Muhammad Yasin Alvi, Akmal Laeeq, Mohammad Ali Khan, Muhammad Azhar Iqbal.
Glucose 6 – Phosphate dehydrogenase deficiency associated with neonatal jaundice
Pak Paed J Mar 2006;30(1):28-33.
The Children`s Hospital & Institute of Child Health, Lahore

Glucose-6 Phosphate Dehydrogenase (G-6PD) deficiency is the commonest enzymopathy in human beings. It is transmitted as X-linked recessive disorder. Acute hemolytic crisis is the most common presentation of G-6PD deficiency, but in neonatal period it usually presents as jaundice. Objectives: To find out the proportion of G-6PD deficiency cases in patients with neonatal pathological hyperbilirubinemia and study the clinical course of disease. Methods: The study was conducted at the neonatal unit of The Children`s Hospital & Institute of Child Health, Lahore from January 2000 – April 2001. One hundred jaundiced neonates with unconjugated hyperbilirubinemia in pathologic range (peak serum bilirubin more than 12 mg/dl in full term and more than 15 mg/dl in preterm neonates) were included. Screening for G6PD deficiency was done by dye decolorization test, which is semi quantitative, visual colorimetric assay. Results: Out of 100 study cases, 62% were male and 38% were female. 10% of the cases were found to be G-6PD deficient; all were male. One case of G-6PD deficiency developed jaundice during first 24 hrs of life, 8 cases between 1 -7 days and one case after 7 days of life. Peak serum bilirubin levels in neonates with G-6PD deficiency were < 20 mg/dl in 2 cases, 20-30 mg/dl in 6 cases and >30 mg/dl in 2 cases. Evidence of hemolysis (reticulocyte count >5% and Hb% <12.5 gm%) was present in two neonates. In the G-6PD deficiency group, 40% of the cases underwent exchange transfusion compared to 26.6% of cases in the G-6PD normal group. One neonate with G-6PD deficiency had kernicterus at admission. Two neonates with G-6PD deficiency died, due to culture proven sepsis. Conclusions: G-6PD assay should be included in all jaundiced neonates with unexplained neonatal unconjugated pathological hyperbilirubinemia. G-6PD deficiency associated neonatal jaundice is not only hemolytic in origin, but is also related to the impairment of hepatic bilirubin conjugation and excretion.

Category: Pediatrics
Keywords: G6PD Deficiency. Hemolysis. Neonatal Hyperbilirubinemia. Jaundice. Neonates.

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