Abstract
Since June, 1972, we have conducted a comprehensive umbilical cord blood screening program for sickle hemoglobinopathies at Yale-New Haven Hospital. When we recently noticed an apparent preponderance of females with hemoglobins AS and SS, we decided to analyze the results of the first 65 months of screening. An FAS hemoglobin pattern (sickle cell trait) was detected in 162/1959 black females vs. 129/2017 in black males (X2=5.14,P<.05). For the FS hemoglobin pattern (sickle cell anemia and sickle β-thalassemia), the difference was even more striking: 15/1959 in black females vs. 4/2017 in black males (X2=6.73,P<.01). For 704 Puerto Rican newborns, the incidence of sickle cell trait was very low (2.1% overall), and there were no significant female-male differences. Excluding the two children with FS cord blood electrophoreses who were subsequently shown to have sickle β-thalassemia, there were 17 black newborns with hemoglobin SS among the first 3976 black births since the inception of the screening program. This represents an incidence of 1 in 234 live births, which far exceeds the 1 in 750 predicted by the Hardy-Weinberg equation. The excess of observed over predicted SS newborns is almost entirely accounted for by the high incidence in black females, 1 in 150. Hemoglobin S may confer a selective advantage in utero that is more marked in females.
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