Recent reports from the US confirm the association between glucose-6-phosphate dehydrogenase (G6PD) deficiency and severe neonatal jaundice with kernicterus. As the contribution of increased hemolysis to this jaundice is often minimal, defective hepatic bilirubin (bili) elimination has been implicated in its pathogensis. Bilirubin is conjugated to mono- and diglucuronide fractions, and in conditions of deficient conjugation (conj), the latter is affected first. Aim To quantify bili conj in jaundiced, term G-6-PD deficient neonates compared to G-6-PD normal controls.Methods A system utilizing alkaline methanolysis and reverse phase high performance liquid chromatography, capable of accurately measuring conj bili and its fractions, was employed. Serum conj bili and its mono-and diconj fractions, from G-6-PD deficient, term, male newborns and control infants, matched for birth weight and sex, all normal for G-6-PD, were measured. All had serum bilirubin levels ≥15 mg/dl. Conj bili fractions were compared, and the number of infants with totally undetectable diconj bili in each group was noted. Non-parametric data, presented as median (range), were analyzed using the Kruskal-Wallis test, and proportions by χ2 analysis.Results Table Conclusion We have demonstrated a deficiency in bili diconjugation in the G6PD deficient infants reminiscent of that seen in other conditions characterized by deficient bili conjugation. Partially defective bili conj has not been previously demonstrated in G6PD deficient neonates and may be a major contributor to the pathogenesis of the associated jaundice.

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