Pyruvate Kinase, Glucose 6-phosphate Dehydrogenase and Glutathione Reductase Deficiencies and Neonatal Jaundice in Basrah, Iraq

Abstract

Hyperbilirubinemia is a common problem of term and preterm neonates. Two hundred four jaundiced neonates admitted to Basrah Maternity and Childrens Hospital over a 6-month period were studied to determine the frequency of pyruvate kinase (PK) deficiency, glucose-6-phosphate dehydrogenase (G6PD) deficiency and glutathione reductase (GSSG-R) deficiency. Forty two neonates (20.5%) had PK deficiency, 68 (33.3%) G6PD deficiency and 40 (19.6%) GSSG-R deficiency. Interaction of more than one enzymopathy was found in 36 neonates (17.6%). Other hemolytic causes of jaundice were ABO incompatibility in 50 (24.5%) of neonates and Rh. incompatibility in 18 (8.8%). In 38 neonates no cause of jaundice was identified. There was a statistically significant increase in the frequency of G6PD deficiency and more than one enzymopathy with increasing severity of jaundice. The highest frequency of kenricterus was found in those with more than one enzymopathy. Red cell enzymopathies are an important cause of jaundice in Iraqi neonates and the presence of more than one enzymopathy carries a greater risk of developing severe jaundice.