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Clinical Manifestations of Primaquine-Sensitive Anemia

Clinical Manifestations of Primaquine-Sensitive Anemia Abstract SEVERAL HEMOLYTIC anemias are characterized by deficiency of the enzyme glucose-6-phosphate dehydrogenase (G-6-PD) in the erythrocyte. Among these are the primaquinesensitive anemias found in Negroes, a group of nonspherocytic anemias found in Caucasians, and the anemia secondary to the ingestion of fava beans in sensitive individuals. Although they share a similar defect in erythrocyte metabolism, these anemias are otherwise different.1-5 When primaquine-sensitive individuals have a severe infection or are exposed to various chemicals and drugs, a hemolytic process may ensue. The mechanism of erythrocyte destruction has not been completely established but a demonstrable and major factor is the deficiency of G-6-PD in the erythrocyte which becomes critical as the cell ages.5 The purpose of this report is to emphasize the variability of clinical manifestations and the diagnostic problems encountered in 14 Negro children and their families with primaquinesensitive anemia. Some clinical patterns of expression are delineated under the References 1. Beutler, E.: " Drug-Induced Hemolytic Anemia (Primaquine Sensitivity) ," in Stanbury, J.B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Diseases , New York: McGraw-Hill Book Co., Inc., 1960, chap 32, pp 1031-1067. 2. Kirkman, H.N.: " Primaquine Sensitivity ," in Gardner, L.E. (ed.): Molecular Genetics and Human Disease , Springfield, Ill: Charles C Thomas, Publisher, 1961, chap 7, pp 106-133. 3. Marks, P.A., and Gross, R.T.: Erythrocyte Glucose-6-Phosphate Dehydrogenase Deficiency: Evidence of Differences Between Negroes and Caucasians With Respect to This Genetically Determined Trait , J Clin Invest 38:2253-2262 ( (December) ) 1959.Crossref 4. Zinkham, W.H.: Metabolism of Glucose by Erythrocytes From Patients With Congenital Hemolytic Disorders , J Pediat 60:614-622 ( (April) ) 1962.Crossref 5. Tarlov, A.R., et al: Primaquine Sensitivity: Glucose-6-Phosphate Dehydrogenase Deficiency; An Inborn Error of Metabolism of Medical and Biological Significance , Arch Intern Med 109:209-234 ( (February) ) 1962.Crossref 6. Brewer, G.J.; Tarlov, A.R.; and Alving, A.S.: The Methemoglobin Reduction Test for Primaquine-Type Sensitivity of Erythrocytes: A Simplified Procedure for Detecting a Specific Hypersusceptibility to Drug Hemolysis , JAMA 180:386-388 ( (May 5) ) 1962.Crossref 7. Beutler, E.: The Glutathione Instability of Drug-Sensitive Red Cells: A New Method for the in Vitro Detection of Drug Sensitivity , J Lab Clin Med 49:84-95 ( (January) ) 1957. 8. Mohler, D.N., and Williams, W.J.: The Effect of Pheylhydrazine on the Adenosine Triphosphate Content of Normal and Glucose-6-Phosphate Dehydrogenase-Deficient Human Blood , J Clin Invest 40:1735-1742 ( (September) ) 1961.Crossref 9. Naylor, J., et al: Activity of Glucose-6-Phosphate Dehydrogenase in Erythrocytes of Patients With Various Abnormal Hemoglobins , Pediatrics 26:285-292 ( (August) ) 1960. 10. Kirkman, H.N.; Schettini, F.; and Pickard, B.M.: Mediterranean Variant of Glucose-6-Phosphate Dehydrogenase , J Lab Clin Med 63:726-735 ( (May) ) 1964. 11. Kirkman, H.N., and Hendrickson, E.M.: Sex-Linked Electrophoretic Difference in Glucose-6-Phosphate Dehydrogenase , Amer J Hum Genet 15:241-258 ( (September) ) 1963. 12. Gross, R.T.; Hurwitz, R.E.; and Marks, P.A.: An Hereditary Enzymatic Defect in Erythrocyte Metabolism: Glucose-6-Phosphate Dehydrogenase Deficiency , J Clin Invest 37: 1176-1184 ( (August) ) 1958.Crossref 13. Jandl, J.H.: The Heinz Body Hemolytic Anemias , Ann Intern Med 58:702-709 ( (April) ) 1963.Crossref 14. Kellermeyer, R.W., et al: Hemolytic Effect of Therapeutic Drugs: Clinical Considerations of the Primaquine-Type Hemolysis , JAMA 180:388-394 ( (May 5) ) 1962.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png American Journal of Diseases of Children American Medical Association

Clinical Manifestations of Primaquine-Sensitive Anemia

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References (14)

Publisher
American Medical Association
Copyright
Copyright © 1965 American Medical Association. All Rights Reserved.
ISSN
0002-922X
DOI
10.1001/archpedi.1965.02090030176013
Publisher site
See Article on Publisher Site

Abstract

Abstract SEVERAL HEMOLYTIC anemias are characterized by deficiency of the enzyme glucose-6-phosphate dehydrogenase (G-6-PD) in the erythrocyte. Among these are the primaquinesensitive anemias found in Negroes, a group of nonspherocytic anemias found in Caucasians, and the anemia secondary to the ingestion of fava beans in sensitive individuals. Although they share a similar defect in erythrocyte metabolism, these anemias are otherwise different.1-5 When primaquine-sensitive individuals have a severe infection or are exposed to various chemicals and drugs, a hemolytic process may ensue. The mechanism of erythrocyte destruction has not been completely established but a demonstrable and major factor is the deficiency of G-6-PD in the erythrocyte which becomes critical as the cell ages.5 The purpose of this report is to emphasize the variability of clinical manifestations and the diagnostic problems encountered in 14 Negro children and their families with primaquinesensitive anemia. Some clinical patterns of expression are delineated under the References 1. Beutler, E.: " Drug-Induced Hemolytic Anemia (Primaquine Sensitivity) ," in Stanbury, J.B.; Wyngaarden, J.B.; and Fredrickson, D.S. (eds.): The Metabolic Basis of Inherited Diseases , New York: McGraw-Hill Book Co., Inc., 1960, chap 32, pp 1031-1067. 2. Kirkman, H.N.: " Primaquine Sensitivity ," in Gardner, L.E. (ed.): Molecular Genetics and Human Disease , Springfield, Ill: Charles C Thomas, Publisher, 1961, chap 7, pp 106-133. 3. Marks, P.A., and Gross, R.T.: Erythrocyte Glucose-6-Phosphate Dehydrogenase Deficiency: Evidence of Differences Between Negroes and Caucasians With Respect to This Genetically Determined Trait , J Clin Invest 38:2253-2262 ( (December) ) 1959.Crossref 4. Zinkham, W.H.: Metabolism of Glucose by Erythrocytes From Patients With Congenital Hemolytic Disorders , J Pediat 60:614-622 ( (April) ) 1962.Crossref 5. Tarlov, A.R., et al: Primaquine Sensitivity: Glucose-6-Phosphate Dehydrogenase Deficiency; An Inborn Error of Metabolism of Medical and Biological Significance , Arch Intern Med 109:209-234 ( (February) ) 1962.Crossref 6. Brewer, G.J.; Tarlov, A.R.; and Alving, A.S.: The Methemoglobin Reduction Test for Primaquine-Type Sensitivity of Erythrocytes: A Simplified Procedure for Detecting a Specific Hypersusceptibility to Drug Hemolysis , JAMA 180:386-388 ( (May 5) ) 1962.Crossref 7. Beutler, E.: The Glutathione Instability of Drug-Sensitive Red Cells: A New Method for the in Vitro Detection of Drug Sensitivity , J Lab Clin Med 49:84-95 ( (January) ) 1957. 8. Mohler, D.N., and Williams, W.J.: The Effect of Pheylhydrazine on the Adenosine Triphosphate Content of Normal and Glucose-6-Phosphate Dehydrogenase-Deficient Human Blood , J Clin Invest 40:1735-1742 ( (September) ) 1961.Crossref 9. Naylor, J., et al: Activity of Glucose-6-Phosphate Dehydrogenase in Erythrocytes of Patients With Various Abnormal Hemoglobins , Pediatrics 26:285-292 ( (August) ) 1960. 10. Kirkman, H.N.; Schettini, F.; and Pickard, B.M.: Mediterranean Variant of Glucose-6-Phosphate Dehydrogenase , J Lab Clin Med 63:726-735 ( (May) ) 1964. 11. Kirkman, H.N., and Hendrickson, E.M.: Sex-Linked Electrophoretic Difference in Glucose-6-Phosphate Dehydrogenase , Amer J Hum Genet 15:241-258 ( (September) ) 1963. 12. Gross, R.T.; Hurwitz, R.E.; and Marks, P.A.: An Hereditary Enzymatic Defect in Erythrocyte Metabolism: Glucose-6-Phosphate Dehydrogenase Deficiency , J Clin Invest 37: 1176-1184 ( (August) ) 1958.Crossref 13. Jandl, J.H.: The Heinz Body Hemolytic Anemias , Ann Intern Med 58:702-709 ( (April) ) 1963.Crossref 14. Kellermeyer, R.W., et al: Hemolytic Effect of Therapeutic Drugs: Clinical Considerations of the Primaquine-Type Hemolysis , JAMA 180:388-394 ( (May 5) ) 1962.Crossref

Journal

American Journal of Diseases of ChildrenAmerican Medical Association

Published: Aug 1, 1965

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