Prospective study of sickle cell anemia in infancy.
Twelve infants with sickle cell anemia identified in the course of a cord blood screening program have been followed prospectively for up to three years of age. The development of hemolytic anemia paralleled the postnatal decline in fetal hemoglobin and was evident in all infants by 12 weeks of age. Vasoocclusive episodes occurred in more than half the infants and seven aplastic crises were documented in four patients. Febrile illnesses were common and one of the twelve infants developed pneumococcal sepsis. This study also demonstrated that functional asplenia is an acquired defect in sickle cell disease. The onset of functional asplenia was documented with splenic scans in six of the nine infants followed for more than one year after birth. There have been no deaths in this series.
D000741:Anemia, Aplastic / Q000097:blood; D000755:Anemia, Sickle Cell / Q000097:blood* / Q000503:physiopathology; D002675:Child, Preschool; D005319:Fetal Hemoglobin / Q000032:analysis; D006444:Hemoglobin C / Q000032:analysis; D006451:Hemoglobin, Sickle / Q000032:analysis; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012157:Mononuclear Phagocyte System / Q000503:physiopathology; D011446:Prospective Studies; D013154:Spleen / Q000503:physiopathology; D013163:Splenomegaly / Q000503:physiopathology; D013667:Technetium; D013789:Thalassemia / Q000097:blood
D006451:Hemoglobin, Sickle; D013667:Technetium; D006444:Hemoglobin C; D005319:Fetal Hemoglobin