P. Gillette, C. Peterson, Y. S. Lu
Mar 21, 1974
Citations
0
Influential Citations
61
Citations
Quality indicators
Journal
The New England journal of medicine
Abstract
Abstract Thirty-one patients with sickle-cell disease, seven to 49 years of age, were given the antisickling drug sodium cyanate (10 to 35 mg per kilogram of body weight per day by mouth) for six to 18 months. Carbamylation of the amino-terminal valine of hemoglobin was dose-related and ranged from 0.03 to 0.67 moles of carbamyl group per mole of hemoglobin tetramer. No toxic effects were observed at a dosage of 35 mg per kilogram per day or less. Occasional gastrointestinal distress and drowsiness occurred. The previously demonstrated ability of sodium cyanate to diminish the hemolytic rate was confirmed. The cyanate-induced increase in hemoglobin concentration (3 to 38 per cent) was dose related and resulted from decreased red-cell destruction. A retrospective analysis of the patients' records revealed a statistically significant decrease in the mean frequency of crises (from 3.6 to 2.1 crises per year) between two groups of patients with low (<0.3 mole of carbamyl group per mole of hemoglobin tetramer)...