Glucose-6-phosphate dehydrogenase deficiency; the single most important cause of neonatal hyperbilirubinaemia in Kano, Nigeria
Introduction: Glucose- 6-phosphate dehydrogenase deficiency is the most common enzymatic disorder of the red cell and an important risk factor for neonatal jaundice.
Methodology: The aim of the study was to determine the incidence of G-6-PD deficiency among jaundiced neonates, and describe the associated morbidity and mortality pattern in them. A prospective cross sectional study was conducted and we studied one hundred consecutive jaundiced neonates (55 males, 45 females) presenting at Aminu Kano Teaching Hospital from between 2004 and August 2005. G-6-PD activity was assayed by Quantitative spectrophotometric method of Kornberg; serum bilirubin and haemoglobin levels were estimated by standard techniques. Exposure to possible Icterogenic agents, clinical features of kernicterus and the outcome were noted.
Results: The incidence of G-6-PD deficiency was found to be 46% with male to female ratio of 3:1 (Χ2 = 15, p = 0.001). A higher proportion (60.6%) of the inborn neonates had G-6-PD deficiency (X2 = 5.5, p = 0.06). Jaundice was noticed significantly earlier in the G-6-PD deficient neonates (mean = 2.0, SD = 1 days) compared to (mean = 2.7, SD = 1.6 days) in the sufficient neonates (t = 2.3, p = 0.02). Sixteen (16%) neonates developed kernicterus, of these 10 (63%) were G-6-PD deficient. The mortality rate among G-6-PD deficient neonates was 15% (7 of 46) twice as much as in the sufficient neonates 7% (4 of 54). Only six neonates 0.6% ware exposed to naphthalene of whom three were G -6PD deficient. Five babies were given traditional medicine two of which were G6-PD deficient.
Conclusion: G-6-PD deficiency is an important risk factor for neonatal jaundice. Jaundice appeared early in the deficient neonates. There is high incidence of kernicterus and mortality among them. Low admission weight significantly contributed to the mortality.
Key Words: G-6-PD deficiency; Neonatal Jaundice; Kernicterus