Neonatal Hyperbilirubinemia Associated With Glucose-6-Phosphate Dehydrogenase Deficiency in Sephardic-Jewish Neonates: Incidence, Severity, and the Effect of Phototherapy

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Abstract
Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is frequently associated with neonatal hyperbilirubinemia, and sometimes kernicterus, often in the absence of any identifiable trigger or hematological evidence of hemolysis. The aim of this study was to compare the incidence and severity of, and the effect of phototherapy on, jaundice in G-6-PD-deficient vs G-6-PD-normal neonates in the Sephardic-Jewish community. Healthy term newborns, born to mothers of families stemming from geographic areas known to be "at risk" for G-6-PD deficiency, were screened for the condition and surveyed for hyperbilirubinemia. Seventy-five G-6-PD-deficient neonates formed the study group, while 266 neonates with normal levels of the enzyme formed the control group. Neonates with any other identifiable cause for jaundice were excluded. Phototherapy was commenced when the serum bilirubin levels reached 16 mg/dL (274 µmol/L) or more, and it was discontinued at 12 mg/dL (205 µmol/L) or less. Hyperbilirubinemia developed in 27 (36%) of the deficient neonates (serum total bilirubin >13.9 mg/dL [238 µmol/L]), compared with 50 (18.8%) of control neonates (P = .002), while 20 (26.7%) of the study group required phototherapy, compared with 31 (11.7%) of control neonates (P = .002). Two neonates in the study group required exchange transfusion (serum bilirubin >20 mg/dL [342 µmol/L]), vs 0 in the control group (not significant). Further analysis was performed on male neonates only: despite a tendency for the hyperbilirubinemia to behave differently with regard to peak bilirubin levels, age at the time of the peak, duration of phototherapy, and rate of bilirubin decrease during phototherapy, these differences failed to attain statistical significance. Values for hematocrit and reticulocyte count did not show evidence of overt hemolysis. Routine daily clinical evaluation and use of phototherapy according to the above criteria made it possible to keep the need for exchange transfusion to a minimum and to prevent kernicterus in this population.

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