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Kernicterus in Rats with an Inherited Deficiency of Glucuronyl Transferase

Kernicterus in Rats with an Inherited Deficiency of Glucuronyl Transferase Abstract This paper describes the occurrence of kernicterus and factors which influence its development in the strain of rats * first studied by Gunn.22,23 The disease of humans designated in 1903 by Schmorl39 as kernicterus has, in our opinion, not been observed or induced in its entirety in animals. Various workers, however, have demonstrated the toxicity of indirect bilirubin.12-15,24,26,44-46 Ernster, Herlin, and Zetterström21 have caused localized pigmentation of the brain of the rabbit by treatment with bilirubin and an S-H blocking agent. Other workers17,19,20 have reported injury of brain tissue in hemolytic anemia of animals, a phenomenon not directly related to the present report, since the rats to be described do not suffer from antibody-antigen disease and have little or no hemolytic Definition of Kernicterus The following criteria are proposed as defining kernicterus: Distinctive evidence of central nervous system dysfunction during life in jaundiced subjects Canary yellow References 1. Arias, I. M., and London, I. M.: Bilirubin Glucuronide Formation in Vitro; Demonstration of a Defect in Gilbert's Disease , Science 126: 563, 1957.Crossref 2. Billing, B. H., and Lathe, G. H.: The Excretion of Bilirubin as an Ester Glucuronide, Giving the Direct van den Bergh Reaction , Biochem. J. 63:6P, 1956. 3. Billing, B. H.; Cole, P. G., and Lathe, G. H.: The Excretion of Bilirubin as as Diglucuronide Giving the Direct van den Bergh Reaction , Biochem, J. 65:774, 1957. 4. Bratton, A. C., and Marshall, E. K.: A New Coupling Component for Sulfanilamide Determination , J. Biol. Chem. 128:537, 1939. 5. Brown, A. K., and Zuelzer, W. W.: Mechanism and Significance of Hyperbilirubinemia in the Newborn with Reference to Kernicterus, read before the 11th Annual Meeting, American Academy for Cerebral Palsy, New Orleans, Nov., 1957. 6. Carbone, J. V., and Grodsky, G. M.: Constitutional Nonhemolytic Hyperbilirubinemia in the Rat: Defect of Bilirubin Conjugation , Proc. Soc. Exper. Biol. & Med. 94:461, 1957. 7. Childs, B., and Najjar, V. A.: Familial Nonhemolytic Jaundice with Kernicterus: a Report of 2 Cases Without Neurologic Damage , Pediatrics 18:369, 1956. 8. Cole, P. G.; Lathe, G. H., and Billing, B. H.: Separation of the Bile Pigments of Serum, Bile and Urine , Biochem. J. 57:514, 1954. 9. Crigler, J. F., and Najjar, V. A.: Congenital Familial Nonhemolytic Jaundice with Kernicterus , Pediatrics 10:169, 1952. 10. Danoff, S.; Boyer, A., and Holt, L. E., Jr.: Observations on the Control of Bilirubinemia, Program of the 28th Annual Meeting of the Society for Pediatric Research, Atlantic City, May 6-7, 1958, Abstract 13, p. 11. 11. Davis, B.: The Binding of Sulfonamide Drugs by Plasma Proteins: A Factor in Determining the Distribution of Drugs in the Body , J. Clin. Invest. 22:753, 1943. 12. Day, R. L.: In Vitro Effect of Bilirubin on Brain Respiration, presented at the 25th Anniversary Celebration of Columbia-Presbyterian Medical Center , Babies Hospital, New York, Oct., 1953. 13. Day, R. L.: Inhibition of Brain Respiration in Vitro by Bilirubin: Reversal of Inhibition by Various Means , Proc. Soc. Exper. Biol. & Med. 85:261, 1954. 14. Day, R.: Kernicterus; Further Observations on the Toxicity of Heme Pigments , Pediatrics 17: 925, 1956. 15. Day, R. L., and Johnson, L.: Kernicterus, to be published. 16. Dameshek, W., and Singer, K.: Familial Nonhemolytic Jaundice , Arch. Int. Med. 67:259, 1941. 17. Dereymaeker, A.: L'ictere nucléaire du nouveau-né , Bruxelles, Editions Arscia, 1949. 18. Dutton, G. J., and Storey, I. D. E.: Uridine Compounds in Gluronic Acid and Metabolism: The Isolation and Structure of Uridine-Diphosphate-Glucuronic Acid , Biochem. J. 59:279, 1955. 19. Eyquem, A.: L'atteinte du systéme nerveux central chez les jeunes animaux présentant une maladie hémolytique expérimentale , Compt. rend. Soc. biol. 142:485, 1948. 20. Eyquem, A.: L'ictére nucléaire expérimental , Acta neurol. et psychiat. belg. 49:965, 1949. 21. Ernster, L.; Herlin, L., and Zetterström, R.: Experimental Studies on the Pathogenesis of Kernicterus , Pediatrics 20:647, 1957. 22. Gunn, C. K.: Hereditary Acholuric Jaundice in a New Mutant Strain of Rats , J. Hered. 29:137, 1938. 23. Gunn, C. K.: Hereditary Acholuric Jaundice in the Rat , Canad. M. A. J. 50:230, 1944. 24. Küster, F., and Krings, H.: Die Bedeutung des Bilirubins für die Pathogenese des Hirnschadens bei der Erythroblastosis fetalis , Ztschr. Kinderh. 67:503, 1950. 25. Karunairatnam, M. C.; Kerr, L. M. H., and Levvy, G. A.: The Glucuronide-Synthesizing System in the Mouse, and Its Relationship to B-Glucuronidase , Biochem. J. 45:496, 1949. 26. Lathe, G. H.: The Chemical Pathology of Bile Pigments: Part I. The Plasma Bile Pigments , Biochemical Society Symposia , No. 12, London, Cambridge University Press, 1954, p. 34. 27. Lathe, G. H., and Walker, M.: An Enzyme Defect in Human Neonatal Jaundice and in Gunn's Strain of Jaundiced Rats , Biochem. J. 67: 9P, 1957. 28. Lathe, G. H., and Walker, M.: The Inhibitory Effect of Human Pregnancy Serum on Bilirubin Conjugation, to be published. 29. Levvy, G. A.: Glucuronide Metabolism with Special Reference to the Steroid Hormones , Vitamins & Hormones 14:267, 1956. 30. Lipschitz, W. L., and Bueding, E.: Mechanism of the Biological Formation of Conjugated Glucuronic Acids , J. Biol. Chem. 129:333, 1939. 31. Malloy, H. T., and Lowenstein, L.: Hereditary Jaundice in the Rat , Canad. M. A. J. 42:122, 1940. 32. Malloy, H. T., and Evelyn, K. A.: The Determination of Bilirubin with the Photoelectric Colorimeter , J. Biol. Chem. 119:481, 1937. 33. Meulengracht, E.: Review of Chronic Intermittent Juvenile Jaundice , Quart. J. Med. 16:83, 1947. 34. Odell, G. B.: In Vitro Studies of the Effect of Sulfonamides on Bilirubin, Program of the 28th Annual Meeting of the Society for Pediatric Research, Atlantic City, May 6-7, 1958, Abstract 156, p. 147. 35. Rosenthal, I. M.; Zimmerman, H. J., and Hardy, N.: Congenital Nonhemolytic Jaundice with Disease of the Central Nervous System , Pediatrics 18:378, 1956. 36. Schmid, R.: Bilirubin Glucuronide, the Direct Reacting Bilirubin, in Serum, Bile and Urine , Science 124:76, 1956. 37. Schmid, R.; Hammaker, L., and Axelrod, J.: The Enzymatic Formation of Bilirubin Glucuronide , Arch. Biochem. 70:285, 1957. 38. Schmid, R.; Axelrod, J., and Hammaker, L.: Congenital Defects in Bilirubin Metabolism , American Society of Clinical Investigation , May, 1957, abstract, p. 63. 39. Schmorl, G.: Zur Kenntnis des Ikterus neonatorum, insbesondere der dabei auftretenden Gehirnveränderungen , Verhandl. deutsch. path. Gesellsch. 15:109-115, 1904. 40. Silverman, W. A.; Andersen, D. H.; Blanc, W. A., and Crozier, D. N.: A Difference in Mortality Rate and Incidence of Kernicterus Among Premature Infants Allotted to Two Prophylactic Antibacterial Regimens , Pediatrics 18:614, 1956. 41. Strominger, J. L.; Kalckar, H. M.; Axelrod, J., and Manvell, E. S.: The Enzymatic Oxidation of Uridine Diphosphate Glucose to Uridine Diphosphate Glucuronic Acid , J. Am. Chem. Soc. 76:6411, 1954. 42. Strominger, J. L.; Maxwell, E. S.; Axelrod, J., and Kalckar, H. M.: The Enzymatic Formation of Uridine Diphosphoglucuronic Acid , J. Biol. Chem. 224:79, 1957. 43. Talafant, E.: Properties and Composition of the Bile Pigment Giving a Direct Diazo Reaction , Nature , London 178:312, 1956. 44. Waters, W. J. W.; Richart, D. A., and Rawson, H. H.: Bilirubin Encephalopathy , Pediatrics 13:319, 1954. 45. Waters, W. J. W., and Britton, H. A.: Bilirubin Encephalopathy: Preliminary Studies Related to Production , Pediatrics 15:45, 1955. 46. Zetterström, R., and Ernster, L.: Bilirubin, an Uncoupler of Oxidative Phosphorylation in Isolated Mitochondria , Nature , London 178:1335, 1956. http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png A.M.A. Journal of Diseases of Children American Medical Association

Kernicterus in Rats with an Inherited Deficiency of Glucuronyl Transferase

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Publisher
American Medical Association
Copyright
Copyright © 1959 American Medical Association. All Rights Reserved.
ISSN
0096-6916
DOI
10.1001/archpedi.1959.02070010593009
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Abstract

Abstract This paper describes the occurrence of kernicterus and factors which influence its development in the strain of rats * first studied by Gunn.22,23 The disease of humans designated in 1903 by Schmorl39 as kernicterus has, in our opinion, not been observed or induced in its entirety in animals. Various workers, however, have demonstrated the toxicity of indirect bilirubin.12-15,24,26,44-46 Ernster, Herlin, and Zetterström21 have caused localized pigmentation of the brain of the rabbit by treatment with bilirubin and an S-H blocking agent. Other workers17,19,20 have reported injury of brain tissue in hemolytic anemia of animals, a phenomenon not directly related to the present report, since the rats to be described do not suffer from antibody-antigen disease and have little or no hemolytic Definition of Kernicterus The following criteria are proposed as defining kernicterus: Distinctive evidence of central nervous system dysfunction during life in jaundiced subjects Canary yellow References 1. Arias, I. M., and London, I. M.: Bilirubin Glucuronide Formation in Vitro; Demonstration of a Defect in Gilbert's Disease , Science 126: 563, 1957.Crossref 2. Billing, B. H., and Lathe, G. H.: The Excretion of Bilirubin as an Ester Glucuronide, Giving the Direct van den Bergh Reaction , Biochem. J. 63:6P, 1956. 3. Billing, B. H.; Cole, P. G., and Lathe, G. H.: The Excretion of Bilirubin as as Diglucuronide Giving the Direct van den Bergh Reaction , Biochem, J. 65:774, 1957. 4. Bratton, A. C., and Marshall, E. K.: A New Coupling Component for Sulfanilamide Determination , J. Biol. Chem. 128:537, 1939. 5. Brown, A. K., and Zuelzer, W. W.: Mechanism and Significance of Hyperbilirubinemia in the Newborn with Reference to Kernicterus, read before the 11th Annual Meeting, American Academy for Cerebral Palsy, New Orleans, Nov., 1957. 6. Carbone, J. V., and Grodsky, G. M.: Constitutional Nonhemolytic Hyperbilirubinemia in the Rat: Defect of Bilirubin Conjugation , Proc. Soc. Exper. Biol. & Med. 94:461, 1957. 7. Childs, B., and Najjar, V. A.: Familial Nonhemolytic Jaundice with Kernicterus: a Report of 2 Cases Without Neurologic Damage , Pediatrics 18:369, 1956. 8. Cole, P. G.; Lathe, G. H., and Billing, B. H.: Separation of the Bile Pigments of Serum, Bile and Urine , Biochem. J. 57:514, 1954. 9. Crigler, J. F., and Najjar, V. A.: Congenital Familial Nonhemolytic Jaundice with Kernicterus , Pediatrics 10:169, 1952. 10. Danoff, S.; Boyer, A., and Holt, L. E., Jr.: Observations on the Control of Bilirubinemia, Program of the 28th Annual Meeting of the Society for Pediatric Research, Atlantic City, May 6-7, 1958, Abstract 13, p. 11. 11. Davis, B.: The Binding of Sulfonamide Drugs by Plasma Proteins: A Factor in Determining the Distribution of Drugs in the Body , J. Clin. Invest. 22:753, 1943. 12. Day, R. L.: In Vitro Effect of Bilirubin on Brain Respiration, presented at the 25th Anniversary Celebration of Columbia-Presbyterian Medical Center , Babies Hospital, New York, Oct., 1953. 13. Day, R. L.: Inhibition of Brain Respiration in Vitro by Bilirubin: Reversal of Inhibition by Various Means , Proc. Soc. Exper. Biol. & Med. 85:261, 1954. 14. Day, R.: Kernicterus; Further Observations on the Toxicity of Heme Pigments , Pediatrics 17: 925, 1956. 15. Day, R. L., and Johnson, L.: Kernicterus, to be published. 16. Dameshek, W., and Singer, K.: Familial Nonhemolytic Jaundice , Arch. Int. Med. 67:259, 1941. 17. Dereymaeker, A.: L'ictere nucléaire du nouveau-né , Bruxelles, Editions Arscia, 1949. 18. Dutton, G. J., and Storey, I. D. E.: Uridine Compounds in Gluronic Acid and Metabolism: The Isolation and Structure of Uridine-Diphosphate-Glucuronic Acid , Biochem. J. 59:279, 1955. 19. Eyquem, A.: L'atteinte du systéme nerveux central chez les jeunes animaux présentant une maladie hémolytique expérimentale , Compt. rend. Soc. biol. 142:485, 1948. 20. Eyquem, A.: L'ictére nucléaire expérimental , Acta neurol. et psychiat. belg. 49:965, 1949. 21. Ernster, L.; Herlin, L., and Zetterström, R.: Experimental Studies on the Pathogenesis of Kernicterus , Pediatrics 20:647, 1957. 22. Gunn, C. K.: Hereditary Acholuric Jaundice in a New Mutant Strain of Rats , J. Hered. 29:137, 1938. 23. Gunn, C. K.: Hereditary Acholuric Jaundice in the Rat , Canad. M. A. J. 50:230, 1944. 24. Küster, F., and Krings, H.: Die Bedeutung des Bilirubins für die Pathogenese des Hirnschadens bei der Erythroblastosis fetalis , Ztschr. Kinderh. 67:503, 1950. 25. Karunairatnam, M. C.; Kerr, L. M. H., and Levvy, G. A.: The Glucuronide-Synthesizing System in the Mouse, and Its Relationship to B-Glucuronidase , Biochem. J. 45:496, 1949. 26. Lathe, G. H.: The Chemical Pathology of Bile Pigments: Part I. The Plasma Bile Pigments , Biochemical Society Symposia , No. 12, London, Cambridge University Press, 1954, p. 34. 27. Lathe, G. H., and Walker, M.: An Enzyme Defect in Human Neonatal Jaundice and in Gunn's Strain of Jaundiced Rats , Biochem. J. 67: 9P, 1957. 28. Lathe, G. H., and Walker, M.: The Inhibitory Effect of Human Pregnancy Serum on Bilirubin Conjugation, to be published. 29. Levvy, G. A.: Glucuronide Metabolism with Special Reference to the Steroid Hormones , Vitamins & Hormones 14:267, 1956. 30. Lipschitz, W. L., and Bueding, E.: Mechanism of the Biological Formation of Conjugated Glucuronic Acids , J. Biol. Chem. 129:333, 1939. 31. Malloy, H. T., and Lowenstein, L.: Hereditary Jaundice in the Rat , Canad. M. A. J. 42:122, 1940. 32. Malloy, H. T., and Evelyn, K. A.: The Determination of Bilirubin with the Photoelectric Colorimeter , J. Biol. Chem. 119:481, 1937. 33. Meulengracht, E.: Review of Chronic Intermittent Juvenile Jaundice , Quart. J. Med. 16:83, 1947. 34. Odell, G. B.: In Vitro Studies of the Effect of Sulfonamides on Bilirubin, Program of the 28th Annual Meeting of the Society for Pediatric Research, Atlantic City, May 6-7, 1958, Abstract 156, p. 147. 35. Rosenthal, I. M.; Zimmerman, H. J., and Hardy, N.: Congenital Nonhemolytic Jaundice with Disease of the Central Nervous System , Pediatrics 18:378, 1956. 36. Schmid, R.: Bilirubin Glucuronide, the Direct Reacting Bilirubin, in Serum, Bile and Urine , Science 124:76, 1956. 37. Schmid, R.; Hammaker, L., and Axelrod, J.: The Enzymatic Formation of Bilirubin Glucuronide , Arch. Biochem. 70:285, 1957. 38. Schmid, R.; Axelrod, J., and Hammaker, L.: Congenital Defects in Bilirubin Metabolism , American Society of Clinical Investigation , May, 1957, abstract, p. 63. 39. Schmorl, G.: Zur Kenntnis des Ikterus neonatorum, insbesondere der dabei auftretenden Gehirnveränderungen , Verhandl. deutsch. path. Gesellsch. 15:109-115, 1904. 40. Silverman, W. A.; Andersen, D. H.; Blanc, W. A., and Crozier, D. N.: A Difference in Mortality Rate and Incidence of Kernicterus Among Premature Infants Allotted to Two Prophylactic Antibacterial Regimens , Pediatrics 18:614, 1956. 41. Strominger, J. L.; Kalckar, H. M.; Axelrod, J., and Manvell, E. S.: The Enzymatic Oxidation of Uridine Diphosphate Glucose to Uridine Diphosphate Glucuronic Acid , J. Am. Chem. Soc. 76:6411, 1954. 42. Strominger, J. L.; Maxwell, E. S.; Axelrod, J., and Kalckar, H. M.: The Enzymatic Formation of Uridine Diphosphoglucuronic Acid , J. Biol. Chem. 224:79, 1957. 43. Talafant, E.: Properties and Composition of the Bile Pigment Giving a Direct Diazo Reaction , Nature , London 178:312, 1956. 44. Waters, W. J. W.; Richart, D. A., and Rawson, H. H.: Bilirubin Encephalopathy , Pediatrics 13:319, 1954. 45. Waters, W. J. W., and Britton, H. A.: Bilirubin Encephalopathy: Preliminary Studies Related to Production , Pediatrics 15:45, 1955. 46. Zetterström, R., and Ernster, L.: Bilirubin, an Uncoupler of Oxidative Phosphorylation in Isolated Mitochondria , Nature , London 178:1335, 1956.

Journal

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

Published: May 1, 1959

References