Abstract
A patient with Crigler-Najjar disease has survived with the help of phototherapy to the age of 2 years without neurological damage. Because long periods of phototherapy are a threat to normal development, a search was made for supplementary treatments. Cholestyramine and a high fat diet were effective, and possibly also aspartic acid. Maintenance therapy with cholestyramine allowed the amount of phototherapy given to be reduced.
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Selected References
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- Altay C., Say B. Phototherapy in nonobstructive, nonhemolytic jaundice. Pediatrics. 1973 Jan;51(1):124–126. [PubMed] [Google Scholar]
- Arias I. M., Gartner L. M., Cohen M., Ezzer J. B., Levi A. J. Chronic nonhemolytic unconjugated hyperbilirubinemia with glucuronyl transferase deficiency. Clinical, biochemical, pharmacologic and genetic evidence for heterogeneity. Am J Med. 1969 Sep;47(3):395–409. doi: 10.1016/0002-9343(69)90224-1. [DOI] [PubMed] [Google Scholar]
- Arias I. M. Inheritable and congenital hyperbilirubinemia. Models for the study of drug metabolism. N Engl J Med. 1971 Dec 16;285(25):1416–1421. doi: 10.1056/NEJM197112162852507. [DOI] [PubMed] [Google Scholar]
- Barrett P. V. The effect of diet and fasting on the serum bilirubin concentration in the rat. Gastroenterology. 1971 Apr;60(4):572–576. [PubMed] [Google Scholar]
- Blumenschein S. D., Kallen R. J., Storey B., Natzschka J. C., Odell G. B., Childs B. Familial nonhemolytic jaundice with late onset of neurological damage. Pediatrics. 1968 Nov;42(5):786–792. [PubMed] [Google Scholar]
- Béthenod M., André J. P., Cotte J., Mathieu M., Collombel C., Cotte M. F., Nivelon J. L. La photothérapie dans la maladie de Crigler et Najjar. Intérêt et limites. Ann Pediatr (Paris) 1967 Dec 2;14(12):829–834. [PubMed] [Google Scholar]
- CRIGLER J. F., Jr, NAJJAR V. A. Congenital familial nonhemolytic jaundice with kernicterus. Pediatrics. 1952 Aug;10(2):169–180. [PubMed] [Google Scholar]
- Callahan E. W., Jr, Thaler M. M., Karon M., Bauer K., Schmid R. Phototherapy of severe unconjugated hyperbilirubinemia: formation and removal of labeled bilirubin derivatives. Pediatrics. 1970 Dec;46(6):841–848. [PubMed] [Google Scholar]
- Careddu P., Marini A. Stimulating bilirubin conjugation. Lancet. 1968 May 4;1(7549):982–983. doi: 10.1016/s0140-6736(68)90939-2. [DOI] [PubMed] [Google Scholar]
- Crigler J. F., Jr, Gold N. I. Effect of sodium phenobarbital on bilirubin metabolism in an infant with congenital, nonhemolytic, unconjugated hyperbilirubinemia, and kernicterus. J Clin Invest. 1969 Jan;48(1):42–55. doi: 10.1172/JCI105973. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Girotti F., Finocchi G., Sartori L., Boscherini B. Congenital non-haemolytic jaundice in a four-year-old girl without disease of the central nervous system. Helv Paediatr Acta. 1969 Aug;24(4):399–403. [PubMed] [Google Scholar]
- Gorodischer R., Levy G., Krasner J., Jaffe S. J. Congenital nonobstructive, nonhemolytic jaundice. Effect of phototherapy. N Engl J Med. 1970 Feb 12;282(7):375–377. doi: 10.1056/NEJM197002122820708. [DOI] [PubMed] [Google Scholar]
- Gray D. W., Mowat A. P. Effects of aspartic acid, orotic acid, and glucose on serum bilirubin concentrations in infants born before term. Arch Dis Child. 1971 Feb;46(245):123–124. doi: 10.1136/adc.46.245.123. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Götze H., Sidiropoulos D., Hess F. A., Berthelot P. Das Crigler-Najjar-Syndrom. Klinische, biochemische, morphologische und therapeutische Aspekte. Helv Paediatr Acta. 1972 Jul;27(3):335–351. [PubMed] [Google Scholar]
- Housset E., Etienne J. P., Petite J. P., Oudéa P., Oudéa M. C., Manchon P. Role du régime alimentaire sur la bilirubinémie du rat Gunn. Applications expérimentales: cholépéritoines, cholostases, action de la novobiocine. Pathol Biol. 1967 May;15(9):457–469. [PubMed] [Google Scholar]
- Karon M., Imach D., Schwartz A. Effective phototherapy in congenital nonobstructive, nonhemolytic jaundice. N Engl J Med. 1970 Feb 12;282(7):377–380. doi: 10.1056/NEJM197002122820709. [DOI] [PubMed] [Google Scholar]
- Kintzel H. W., Hinkel G. K., Schwarze R. The decrease in the serum bilirubin level in premature infants by orotic acid. Acta Paediatr Scand. 1971 Jan;60(1):1–5. doi: 10.1111/j.1651-2227.1971.tb06609.x. [DOI] [PubMed] [Google Scholar]
- LESTER R., HAMMAKER L., SCHMID R. A new therapeutic approach to unconjugated hyperbilirubinaemia. Lancet. 1962 Dec 15;2(7268):1257–1257. doi: 10.1016/s0140-6736(62)92820-9. [DOI] [PubMed] [Google Scholar]
- Land V. J., Zarkowsky H. S., Vietti T. J. Phototherapy for jaundice. N Engl J Med. 1970 Feb 12;282(7):397–397. doi: 10.1056/nejm197002122820720. [DOI] [PubMed] [Google Scholar]
- Marini A., Flauto U., Careddu P. Somministrazione di uridindifosfoglucosio (UDPG) e comportamento della bilirubinemia nel neonato. Minerva Pediatr. 1969 Jul 28;21(30):1386–1389. [PubMed] [Google Scholar]
- Matsuda I., Shirahata T. Effects of aspartic acid and orotic acid upon serum bilirubin level in newborn infants. Tohoku J Exp Med. 1966 Oct;90(2):133–136. doi: 10.1620/tjem.90.133. [DOI] [PubMed] [Google Scholar]
- Mowat A. Double-blind trial of effects of aspartic acid, orotic acid and glucose on serum bilirubin concentrations in infants born before term. Arch Dis Child. 1971 Jun;46(247):397–397. doi: 10.1136/adc.46.247.397-a. [DOI] [PMC free article] [PubMed] [Google Scholar]
- Poland R. L., Odell G. B. Physiologic jaundice: the enterohepatic circulation of bilirubin. N Engl J Med. 1971 Jan 7;284(1):1–6. doi: 10.1056/NEJM197101072840101. [DOI] [PubMed] [Google Scholar]
- VISINTINE R. E., MICHAELS G. D., FUKAYAMA G., CONKLIN J., KINSELL L. W. Xanthomatous biliary cirrhosis treated with cholestyramine. A bile-acid-adsorbing resin. Lancet. 1961 Aug 12;2(7198):341–343. doi: 10.1016/s0140-6736(61)90634-1. [DOI] [PubMed] [Google Scholar]
