α-Thalassemia in Premature Newborns

Abstract

ABSTRACT. In this study we have carried out a-globin gene mapping, hemoglobin (Hb) Bart's quantitation serum bilirubin, and red blood cell indices determination in a group of Sardinian appropriate for gestational age premature infants (from 32 to 35 wk gestation) in order to define the incidence in this population of the different a-thalassemia syndromes, their expression rate, and the correlation between the a-globin genotype and phenotype at this developmental stage. The gene frequencies of deletion (-a) and nondeletion (aath) a-thalassemia were 0.29 and 0.04, respectively, and thus not different from those found in full-term newborns from the same population. The majority of premature newborns with a single a-globin gene deletion [(-alas) genotype] were hematologically silent. Those who manifested increased Hb Bart's (1.2 to 3.