Abstract
Three cases of chronic nonhaemolytic jaundice with conjugated bilirubin in the serum are described in a Chinese family. Bromsulphthalein excretion tests gave results typical of the Dubin-Johnson syndrome. Liver histology in the proband showed cytoplasmic pigment of the lipofuscinmelanin variety, and intravenous cholecystography failed to show visualisation of the gallbladder. Unusual findings included onset during the neonatal period in the proband and the presence of some iron pigment in the hepatic cells with a little canalicular cholestasis. It is suggested that the infant may have had a concomitant nonspecific hepatitis. These cases are regarded as belonging to a disease group in which the Dubin-Johnson syndrome is at one end of a spectrum. The mode of inheritance is discussed.
Full text
PDF




Images in this article
Selected References
These references are in PubMed. This may not be the complete list of references from this article.
- ARIAS I. M. Studies of chronic familial non-hemolytic jaundice with conjugated bilirubin in the serum with and without an unidentified pigment in the liver cells. Am J Med. 1961 Oct;31:510–518. doi: 10.1016/0002-9343(61)90135-8. [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]
- BEKER S., READ A. E. Familial DubinJohnson syndrome. Gastroenterology. 1958 Oct;35(4):387–389. [PubMed] [Google Scholar]
- Berk P. D., Bloomer J. R., Howe R. B., Berlin N. I. Constitutional hepatic dysfunction (Gilbert's syndrome). A new definition based on kinetic studies with unconjugated radiobilirubin. Am J Med. 1970 Sep;49(3):296–305. doi: 10.1016/s0002-9343(70)80020-1. [DOI] [PubMed] [Google Scholar]
- Butt H. R., Anderson V. E., Foulk W. T., Baggenstoss A. H., Schoenfield L. J., Dickson E. R. Studies of chronic idiopathic jaundice (Dubin-Johnson syndrome). II. Evaluation of a large family with the trait. Gastroenterology. 1966 Nov;51(5):619–630. [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]
- DUBIN I. N. Chronic idiopathic jaundice; a review of fifty cases. Am J Med. 1958 Feb;24(2):268–292. doi: 10.1016/0002-9343(58)90315-2. [DOI] [PubMed] [Google Scholar]
- DUBIN I. N., JOHNSON F. B. Chronic idiopathic jaundice with unidentified pigment in liver cells; a new clinicopathologic entity with a report of 12 cases. Medicine (Baltimore) 1954 Sep;33(3):155–197. doi: 10.1097/00005792-195409000-00001. [DOI] [PubMed] [Google Scholar]
- Edwards R. H. Inheritance of the Dubin-Johnson-Sprinz syndrome. Gastroenterology. 1975 Apr;68(4 Pt 1):734–749. [PubMed] [Google Scholar]
- JOHN G. G., KNUDTSON K. P. Chronic idiopathic jaundice; two cases occurring in siblings, with histochemical studies. Am J Med. 1956 Jul;21(1):138–142. doi: 10.1016/0002-9343(56)90030-4. [DOI] [PubMed] [Google Scholar]
- Kondo T., Yagi R., Kuchiba K. Letter: Dubin-Johnson syndrome in a neonate. N Engl J Med. 1975 May 8;292(19):1028–1029. doi: 10.1056/nejm197505082921913. [DOI] [PubMed] [Google Scholar]
- MANDEMA E., de FRAITURE W., NIEWEG H. O., ARENDS A. Familial chronic idiopathic jaundice (Dubin-Sprinz disease), with a note on bromsulphalein metabolism in this disease. Am J Med. 1960 Jan;28:42–50. doi: 10.1016/0002-9343(60)90221-7. [DOI] [PubMed] [Google Scholar]
- SCHIFF L., BILLING B. H., OIKAWA Y. Familial nonhemolytic jaundice with conjugated bilirubin in the serum; a case study. N Engl J Med. 1959 Jun 25;260(26):1315–1318. doi: 10.1056/NEJM195906252602604. [DOI] [PubMed] [Google Scholar]
- SCHOENFIELD L. J., McGILL D. B., HUNTON D. B., FOULK W. T., BUTT H. R. Studies of chronic idiopathic jaundice (Dubin-Johnson syndrome). I. Demonstration of hepatic excretory defect. Gastroenterology. 1963 Feb;44:101–111. [PubMed] [Google Scholar]
- SPRINZ H., NELSON R. S. Persistent non-hemolytic hyperbilirubinemia associated with lipochrome-like pigment in liver cells: report of four cases. Ann Intern Med. 1954 Nov;41(5):952–962. doi: 10.7326/0003-4819-41-5-952. [DOI] [PubMed] [Google Scholar]
- Seligsohn U., Shani M., Ramot B., Adam A., Sheba C. Dubin-Johnson syndrome in Israel. II. Association with factor-VII deficiency. Q J Med. 1970 Oct;39(156):569–584. [PubMed] [Google Scholar]
- Shani M., Seligsohn U., Adam A. The inheritance of Dubin-Johnson syndrome. Isr J Med Sci. 1973 Sep-Oct;9(9):1427–1430. [PubMed] [Google Scholar]
- Shani M., Seligsohn U., Gilon E., Sheba C., Adam A. Dubin-Johnson syndrome in Israel. I. Clinical, laboratory, and genetic aspects of 101 cases. Q J Med. 1970 Oct;39(156):549–567. [PubMed] [Google Scholar]
- Sveger T. Liver disease in alpha1-antitrypsin deficiency detected by screening of 200,000 infants. N Engl J Med. 1976 Jun 10;294(24):1316–1321. doi: 10.1056/NEJM197606102942404. [DOI] [PubMed] [Google Scholar]
- Talbot I. C., Mowat A. P. Liver disease in infancy: histological features and relationship to alpha-antitrypsin phenotype. J Clin Pathol. 1975 Jul;28(7):559–563. doi: 10.1136/jcp.28.7.559. [DOI] [PMC free article] [PubMed] [Google Scholar]
- WHEELER H. O., MELTZER J. I., BRADLEY S. E. Biliary transport and hepatic storage of sulfobromophthalein sodium in the unanesthetized dog, in normal man, and in patients with hepatic disease. J Clin Invest. 1960 Jul;39:1131–1144. doi: 10.1172/JCI104128. [DOI] [PMC free article] [PubMed] [Google Scholar]
- WOLF R. L., PIZETTE M., RICHMAN A., DREILING D. A., JACOBS W., FERNANDEZ O., POPPER H. Chronic idiopathic jaundice. A study of two afflicted families. Am J Med. 1960 Jan;28:32–41. doi: 10.1016/0002-9343(60)90220-5. [DOI] [PubMed] [Google Scholar]

