Abstract
In the Hgb S homozygote, sickle hemoglobin is present at 12-16 weeks of gestation and accounts for 10-20% of the Hb at birth. In the neonate with sickle cell anemia, about 10% of circulating RBC can be induced to sickle and isolated instances of neonatal morbidity and mortality have been ascribed to sickling. 34 infants with SS disease were diagnosed by cord blood Hb electrophoresis, performed on all black infants at our hospitals. At Yale, stillbirths are also studied. The number of patients exceeded that predicted by gene frequency, making it unlikely that any cases were missed. Neonatal physical measurements and clinical courses were reviewed retrospectively. 32/34 infants were AGA with respect to birthweight, length and head circumference. 5/34 infants had gestational ages <38 weeks and weighed <2500 gm. One postmature and one premature were SGA. 4/34 had 1 min. APGAR's <8, but all were >8 at 5 min. 7/34 developed clinical jaundice. In 2 cases, ABO EBF was proven; 4 others were premature. Phototherapy was used in 4 cases. No regular transfusions or exchange transfusions were performed. No infants developed sepsis. Hospital stay was prolonged only in the prematures. None of these patients had significant morbidity which could be ascribed to sickling, and there were no neonatal deaths. Therefore, these data do not indicate very important consequences of SS disease during gestation or in the neonatal period.
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Pearson, H., Parke, J., Ehrenkranz, R. et al. 646 FETAL AND NEONATAL CORRELATES OF SICKLE CELL ANEMIA. Pediatr Res 12 (Suppl 4), 471 (1978). https://doi.org/10.1203/00006450-197804001-00651
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DOI: https://doi.org/10.1203/00006450-197804001-00651