The relationship of erythrocyte glucose-6-phosphate dehydrogenase deficiency to hyperbilirubinemia in Negro premature infants

doi:10.1016/S0022-3476(67)80044-1

The Journal of Pediatrics

Volume 70, Issue 4, April 1967, Pages 595-601

https://doi.org/10.1016/S0022-3476(67)80044-1 Get rights and content

All of the Negro nfants admitted to a premature nursery during a four month interval were essayed for glucose-6-phosphate dehydrogenase deficiency. Ten of 87 were found to be deficient. Serum bilirubin levels of 20 mg. per cent or more were attained in six of these ten infants, and five required exchange transfusions. In contrast, only eight of the remaining seventy-seven infants required exchange transfusions. Glucose-6-phosphate dehydrogenase deficiency of the Negro premature infant, unlike that of the Negro term infant, appears to be associated with an increased risk of hyperbilirubinemia.

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Cited by (23)

The Preterm Infant. A High-Risk Situation for Neonatal Hyperbilirubinemia Due to Glucose-6-Phosphate Dehydrogenase Deficiency
2016, Clinics in Perinatology
Citation Excerpt :
The high-risk nature of preterm, G6PD-deficient newborns for significant hyperbilirubinemia was apparent already in the pre-phototherapy era. Eshaghpour and colleagues26 identified 10 of 87 (11.5%) African American infants admitted to a premature infant nursery in Philadelphia, PA to be G6PD-deficient. Six (60%) of these developed TB levels greater than 20 mg/dL, and exchange transfusion was performed in 5 (50%).
Erythrocyte Disorders in Infancy
2005, Avery's Diseases of the Newborn
Erythrocyte enzyme disorders in children
1980, Pediatric Clinics of North America
Erythrocyte metabolic abnormalities should be considered as a possible cause of hemolysis when there is no evidence of an immune-mediated hemolytic anemia, no consumptive red blood cell disorder, no morophologic or laboratory data to suggest a problem of the red cell membrane, and no evidence of a quantitative or qualitative defect in hemoglobin synthesis. Glucose-6-phosphate dehydrogenase deficiency is clearly the most common enzyme deficiency causing clinical problems.
Un déficit en glucose 6 phosphate déshydrogénase peut-il à lui seul expliquer un ictère néo-natal ?
1978, Revue Francaise de Transfusion et Immuno-hematologie
De nombreux auteurs ont insisté sur le rôle du déficit en glucose 6-phosphate déshydrogénase (G₆ PD) érythrocytaire dans la survenue d'un ictère néonatal, parfois grave, pouvant nécessiter une exsanguino-transfusion.
Compte tenu du caractère le plus souvent provoqué de l'hémolyse chez les déficitaires, le but de notre étude a été de rechercher si le déficit pouvait à lui seul induire une hyperbilirubinémie néonatale ou si des « stressoxydatifs étaient impliqués dans la survenue de l'ictère.
L'étude de l'activité enzymatique de la G₆PD érythrocytaire a été réalisée chez 289 nouveau-nés noirs d'origine africaine ou antillaise par le test au Tetrazolium. 21 nouveau-nés sont déficitaires, 8 filles sont porteuses de la tare, soit 10,03 %.
Aucun ictère néonatal n'est observé. Avec Bienzle et coll., on peut supposer que l'« environnementjoue un rôle primordial dans la survenue d'un ictère chez le nouveau-né déficitaire noir.
Des facteurs déclenchant cet ictère pouvant être méconnus, il nous apparaît toutefois indispensable de rechercher un déficit chez tout nouveau-né ictérique nécessitant une exsanguino-transfusion.
Several authors have insisted on the role of a glucose 6-phosphate deshydrogenase (G₆PD) deficiency in the occurrence of neonatal jaundice which can be severe, leading to exchange transfusion.
Considering the fact that haemolysis, occuring in deficient patients, is most of the time induced, our aim was to search if a neonatal hyperbilirubinemia could be explained by the sole G6PD deficiency or by extra « oxidant stress .
Red blood cell G₆PD activity of 289 newborn babies of African or Antillian origin was tested by a Tetrazolium linked method. 21 newborns were deficient. 8 female babies were heterozygous for G₆PD deficiency.
No neonatal jaundice was observed. With Bienzle and all., we can suppose that the « environnementtakes an important part in the incidence of neonatal jaundice among G₆PD deficient newborn of African or Antillian origin.
However as triggering factors could be ignored, we think compulsary to search a G₆PD deficiency in every neonatal jaundice necessitating an exchange-transfusion.
Hemolysis in the newborn infant resulting from deficiencies of red blood cell enzymes: Diagnosis and management
1974, The Journal of Pediatrics
Inherited deficiencies of red cell enzymes are recognized causes of hemolysis in newborn infants. Neonatal manifestations include erythroblastosis fetalis, anemia, hyperbilirubinemia, or a combination of these. Some infants with enzyme deficiencies demonstrate no neonatal manifestations; in others death may result from hydrops fetalis or kernicterus. In cases of suspected hemolysis, simple diagnostic measures will identify the most common causes of the anemia and/or the hyperbilirubinemia; these are not inherited deficiencies of enzymes but are red cell sensitization and congenital and acquired infections. Persistent hemolysis and abnormalities in the G-6-PD screening test, in the Heinz body preparation, or in an autohemolysis test support a tentative diagnosis of enzyme deficiency. The diagnosis is confirmed by specific enzyme assay. Appropriate therapy depends on the severity of anemia and/or hyperbilirubinemia and may include transfusion of packed red cells or an exchange transfusion. Careful follow-up is essential to determine the chronicity and severity of hemolysis.
Congenital hemolytic anemias and red cell enzyme deficiencies
1973, Current Problems in Pediatrics

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Supported in part by United State Public Health Service Grants AM 02231 and HO 01919.

Presented in part at the 76th Meeting of the American Pediatric Society, April, 1966.

^*: Address, Hospital of the University of Pennsylvania, 3600 Spruce St., Philadelphia, Pa. 19104.

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Original articleThe relationship of erythrocyte glucose-6-phosphate dehydrogenase deficiency to hyperbilirubinemia in Negro premature infants+

Lancet

Lancet

Lancet

Lancet

J. Pediat.

J. Pediat.

J. Pediat.

Lancet

Lancet

Neonatal jaundice in glucose-6-phosphate dehydrogenase-deficient infants

Brit. M. J.

Increased incidence of severe hyperbilirubinemia among newborn Chinese infants with G-6-PD deficiency

Pediatrics

Original article
The relationship of erythrocyte glucose-6-phosphate dehydrogenase deficiency to hyperbilirubinemia in Negro premature infants+