Abstract
Extract: A total of 104 of 693 Negro infants (15.0%) had moderate or small amounts of hemoglobin Barts visible on starch gel electrophoresis. Moderate amounts were found in 21 infants (3.0%) and small amounts in 83 infants (12.0%). In 17 Negro infants judged to have moderate amounts of Hb Barts, the quantitation showed 2.0–9.3% with a mean of 5.4 ± 2.1 (1 SD). A significant decrease in mean cell volume and mean cell hemoglobin was found in the Negro neonate with more than 2% Hb Barts studied at 4 days of age. In 10 Negro infants with more than 2.0% Hb Barts studied at 4 days of age, the α α/(β + γ) ratio was 0.97 ± 0.06 (1 SD) (range 0.88–1.06). In nine infants aged 5—24 months who had more than 2.0% Hb Barts in the newborn period, including six infants studied in the first group, the mean α/β ratio was 0.74 ± 0.06, (range 0.65–0.83). Each of the nine infants with more than 2.0% Hb Barts at birth had marked microcytosis and hypochromia at 5—24 months despite adequate iron therapy. Two newborn infants with moderate levels of Hb Barts at birth (8.2% and 6.8%) and balanced total globin synthesis had no free radioactive α chain by gel filtration studies. Our studies indicate clearly that the presence of more than 2% Hb Barts in the newborn period denotes the presence of α-thalassemia trait.
Speculation: In a group of Negro newborn infants, 3% had more than 2% Hb Barts. These infants had the genetic disorder, α-thalassemia trait. An additional 12% of the infants had elevated levels of Hb Barts between 1% and 2%. This group may also have an α-thalassemia disorder, as has been shown in other racial groups. The absence of hydrops fetalis due to α-thalassemia in Negro neonates suggests that the molecular defect of α-thalassemia detected in Negro neonates differs from that seen in Orientals in that it is not associated with a complete absence of α-chain synthesis.
Similar content being viewed by others
Article PDF
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Friedman, S., Atwater, J., Gill, F. et al. α-Thalassemia in Negro Infants. Pediatr Res 8, 955–959 (1974). https://doi.org/10.1203/00006450-197412000-00008
Issue Date:
DOI: https://doi.org/10.1203/00006450-197412000-00008