In a prospective study, 457 black children admitted consecutively for serious infections were classified as to hemoglobin genotype, type of infection, and etiologic agent. Of these 457 patients, eight had sickle cell anemia (hemoglobin [Hb] SS). This was eight times the expected frequency. The Hb SS patients had a 25% mortality as compared to 1.5% for Hb AA and AS patients.
In analyzing the type of infections and etiologic agents, a significantly increased frequency of pyelonephritis, diarrhea, sepsis, and meningitis was found among the sickle cell disease patients. These infections were due to two organisms, the pneumococcus and Escherichia coli. An increased frequency of infections or specific etiologic agent was not found in the Hb AS group.
(JAMA 230:1145-1148, 1974)