mosby, Inc 1995; Elsevier
• Subjects: Anemias. Hemoglobinopathies; Bilirubin - blood; Biological and medical sciences; Blood Transfusion; Carboxyhemoglobin - analysis; Diseases of red blood cells; Female; Glucosephosphate Dehydrogenase Deficiency - blood; Glucosephosphate Dehydrogenase Deficiency - epidemiology; Glucosephosphate Dehydrogenase Deficiency - mortality; Hematologic and hematopoietic diseases; Humans; Infant Mortality; Infant, Newborn; Jaundice - blood; Jaundice - complications; Jaundice - therapy; Kernicterus - diagnosis; Kernicterus - etiology; Kernicterus - mortality; Male; Medical sciences; Nigeria - epidemiology; Tropical medicine; Nigeria; Africa; COHb; G6PD; TSB; Human; Jaundice; Intramolecular oxidoreductases; Enzyme; Deficiency; Mortality; Metabolic diseases; Carboxyhemoglobin; Hemopathy; Enzymopathy; Epidemiology; Morbidity; Genetic disease; Hemolytic anemia; Isomerases; Hyperbilirubinemia; Newborn; Glucose-6-phosphate isomerase; Hemoglobin; Carbon monoxide
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/S0022-3476(95)70510-4

Our objective was to determine whether glucose-6-phosphate dehydrogenase (G6PD) deficiency and elevated carboxyhemoglobin (COHb) levels correlated with bilirubin-related morbidity and mortality rates. For this purpose, we studied 55 clinically jaundiced infants admitted to a rural mission hospital in southern Nigeria. Total serum bilirubin levels (range, 80 to 1016 μmol/L [4.7 to 59.4 mg/dl]) correlated with the percentage COHb concentrations (COHb = 0.45 + 0.08* Total serum bilirubin; r = 0.72). Infants were divided into two groups of equal size around the median COHb concentration (COHb range, 0.43% to 5.93% [median = 1.40%], with ambient carbon monoxide of 0.65 ± 0.03 μL/L). The COHb levels > 1.40% were associated with the need for exchange transfusion (15/28, or 54%, vs 5/27, or 19%; p < 0.01) and with an increased incidence of clinical findings compatible with kernicterus (9/28, or 32%, vs 0/27, or 0%; p < 0.01). Mortality rate was 29% (8/29) among infants with higher COHb levels, and 7% (2/28) in those with lower levels ( p = 0.08). Thirty-one percent (14/45) of the clinically jaundiced infants tested had G6PD deficiency. Thirty-six percent of the infants with G6PD deficiency died with presumed kernicterus, compared with only 3% (1/31) of the infants with a normal G6PD screening test result ( p < 0.01). These data suggest that G6PD deficiency and increased bilirubin production, as indexed by COHb, are associated with jaundice-related morbidity and death in Nigerian infants. (J P EDIATR 1995;126:102-8)