Erythrocyte glucose-6-phosphate dehydrogenase deficiency in male newborn babies and its relationship with neonatal jaundice

Revista Brasileira de Hematologia e Hemoterapia

Marli Auxiliadora C. Iglessias²  Rosa Maria V. Santos¹  Maria Do Socorro T. Amorim¹  Rosângela T. Silva¹  Susiane S. Moreira¹  Orlando C. O. Barretto¹  Tereza Maria D. Medeiros¹ 

[1] ,Universidade Federal do Rio Grande do NorteNatal RN

关键词: Glucosephosphate dehydrogenase;    infant;    newborn;    jaundice;    hyperbilirubinemia;    Glucosefosfato desidrogenase;    recém-nascido;    icterícia neonatal;    hiperbilirrubinemia;   

DOI  :  10.1590/S1516-84842010005000086

来源: SciELO

【 摘 要 】

Glucose-6-phosphate dehydrogenase (G6PD) deficiency, the commonest red cell enzymopathy in humans, has an X-linked inheritance. The major clinical manifestations are drug induced hemolytic anemia, neonatal jaundice and chronic nonspherocytic hemolytic anemia. The incidence of neonatal hyperbilirubinemia is much greater in G6PD-deficient neonates than babies without this deficiency. The aim of this study was to ascertain the presence of neonatal jaundice in erythrocyte G6PD-deficient male newborns. Samples of umbilical cord blood from a total of 204 male newborns of the Januário Cicco School Maternity located in Natal, Rio Grande do Norte, Brazil were analyzed. The G6PD deficiency was identified by the methemoglobin reduction test (Brewer's test). The deficiency was confirmed by quantitative spectrophotometric assay for enzyme activity and cellulose acetate electrophoresis was used to identify the G6PD variant. Eight newborns were found to be G6PD deficient with four of them exhibiting jaundice during the first 48 hours after birth with bilirubin levels higher than 10 mg/dL. All deficient individuals presented the G6PD A- variant at electrophoresis. Our findings confirmed the association between G6PD deficiency and neonatal jaundice. Hence, early diagnosis of the deficiency at birth is essential to control the appearance of jaundice and to prevent the exposure of these newborns to known hemolytic agents.

【 授权许可】

CC BY-NC-ND   
 All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License