Life-Threatening Complications of Sickle Cell Disease in Children
- Myron L. Mills, MD
Since this article does not have an abstract, we have provided the first 150 words of the full text.
Excerpt
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.1 In 1949, Pauling and Itano2 published their findings relating to the different electrophoretic properties of hemoglobin from patients with SCA. Later, in 1957, Ingram3 published 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
Footnotes
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Reprint requests to Section of Emergency Medicine, Arizona Health Sciences Center, Tucson, AZ 85724 (Dr Mills).
