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Congenital Hemolytic Anemia in the Newborn: Relationship to Kernicterus

Congenital Hemolytic Anemia in the Newborn: Relationship to Kernicterus Abstract The natural history of congenital hemolytic jaundice (congenital hemolytic anemia, hereditary spherocytosis) is well known in the medical literature. A number of reviews have pointed out the usual features of this disease, which is characterized clinically by a positive family history, dominant mode of inheritance, recurrent crises, splenomegaly, secondary cholelithiasis, microspherocytes in the blood smear, increased RBC osmotic fragility in hypotonic saline, and effective treatment by means of splenectomy.1-5 The age of onset of clinical symptoms has been variable but is stated to be most commonly in childhood or in young adulthood.4 There are a number of reports concerning symptoms in infancy, the cases usually attracting attention because of anemia.6-12 The often-quoted maxim, "children, and especially infants, with this disease are more sick than jaundiced," reflects the concepts regarding the usual findings in this age group.6 Debré 7 stated that jaundice never shows during the first years, References 1. Tileston, W.: Hemolytic Jaundice , Medicine 1:355, 1922.Crossref 2. Meulengracht, E.: Der chronische hereditäre hämolytische Ikterus , Leipzig, Dr. Werner Klinkhardt, 1922. 3. Dawson, L.: Hemolytic Icterus , Brit. M. J. 1:921, 1931.Crossref 4. Dacie, J. F.: The Hemolytic Anemias , New York, Grune & Stratton, Inc., 1954. 5. Young, L. E.: Hereditary Spherocytosis , Am. J. Med. 18:486, 1955.Crossref 6. Diamond, L. K.: Congenital Hemolytic Anemia in Infancy and Childhood , M. Clin. North America 21:401, 1937. 7. Debré, R.; Lamy, M.; Sée, G., and Schrameck, G.: Congenital and Familial Hemolytic Disease in Children , Am. J. Dis. Child. 56:1189, 1938. 8. Fallon, M.: Jaundice and Conditions Associated with This Phenomenon , J. Pediat. 23:721, 1943.Crossref 9. Conrad, E., and Schmidt, R. E.: Congenital Hemolytic Anemia—a Case Requiring Early Splenectomy , Am. J. Dis. Child. 72:731, 1946. 10. Macauley, D.: Acholuric Jaundice in a Newborn Infant , Arch. Dis. Childhood 26:241, 1951.Crossref 11. Shapiro, C. M.; Josephson, A. M.; Rozengvaig, S., and Kauffman, A.: Hereditary Spherocytosis in the Neonatal Period , J. Pediat. 50:308, 1957.Crossref 12. Robinson, G. C.: Hereditary Spherocytosis in Infancy , J. Pediat. 50:446, 1957.Crossref 13. Newns, G.: Acholuric Jaundice in Infancy , Great Ormond St. J. 1:32, 1951. 14. Bernard, J.; Boiron, M., and Estager, J.: Une grande famille hémolytique , Semaine hôp. Paris 28.4:3741, 1952. 15. Roddy, R., Editor: Clinical Conference at St. Christopher's Hospital for Children: Two Cases of Hereditary Spherocytosis in the Newborn Period , J. Pediat. 44:213, 1954.Crossref 16. Betke, K.: Icterus neonatorum gravis als Manifestation einer hereditären Sphärocytose , Ztschr. Kinderh. 78:359, 1956.Crossref 17. Robinson, G. C.; Phillips, R. M., and Prystowsky, M.: Spherocytosis and Increased Fragility Occurring in Erythroblastosis Fetalis Associated with ABO Incompatibility , Pediatrics 7:164, 1951. 18. Hsia, D. Y. Y., and Gellis, S. S.: Studies on Erythroblastosis Due to ABO Incompatibility , Pediatrics 13:503, 1954. 19. Zuelzer, W. W., and Cohen, F.: ABO Hemolytic Disease and Heterospecific Pregnancy , Pediat. Clin. North America 1957, 405. 20. Gellis, S. S., and Hsia, D. Y. Y.: Jaundice in Infancy , Pediat. Clin. North America 1955, 449. 21. Hsia, D. Y. Y.; Allen, F. H., Jr.; Gellis, S. S., and Diamond, L. K.: Erythroblastosis Fetalis: VIII. Studies of Serum Bilirubin in Relation to Kernicterus , New England J. Med. 247:668, 1952.Crossref 22. Zuelzer, W. W., and Mudgett, R. T.: Kernicterus: Etiologic Study Based on 55 Cases , Pediatrics 6:452, 1950. 23. Dacie, J. V., and Mollison, P. L.: Survival of Normal Erythrocytes After Transfusion to Patients with Familial Hemolytic Anemia , Lancet 1:550, 1943.Crossref 24. King, H., and Shumacker, H. B., Jr.: Splenic Studies: Susceptibility to Infection After Splenectomy Performed in Infancy , Ann. Surg. 136:239, 1952.Crossref 25. Gofstein, 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 26. Owren, P. A.: Congenital Hemolytic Jaundice , Blood 3:231, 1948. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Congenital Hemolytic Anemia in the Newborn: Relationship to Kernicterus

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References (30)

Publisher
American Medical Association
Copyright
Copyright © 1957 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1957.04030070028005
Publisher site
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Abstract

Abstract The natural history of congenital hemolytic jaundice (congenital hemolytic anemia, hereditary spherocytosis) is well known in the medical literature. A number of reviews have pointed out the usual features of this disease, which is characterized clinically by a positive family history, dominant mode of inheritance, recurrent crises, splenomegaly, secondary cholelithiasis, microspherocytes in the blood smear, increased RBC osmotic fragility in hypotonic saline, and effective treatment by means of splenectomy.1-5 The age of onset of clinical symptoms has been variable but is stated to be most commonly in childhood or in young adulthood.4 There are a number of reports concerning symptoms in infancy, the cases usually attracting attention because of anemia.6-12 The often-quoted maxim, "children, and especially infants, with this disease are more sick than jaundiced," reflects the concepts regarding the usual findings in this age group.6 Debré 7 stated that jaundice never shows during the first years, References 1. Tileston, W.: Hemolytic Jaundice , Medicine 1:355, 1922.Crossref 2. Meulengracht, E.: Der chronische hereditäre hämolytische Ikterus , Leipzig, Dr. Werner Klinkhardt, 1922. 3. Dawson, L.: Hemolytic Icterus , Brit. M. J. 1:921, 1931.Crossref 4. Dacie, J. F.: The Hemolytic Anemias , New York, Grune & Stratton, Inc., 1954. 5. Young, L. E.: Hereditary Spherocytosis , Am. J. Med. 18:486, 1955.Crossref 6. Diamond, L. K.: Congenital Hemolytic Anemia in Infancy and Childhood , M. Clin. North America 21:401, 1937. 7. Debré, R.; Lamy, M.; Sée, G., and Schrameck, G.: Congenital and Familial Hemolytic Disease in Children , Am. J. Dis. Child. 56:1189, 1938. 8. Fallon, M.: Jaundice and Conditions Associated with This Phenomenon , J. Pediat. 23:721, 1943.Crossref 9. Conrad, E., and Schmidt, R. E.: Congenital Hemolytic Anemia—a Case Requiring Early Splenectomy , Am. J. Dis. Child. 72:731, 1946. 10. Macauley, D.: Acholuric Jaundice in a Newborn Infant , Arch. Dis. Childhood 26:241, 1951.Crossref 11. Shapiro, C. M.; Josephson, A. M.; Rozengvaig, S., and Kauffman, A.: Hereditary Spherocytosis in the Neonatal Period , J. Pediat. 50:308, 1957.Crossref 12. Robinson, G. C.: Hereditary Spherocytosis in Infancy , J. Pediat. 50:446, 1957.Crossref 13. Newns, G.: Acholuric Jaundice in Infancy , Great Ormond St. J. 1:32, 1951. 14. Bernard, J.; Boiron, M., and Estager, J.: Une grande famille hémolytique , Semaine hôp. Paris 28.4:3741, 1952. 15. Roddy, R., Editor: Clinical Conference at St. Christopher's Hospital for Children: Two Cases of Hereditary Spherocytosis in the Newborn Period , J. Pediat. 44:213, 1954.Crossref 16. Betke, K.: Icterus neonatorum gravis als Manifestation einer hereditären Sphärocytose , Ztschr. Kinderh. 78:359, 1956.Crossref 17. Robinson, G. C.; Phillips, R. M., and Prystowsky, M.: Spherocytosis and Increased Fragility Occurring in Erythroblastosis Fetalis Associated with ABO Incompatibility , Pediatrics 7:164, 1951. 18. Hsia, D. Y. Y., and Gellis, S. S.: Studies on Erythroblastosis Due to ABO Incompatibility , Pediatrics 13:503, 1954. 19. Zuelzer, W. W., and Cohen, F.: ABO Hemolytic Disease and Heterospecific Pregnancy , Pediat. Clin. North America 1957, 405. 20. Gellis, S. S., and Hsia, D. Y. Y.: Jaundice in Infancy , Pediat. Clin. North America 1955, 449. 21. Hsia, D. Y. Y.; Allen, F. H., Jr.; Gellis, S. S., and Diamond, L. K.: Erythroblastosis Fetalis: VIII. Studies of Serum Bilirubin in Relation to Kernicterus , New England J. Med. 247:668, 1952.Crossref 22. Zuelzer, W. W., and Mudgett, R. T.: Kernicterus: Etiologic Study Based on 55 Cases , Pediatrics 6:452, 1950. 23. Dacie, J. V., and Mollison, P. L.: Survival of Normal Erythrocytes After Transfusion to Patients with Familial Hemolytic Anemia , Lancet 1:550, 1943.Crossref 24. King, H., and Shumacker, H. B., Jr.: Splenic Studies: Susceptibility to Infection After Splenectomy Performed in Infancy , Ann. Surg. 136:239, 1952.Crossref 25. Gofstein, 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 26. Owren, P. A.: Congenital Hemolytic Jaundice , Blood 3:231, 1948.

Journal

A.M.A. Journal of Diseases of Children – American Medical Association

Published: Dec 1, 1957

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