Effect of Carbonic Anhydrase Inhibitors on the Course of Sickle-Cell Disease

The inhibition of carbonic anhydrase in the mature erythrocyte has been utilized by Hilkovitz ¹ as a therapeutic measure in the treatment of sickle-cell disease. Evidence presented in his observations suggests that definite beneficial results may be expected when the inhibitor, acetazolamide, is administered in the recommended dosage. The logic of this procedure, as presented, is based on the knowledge that carbonic anhydrase, an enzyme present in the mature erythrocyte, catalyzes the reversible reaction. Inhibition of this reaction in sickle-cell disease could presumably affect the degree of hemoglobin reduction and diminish in vivo sickling, with a subsequent increase in erythrocyte count. His optimistic approach to the therapy of sickle-cell disease warranted further evaluation. The purpose of this communication is to present our findings after administration of comparable amounts of carbonic anhydrase inhibitors to subjects having sickle-cell disease.

Clinical Material  Four adult patients having sickle-cell disease were studied. Electrophoretic patterns, genetic