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Exchange Transfusion in a Neonatal Crisis of Congenital Hemolytic Anemia

Exchange Transfusion in a Neonatal Crisis of Congenital Hemolytic Anemia Abstract Hereditary spherocytosis is a congenital hemolytic disease which is characterized by familial transmission, moderate anemia, and decreased red blood cell survival time; red blood cells which demonstrate a spherical configuration and increased mechanical and osmotic fragility; jaundice, splenomegaly, and marked improvement following splenectomy. Despite persistence of the basic defect in the erythrocytes, splenectomy "promptly, completely, and permanently relieves anemia and jaundice in nearly all cases of hereditary spherocytosis."2 Some workers have reported an increased incidence of severe and often fulminating infections following splenectomy in early childhood, suggesting decreased resistance to infection after splenectomy.2,3 One suggests use of more rigid criteria for splenectomy and close postoperative supervision. Another recent article suggests that there is an increased incidence of severe infection in splenectomized persons but that there is no correlation between age at splenectomy and severe sepsis.4 The procedure of exchange transfusion has been used in a variety of References 1. Young, L. E.: Hereditary Spherocytosis , Am. J. Med. 18:486, 1955.Crossref 2. King, H., and Shumacker, H. B., Jr.: Splenic Studies: I. Susceptibility to Infection After Splenectomy Performed in Infancy , Ann. Surg , 136:239, 1952.Crossref 3. Smith, C. H.; Erlandson, M.; Schulman, I., and Stern, G.: Hazard of Severe Infections in Splenectomized Infants and Children , Am. J. Med. 22: 390, 1955.Crossref 4. Gof stein, R., and Gellis, S. S.: Splenectomy in Infancy and Childhood: The Question of Overwhelming Infection Following Operation , A.M.A. J. Dis. Child. 91:566, 1956.Crossref 5. Wallerstein, H.: Treatment of Severe Erythroblastosis by Simultaneous Removal and Replacement of Blood of Newborn Infant , Science 103:583, 1946.Crossref 6. Bessis, M.: The Use of Replacement Transfusion in Diseases Other Than Hemolytic Disease of the Newborn , Blood 4:324, 1949. 7. Kuhns, W. J., and Bauerlein, T. C.: Exchange Transfusion in Hemolytic Anemia Complicating Disseminated Lupus Erythematosus , A.M.A. Arch. Int. Med. 92:284, 1953.Crossref 8. Clinical Conference at St. Christopher's Hospital for Children, Philadelphia, J. Pediat. 44:213, 1954.Crossref 9. Allen, F. H., Jr., and Diamond, L. K.: Erythroblastosis Fetalis , New England J. Med. 257:659-668, 705-712, 761-772, 1957.Crossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Exchange Transfusion in a Neonatal Crisis of Congenital Hemolytic Anemia

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References (7)

Publisher
American Medical Association
Copyright
Copyright © 1960 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1960.02070030233014
Publisher site
See Article on Publisher Site

Abstract

Abstract Hereditary spherocytosis is a congenital hemolytic disease which is characterized by familial transmission, moderate anemia, and decreased red blood cell survival time; red blood cells which demonstrate a spherical configuration and increased mechanical and osmotic fragility; jaundice, splenomegaly, and marked improvement following splenectomy. Despite persistence of the basic defect in the erythrocytes, splenectomy "promptly, completely, and permanently relieves anemia and jaundice in nearly all cases of hereditary spherocytosis."2 Some workers have reported an increased incidence of severe and often fulminating infections following splenectomy in early childhood, suggesting decreased resistance to infection after splenectomy.2,3 One suggests use of more rigid criteria for splenectomy and close postoperative supervision. Another recent article suggests that there is an increased incidence of severe infection in splenectomized persons but that there is no correlation between age at splenectomy and severe sepsis.4 The procedure of exchange transfusion has been used in a variety of References 1. Young, L. E.: Hereditary Spherocytosis , Am. J. Med. 18:486, 1955.Crossref 2. King, H., and Shumacker, H. B., Jr.: Splenic Studies: I. Susceptibility to Infection After Splenectomy Performed in Infancy , Ann. Surg , 136:239, 1952.Crossref 3. Smith, C. H.; Erlandson, M.; Schulman, I., and Stern, G.: Hazard of Severe Infections in Splenectomized Infants and Children , Am. J. Med. 22: 390, 1955.Crossref 4. Gof stein, R., and Gellis, S. S.: Splenectomy in Infancy and Childhood: The Question of Overwhelming Infection Following Operation , A.M.A. J. Dis. Child. 91:566, 1956.Crossref 5. Wallerstein, H.: Treatment of Severe Erythroblastosis by Simultaneous Removal and Replacement of Blood of Newborn Infant , Science 103:583, 1946.Crossref 6. Bessis, M.: The Use of Replacement Transfusion in Diseases Other Than Hemolytic Disease of the Newborn , Blood 4:324, 1949. 7. Kuhns, W. J., and Bauerlein, T. C.: Exchange Transfusion in Hemolytic Anemia Complicating Disseminated Lupus Erythematosus , A.M.A. Arch. Int. Med. 92:284, 1953.Crossref 8. Clinical Conference at St. Christopher's Hospital for Children, Philadelphia, J. Pediat. 44:213, 1954.Crossref 9. Allen, F. H., Jr., and Diamond, L. K.: Erythroblastosis Fetalis , New England J. Med. 257:659-668, 705-712, 761-772, 1957.Crossref

Journal

A.M.A. Journal of Diseases of ChildrenAmerican Medical Association

Published: Feb 1, 1960

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