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June 1976

The Clinical Features of Sickle Cell Disease: Clinical Studies

Arch Ophthalmol. 1976;94(6):1053. doi:10.1001/archopht.1976.03910030541020

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From the single gene-single enzyme principle, the author develops a detailed description of how exchange of one amino acid can appear in vastly diverse manners. Despite the title, the book is not limited strictly to the clinical features of sickle cell disease; the historical background, biochemical features, diagnostic tests, and worldwide geographical distribution of the sickle hemoglobin are initially presented. A detailed description of the genetic expression of sickle cell in each organ system is given, and each chapter is developed in a well-organized manner. The bibliography is excellent and complete through mid-1973.

After exposure in medical school to severely afflicted patients with sickle cell disease, most physicians picture sickle cell anemia as a largely devastating disease. The author traveled away from the hospital to the basically closed populations of the villages of Jamaica. He has determined the prevalence of each manifestation in this unselected cross section of patients with

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