Glucose 6 Phosphate Dehydrogenase Deficient Neonates

Glucose 6 Phosphate Dehydrogenase Deficient Neonates

Background: Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is a common disorder that carries a high incidence of severe neonatal hyperbilirubinemia with the potential of irreversible bilirubin encephalopathy. The pathogenesis of the associated bilirubinemia was elucidated.

Methods: Fifty two G-6-PD-deficient male infants and 166 unaffected male infants were studied. Serum total bilirubin (STB) was determined within 3 hours of birth, on the third day of life, and thereafter as necessary.

Finding: Initial and second STB values were significantly greater in G-6-PD-deficient infants than in the control group. The rate of increase between the 2 measures was higher in the G-6-PD-deficient infants. Hyperbilirubinemia developed in 30.8% of the G-6-PD-deficient infants and i n 6% of the control infants. In both groups, the initial STB values were significantly associated with the second STB values and subsequent development of hyperbilirubinemia. A significantly greater percentage of G-6-PD-deficient infants with an initial STB value at least equal to the mean subsequently had hyperbilirubinemia compared with infants with initial STB values lower than the mean, the relative risk being 3.7.

Conclusions: These data suggest that jaundice in G-6-PD-deficient newborns begins in the immediate perinatal period, probably in utero. The G-6-PD-deficient neonates studied had significantly greater STB values compared with unaffected infants shortly after birth as well as 3 days after birth.