Life-Threatening Complications of Sickle Cell Disease in Children

SINCE 1910, when sickle cell anemia (SCA) was first described by Dr James Herrick, a Chicago cardiologist, a prodigious amount of clinical and laboratory investigation has taken place in an effort to elucidate the pathogenesis, etiology, and treatment of SCA.¹In 1949, Pauling and Itano²published their findings relating to the different electrophoretic properties of hemoglobin from patients with SCA. Later, in 1957, Ingram³published the results of his work detailing the biochemical defect in hemoglobin S. From the early 1950s to the present time, the sickle erythrocyte and hemoglobin S molecule have been studied by sophisticated techniques that have revealed their structure and function at the molecular level. Techniques employed for these studies have included low-resolution x-ray crystallography, electron microscopy with image reconstruction, hemoglobin S polymerization kinetics, high-resolution proton nuclear magnetic resonance spectroscopy, and fiber x-ray diffraction. The basic defect in hemoglobin S is the substitution