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.
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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.
Meulengracht, E. Icterus Intermittens Juvenilis.Klin. Wchnschr. 18:118, 1939.
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.
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.
Dubin, I. N. Chronic idiopathic jaundice: A review of fifty cases.Am. J. Med. 24:268, 1958.
Rotor, A. B., Manahan, L., andFlorentin, A. Familial non-hemolytic jaundice with direct Van den Bergh reaction.Acta Med. phillipp. 5:37, 1948.
Arias, I. M. Progress in Liver Diseases. Grune, New York. 1961.
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.
Arias, I. M. Gilbert's disease.Bull. New York Acad. Med. 35:450, 1959.
Schmid, R., andHammaker, M. Glucuronide formation in patients with constitutional hepatic dysfunction (Gilbert's disease).New England J. Med. 260: 1310, 1959.
Arias, I. M. Recent advances in the metabolism of bilirubin and their clinical implications.M. clin. North America 44: 607, 1960.
Israel, L., Suderman, H., andRitzman, S. Hyperbilirubinemia due to an alternate path of bilirubin production.Am. J. Med., 27:693. 1959.
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.
Post, J., Benton, J. G., andBreakstone, R. Observations on a cytoplasmic hepatic cell pigment in man.Arch. Path. 52:67, 1951.
Lehtinen, M., andFortelius, P. Three cases of chronic idiopathic jaundice. Clinical and histological observations.Acta med. scandinav. 171:561, 1962.
Essner, E., andNovikoff, A. Human hepatocellular pigments and lysosomes.J. Ultrastruct. Res. 3:374, 1960.
Novikoff, A., andEssner, E. The liver cell.Am. J. Med. 29:102, 1960.
Wellings, S. R., andSiegel, B. V. Electron microscopy of human malignant melanoma.J. Nat. Cancer Inst. 24:437, 1960.
Hogan, M. J., andFeeney, L. Ultrastructure of malignant melanoma of the choroid.Invest. Ophthal. 1:544, 1962.
Birbeck, M. S. C. Electron microscopy of melanocytes.Brit. M. Bull. 18:220, 1962.
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.
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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.
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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
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DOI: https://doi.org/10.1007/BF02243521