Abstract
The material presented is divided into eight chapters. The first three, authored by Dr. Murayama, discuss the chemical and three-dimensional structure of human hemoglobin, the bioenergetics of sickling, and Dr. Murayama's hypotheses explaining the mechanism of the sickling phenomenon. This material is stated in a clear and concise fashion. After discussing the primary, secondary, tertiary, and quarternary structure of the hemoglobin molecule, Dr. Murayama reviews his published experiments and uses them as a basis for his hypothesis of the sickling phenomenon, including the need for a cofactor. Not everybody agrees with Dr. Murayama's ideas, but they are well presented here. Dr. Nalbandian follows these introductory chapters with a detailed discussion of his ideas on sickle cell disease, starting with intrauterine erythropoeisis, and then discusses the Murayama (dithionate-urea) test, an automated mass screening program for sickle cell disease, and the theoretical and clinical practical use of urea in sickle cell disease.
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