Skip to main content
Log in

Constitutional hyperbilirubinemia with unconjugated bilirubin in the serum and pigment deposition in the liver

Report of a case

  • Case Reports
  • Published:
The American Journal of Digestive Diseases Aims and scope Submit manuscript

Summary

A case of constitutional hyperbilirubinemia with mild elevations of un-conjugated bilirubin in the serum was reported which was further characterized by an accumulation of pigment granules in the liver cells of the Dubin-Johnson-Sprinz-Nelson type. Electron-microscopic studies demonstrated predominantly lipofuscin-like pigments in the liver cells. The pathogenesis of this condition and the chemical nature of these pigments were briefly discussed. Attention was called to a variant of the Gilbert-Meulengracht syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  1. Gilbert, A., Castaigne, J., andLereboullet, P. De ictere familial: Contribution à l'étude de la diathre biliaire.Bull. Soc. mid hop. Paris 17:948, 1900.

    Google Scholar 

  2. Meulengracht, E. Icterus Intermittens Juvenilis.Klin. Wchnschr. 18:118, 1939.

    Google Scholar 

  3. Dubin, I. N., andJohnson, F. B. Chronic idiopathic jaundice with unidentified pigment in liver cells. A new clinicopathologic entity with a report of 12 cases.Medicine 33: 155, 1954.

    Google Scholar 

  4. Sprinz, H., andNelson, R. S. Persistent non-hemolytic hyperbilirubinemia associated with lipochrome pigment in liver cells: Report of four cases.Ann. Int. Med. 41: 952, 1954.

    Google Scholar 

  5. Dubin, I. N. Chronic idiopathic jaundice: A review of fifty cases.Am. J. Med. 24:268, 1958.

    Google Scholar 

  6. Rotor, A. B., Manahan, L., andFlorentin, A. Familial non-hemolytic jaundice with direct Van den Bergh reaction.Acta Med. phillipp. 5:37, 1948.

    Google Scholar 

  7. Arias, I. M. Progress in Liver Diseases. Grune, New York. 1961.

    Google Scholar 

  8. Foulk, W. T., Butt, H. R., Aven, C. A., Whitcomb, F. F., andMason, H. L. Constitutional hepatic dysfunction (Gilbert's disease), its natural history and related syndromes.Medicine 38:25, 1959.

    Google Scholar 

  9. Arias, I. M. Gilbert's disease.Bull. New York Acad. Med. 35:450, 1959.

    Google Scholar 

  10. Schmid, R., andHammaker, M. Glucuronide formation in patients with constitutional hepatic dysfunction (Gilbert's disease).New England J. Med. 260: 1310, 1959.

    Google Scholar 

  11. Arias, I. M. Recent advances in the metabolism of bilirubin and their clinical implications.M. clin. North America 44: 607, 1960.

    Google Scholar 

  12. Israel, L., Suderman, H., andRitzman, S. Hyperbilirubinemia due to an alternate path of bilirubin production.Am. J. Med., 27:693. 1959.

    Google Scholar 

  13. Sagild, U., Dalgaard, O. Z., andTygstrup, N. Constitutional hyperbilirubinemia with unconjugated bilirubin in the serum and lipochrome pigment granules in the liver.Ann. Int. Med. 56:308, 1962.

    Google Scholar 

  14. Post, J., Benton, J. G., andBreakstone, R. Observations on a cytoplasmic hepatic cell pigment in man.Arch. Path. 52:67, 1951.

    Google Scholar 

  15. Lehtinen, M., andFortelius, P. Three cases of chronic idiopathic jaundice. Clinical and histological observations.Acta med. scandinav. 171:561, 1962.

    Google Scholar 

  16. Essner, E., andNovikoff, A. Human hepatocellular pigments and lysosomes.J. Ultrastruct. Res. 3:374, 1960.

    Google Scholar 

  17. Novikoff, A., andEssner, E. The liver cell.Am. J. Med. 29:102, 1960.

    Google Scholar 

  18. Wellings, S. R., andSiegel, B. V. Electron microscopy of human malignant melanoma.J. Nat. Cancer Inst. 24:437, 1960.

    Google Scholar 

  19. Hogan, M. J., andFeeney, L. Ultrastructure of malignant melanoma of the choroid.Invest. Ophthal. 1:544, 1962.

    Google Scholar 

  20. Birbeck, M. S. C. Electron microscopy of melanocytes.Brit. M. Bull. 18:220, 1962.

    Google Scholar 

  21. Bollman, J. L. Bile Pigments of Serum in Disease of Liver, in Hepatitis Frontiers (Ed. by Hartman, F. W., Logrippo, G. A., Mateer, J. G., and Barron, J.), Little, Boston, 1957.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Additional information

The authors wish to express their appreciation to Irwin M. Arias, M.D., for his diazotization and paper chromatographic analysis of the patient's serum.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Herman, J.D., Cooper, E.B., Takeuchi, A. et al. Constitutional hyperbilirubinemia with unconjugated bilirubin in the serum and pigment deposition in the liver. Digest Dis Sci 9, 160–169 (1964). https://doi.org/10.1007/BF02243521

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF02243521

Keywords

Navigation