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Hepatic transport and metabolism of various organic anions in patients with congentital non-hemolytic hyperbilirubinemia, including constitutional indocyanine green excretory defect

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References

  1. Rotor AB, Manahan L, Florentin A. Familial non-hemolytic jaundice with direct van den Bergh reaction. Acta Med Philip 1948;5:37–49.

    Google Scholar 

  2. Porush JG, Delman AJ, Feuer MM. Chronic idiopathic jaundice with normal liver histology. Arch Intern Med 1962;109:302–309.

    PubMed  CAS  Google Scholar 

  3. Dubin IN, Johnson FB. Chronic idiopathic jaundice with unidentified pigment in liver cells; new clinicopathologic entity with report of 12 cases. Medicine 1954;33:155–197.

    PubMed  CAS  Google Scholar 

  4. Malloy HT, Evelyn KA. The determination of bilirbin with photoelectric colorimeter. J Biol Chem 1937;119:481–490.

    CAS  Google Scholar 

  5. Bailey A, Robinson D, Dawson AM. Does Gilbert's disease exist? Lancet 1977;30:931–933.

    Google Scholar 

  6. Portman OW, Chowdhury JR, Chowdhury NR, et al. A nonhuman primate model of Gilbert's syndrome. Hepatology 1984; 4:175–179.

    PubMed  CAS  Google Scholar 

  7. Cornelius CE, Osbun BI, Gronwall RR, et al. Dubin-Johnson syndrome in immature sheep. Am J Dig Dis 1968;13:1072–1076.

    Article  PubMed  CAS  Google Scholar 

  8. Masuda M, Takino T, Ogasawara K, et al. Clinical study of constitutional hyperbilirubinemia (in Japanese). J Intern Med 1963;11:687–696.

    Google Scholar 

  9. Namihisa T, Yamaguchi K. Constitutional hyperbilirubinemia in Japan. Studies of the 139 cases reported during the period from 1963 to 1969 (in Japanese). J Jpn Soc Intern Med 1971;60: 518–527.

    CAS  Google Scholar 

  10. Takino T, Takahashi T, Okuno T. Clinical study of constitutional hyperbilirubinemia in Japan. A nationwide survey between 1970 and 1974 (in Japanese). Jpn J Gastroenterol 1977;74:1518–1528.

    CAS  Google Scholar 

  11. Nambu M, Ueda C, Yoshimura K, et al. A new type of constitutional hyperbilirubinemia with unconjugated bilirubin: A case of non-hemolytic hyperbilirubinemia with marked retention of sulfobromophthalein test (in Japanese). Acta Hepatol Jpn 1980;21:1690–1697.

    Google Scholar 

  12. Seligsohn U, Shani M. The Dubin-Johnson syndrome and pregnancy. Acta Hepato-Gastroenterol 1977;24:167–169.

    CAS  Google Scholar 

  13. Bradley EM, Bellamy HM, Knudsen KB, et al. Impairment of biliary transport as a cause of sulfobromophthalein retention in fasting men. Metabolism 1969;18:675–683.

    Article  PubMed  CAS  Google Scholar 

  14. Takigami T, Okada K, Namihisa T, et al. Rotor's syndrome and Dubin-Johnson syndrome. Co-existence in a small village, especially in a certain family (in Japanese). Acta Hepatol Jpn 1976;17:589–598.

    Google Scholar 

  15. Blashke TF, Berk PD, Scharschmidt BF. Crigler-Najjar syndrome: An unusual course with development of neurologic damage at age eighteen. Pediatr Res 1974;8:573–590.

    Google Scholar 

  16. Matsuki S, Shibata H, Kokubo S, et al. A comparative study of two cases of adult Crigler-Najjar syndrome (in Japanese). Acta Hepatol Jpn 1986;27:1622–1627.

    Google Scholar 

  17. Yamaguchi T, Yamaguchi N, Nakajima H, et al. Studies on bilirubin metabolism. III. Separation and determination of bilirubin IX conjugates from biological specimens by means of high-performance liquid chromatography. Demonstration of a new bilirubin conjugate [C] and its biological significance. Proc Jpn Acad 1979;55(B):456–459.

    CAS  Google Scholar 

  18. Fevery J, Blanckaert N, Leroy P, et al. Analysis of bilirubins in biological fluids by extraction and thin layer chromatography of the intact tetrapyrroles: Application to bile of patients with Gilbert's syndrome, hemolysis, or cholelithiasis. Hepatology 1983;3:177–183.

    PubMed  CAS  Google Scholar 

  19. Inagaki T, Ohta S, Takigawa H, et al. Analysis of bilirubin in bile using HPLC (in Japanese). Acta Hepatol Jpn 1980;4:492.

    Google Scholar 

  20. Kondo T, Kuchiba K, Ohtsuka Y, et al. Clinical and genetic studies on Dubin-Johnson syndrome in a cluster area in Japan. Jpn J Human Genet 1974;18:378–392.

    Google Scholar 

  21. Shani M, Seligsohn U, Gilon E, et al. Dubin-Johnson syndrome in Israel. I. Clinical, laboratory, and genetic aspects of 101 cases. Q J Med New Series 1970;39:549–567.

    CAS  Google Scholar 

  22. Owens D, Sherlock S. The diagnosis of Gilbert's syndrome. Role of the reduced caloric intake test. Br Med J 1973;III: 559–563.

    Google Scholar 

  23. Bloomer JR, Barrett PV, Rodkey FL, et al. Studies on the mechanism of fasting hyperbilirubinemia. Gastroenterology 1971;61:479–487.

    PubMed  CAS  Google Scholar 

  24. Felsher BF, Carpio NM. Caloric intake and unconjugated hyperbilirubinemia. Gastroenterology 1975;69:42–47.

    PubMed  CAS  Google Scholar 

  25. Ohkubo H, Musa H, Okuda K. Effect of caloric restriction on the kinetics of indocyanine green in patients with liver diseases and in the rat. Dig Dis 1978;23:1017–1024.

    CAS  Google Scholar 

  26. Portman OW, Alexander M, Cornelius CE, et al. The effects of nutrition on unconjugated plasma bilirubin concentrations in squirrel monkeys. Hepatology 1984;4:454–460.

    PubMed  CAS  Google Scholar 

  27. Schiff L, Billing BH, Oikawa Y. Familial non-hemolytic jaundice with conjugated bilirubin in the serum. A case study. N Engl J Med 1959;260:1315–1318.

    PubMed  CAS  Google Scholar 

  28. Wolf RL, Pizette M, Richman A, et al. Chronic idiopathic jaundice. A study of two afficted families. Am J Med 1960;28: 32–41.

    Article  PubMed  CAS  Google Scholar 

  29. Arias IM. 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; 31:510–518.

    Article  PubMed  CAS  Google Scholar 

  30. Mandema E, Fraiture H, Nieweg HO, et al. Familial chronic idiopathic jaundice (Durbin-Sprinz disease), with a note on bromsulphalein metabolism in this disease. Am J Med 1960;28: 42–50.

    Article  PubMed  CAS  Google Scholar 

  31. Rodes J, Zubizarreta A, Bruguer M. Metabolism of bromsulphalein in Dubin-Johnson syndrome. Dig Dis 1972;17:545–552.

    CAS  Google Scholar 

  32. Namihisa T, Nambu M, Kobayashi N, et al. Hepatic dye transport in constitutional hyperbilirubinemia and constitutional ICG excretory defect (in Japanese). Acta Hepatol Jpn 1975;16: 25–35.

    Google Scholar 

  33. Schoenfield LJ, McGill DB, Hunton DB. Studies of chronic idiopathic jaundice (Dubin-Johnson syndrome) I. Determination of hepatic excretory defect. Gastroenterology 1963;44: 101–110.

    PubMed  CAS  Google Scholar 

  34. Erlinger S, Dhumeaux D, Desjeux JF, et al. Hepatic handling of unconjugated dyes in the Dubin-Johnson syndrome. Gastroenterology 1973;64:106–119.

    PubMed  CAS  Google Scholar 

  35. Okumura H, Satomura K, Yano T, et al. Dubin-Johnson syndrome with the secondary rise of BSP in serum (in Japanese). Acta Hepatol Jpn 1973;14:470–472.

    Google Scholar 

  36. Caesar J, Shaldon L, Chiandussi L, et al. The use of indocyanine green in the measurement of hepatic blood flow and as a test of hepatic function. Clin Sci 1961;21:43–57.

    PubMed  CAS  Google Scholar 

  37. Nambu M. Hepatic clearance of indocyanine green in liver disease (in Japanese). Jpn J Gastroenterol 1966;63:777–793.

    Google Scholar 

  38. Namihisa T, Nambu M, Yamaguchi K, et al. Chronic nonhemolytic jaundice. Dubin-Johnson syndrome, and Rotor's disease, with special reference to dye transport and metabolism (in Japanese). Acta Hepatol Jpn 1969;10:19–28.

    Google Scholar 

  39. Namhisa T, Yamaguchi K, Nambu M. Constitutional conjugated hyperbilirubinemia (Dubin-Johnson syndrome and Rotor's type of hyperbilirubinemia). New definitions bases on studies of transport with indocyanine green and bromsulfophthalein. Gastroenterol Jpn 1973;8:217–223.

    Google Scholar 

  40. Abe H, Okuda K. Biliary excretion of conjugated sulfobromophthalein (BSP) in constitutional conjugated hyperbilirubinemias. Digestion 1975;13:272–283.

    PubMed  CAS  Google Scholar 

  41. Barber-Riley G, Goetzee AE, Richards TG, et al. The transfer of bromsulphthalein from the plasma to the bile in man. Clin Sci 1961;20:149–159.

    PubMed  CAS  Google Scholar 

  42. Wheeler HO, Meltzer JI, Bradley SE. 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;39:1131–1141.

    PubMed  CAS  Google Scholar 

  43. Kanai T. Compartment analysis of transport and tolerance test of indocyanine green in normal subjects and in patients with hepatic dysfunction (in Japanese). Jpn J Gastroenterol 1972;69: 228–243.

    CAS  Google Scholar 

  44. Dhumeaux D, Berthelot P. Chronic hyperbilirubinemia associated with hepatic uptake and storage impairment. A new syndrome resembling that of the mutant Southdown sheep. Gastroenterology 1975;69:988–993.

    PubMed  CAS  Google Scholar 

  45. Namihisa T. Nambu M. Rotor's and Dubin-Johnson syndrome (letter). N Engl J Med 1977;297:560.

    PubMed  CAS  Google Scholar 

  46. Berk PD, Bloomer JR, Home RB, et al. Constitutional hepatic dysfunction (Gilbert's syndrome). A new definition based on kinetic studies with unconjugated radio-bilirubin. Am J Med 1970;49:296–305.

    PubMed  CAS  Google Scholar 

  47. Gentile S, Persico M, Tiribelli C. Abnormal hepatic uptake of low doses of sulfobromophthalein in Gilbert's syndrome: The role of reduced affinity of the plasma membrane carrier of organic anions. Hepatology 1990;12:213–217.

    PubMed  CAS  Google Scholar 

  48. Levi AJ, Gatmaitan Z, Arias IM. Two hepatic cytoplasmic protein fractions, Y and Z, and their possible role in the hepatic uptake of bilirubin, sulfobromophthalein, and other anions. J Clin Invest 1969;48:2156–2167.

    PubMed  CAS  Google Scholar 

  49. Namihisa T, Nambu M, Yamashiro Y. Sufobromophthalein and indocyanine green binding by the hepatic cytoplasmic proteins. Gastroenterol Jpn 1977;12:466–468.

    PubMed  CAS  Google Scholar 

  50. Fleischner G, Kamisaka K, Habig W, et al. Human ligandin: Characterization and quantitation (abstract). Gastroenterology 1975;69:821.

    Google Scholar 

  51. Tsuru T, Kamisaka K, Hirano M, et al. Quantification of human serum ligandin by radioimmunoassay (in Japanese). Acta Hepatol Jpn 1977;18:919–924.

    CAS  Google Scholar 

  52. Arias IM. Chronic unconjugated hyperbilirubinemia without overt signs of hemolysis in adolescents and adults. J Clin Invest 1962;41:2233–2245.

    PubMed  CAS  Google Scholar 

  53. Felsher BF, Craig JR, Carpio N. Hepatic bilirubin glucuronidation in Gilbert's syndrome. J Lab Clin Med 1973;81:829–837.

    PubMed  CAS  Google Scholar 

  54. Black M, Billing BH. Hepatic bilirubin UDP-glucuronyl transferase activity in liver disease and Gilbert's syndrome. N Engl J Med 1969;280:1266–1271.

    PubMed  CAS  Google Scholar 

  55. Bernstein LH, Ben-Ezzer J, Gartner L, et al. Hepatic intracellular distribution of tritium-labeled unconjugated and conjugated bilirubin in normal and Gunn rats. J Clin Invest 1966; 45:1194–1201.

    PubMed  CAS  Google Scholar 

  56. Muraca M, Fevery J, Blanckaert N. Relationships between serum bilirubins and production and conjugation of bilirubin. Studies in Gilbert's syndrome, Crigler-Najjar disease, hemolytic disorder, and rat models. Gastroenterology 1987;92:309–317.

    PubMed  CAS  Google Scholar 

  57. Koskelo P, Toivonen L, Adlercreuys H. Urinary coproporphyrin isomer distribution in the Dubin-Johnson syndrome. Clin Chem 1967;13:1006–1009.

    PubMed  CAS  Google Scholar 

  58. Wolkoff AW, Wolpert E, Pascasio FM, et al. Rotor's syndrome: A distinct inheritable pathophysiologic entity (abstract). Gastroenterology 1974;67:838.

    Google Scholar 

  59. Evans J, Lefkowitch J, Lim CK, et al. Fecal porphyrin abnormalities in a patient with features of Rotor's syndrome. Gastroenterology 1981;81:1125–1130.

    PubMed  CAS  Google Scholar 

  60. Krebs J, Brauer RW. Metabolism of sulfobromophthalein sodium (BSP) in the rat. Am J Physiol 1958;194:37–43.

    PubMed  CAS  Google Scholar 

  61. Yamamuro W, Nakagawa K, Mizuyoshi H, et al. Studies on the metabolism of three BSP fractions in patients with constitutional jaundice (in Japanese). Acta Hepatol Jpn 1985;12: 1646–1653.

    Google Scholar 

  62. Okumura H, Satomura K, Yano T, et al. A case of Dubin-Johnson syndrome with BSP secondary rise in serum (in Japanese). Acta Hepatol Jpn 1973;14:470–472.

    Google Scholar 

  63. Yamamoto T, Kosaka K, Ichida F, et al. Studies of the effect of bucolome on jaundice of Gilbert's syndrome and Dubin-Johnson syndrome (in Japanese). Jpn Arch Intern Med 1974;21: 427–437.

    Google Scholar 

  64. Merdier C, Burke M, Shani M, et al. The effect of phenobarbital on patients with Dubin-Johnson syndrome. Digestion 1976;14:394–399.

    Google Scholar 

  65. Shani M, Seligsohn U, Ben-Ezzer J. Effect of phenobarbital on liver functions in patients with Dubin-Johnson syndrome. Gastroenterology 1974;67:303–308.

    PubMed  CAS  Google Scholar 

  66. Reyes H, Levi J, Gatnaitan Z, et al. Studies of Y and Z, two hepatic cytoplasmic organic anion binding proteins. Effect of drugs, chemicals, hormones, and cholestasis. J Clin Invest 1971; 50:2242–2252.

    PubMed  CAS  Google Scholar 

  67. Nambu M, Yoshida K, Komatsu I, et al. Serum bile acid levels in patients with constitutional hyperbilirubinemias and constitutional ICG excretory defect (in Japanese). Acta Hepatol Jpn 1978;19:770–778.

    CAS  Google Scholar 

  68. Roda A, Roda E, Sama C, et al. Serum primary bile acids in Gilbert's syndrome. Gastroenterology 1982;82:77–83.

    PubMed  CAS  Google Scholar 

  69. Bar-Meir S, Levy R, Halperin Z, et al. Impairment of bromosulfophthalein clearance by bile salt administration in normal volunteers and patients with Dubin-Johnson syndrome. J Med Sci 1982;18:211–214.

    CAS  Google Scholar 

  70. Ueda H, Asahara A, Hishimuma S, et al. Sequential hepatobiliary scintigraphy of patients with constitutional jaundices and ICG excretory defect disease with99mTc-HIDA and99mTc-PI (in Japanese). Acta Hepatol Jpn 1979;20:795–806.

    Google Scholar 

  71. Bar-Mair S, Baron T, Seligson U, et al.99mTc-HIDA cholescintigraphy in Dubin-Johnson and Rotor's syndromes. Radiology 1982;142:743–746.

    Google Scholar 

  72. Kawaguchi S, Iio M, Yamada H, et al. Comparative evaluation of99mTc-labeled new hepatobiliary scanning agents,99mTc-HIDA and99mTc-(Sn)-PI (especially in cases of constitutional jaundices) (in Japanese). Acta Hepatol Jpn 1978;12:1141–1152.

    Google Scholar 

  73. Namihisa T, Nambu M, Kobayashi N, et al. Hepatic transport of indocyanine green and bromsulphalein in two cases with discrepancy (in Japanese). Jpn J Gastroenterol 1973;70: 966–976.

    CAS  Google Scholar 

  74. Namihisa T, Nambu M, Kobayashi N, et al. Nine cases with marked retention of indocyanine green test and normal sulfobromophthalein test without abnormal liver histology: Constitutional indocyanine green excretory defect. Hepatogastroenteroly 1981;28:6–12.

    CAS  Google Scholar 

  75. Okuda K, Ohkubo H, Musa H, et al. Marked delay in indocyanine green plasma clearance with a near-normal bromosulphophthalein retention test: A constitutional abnormality? Gut 1976;17:588–594.

    PubMed  CAS  Google Scholar 

  76. Namihisa T, Nambu M, Kobayashi H, et al. A case with apparent discrepancy between ICG test and BSP test (constitutional ICG excretory defect). Characteristic change in plasma disappearance curve (in Japanese). Jpn J Gastroenterol 1974; 71:168–173.

    CAS  Google Scholar 

  77. Kitamura T, Alroy J, Gatmaitan Z, et al. Defective biliary excretion of epinephrine metabolites in mutant (TR−) rats: Relation to the pathogenesis of black liver in the Dubin-Johnson syndrome and Corriedale sheep with an analogous excretory defect. Hepatology 1992;15:1154–1159.

    PubMed  CAS  Google Scholar 

  78. Takikawa H, Sano N, Narita T, et al. Biliary excretion of bile acid conjugates in a hyperbilirubinemic mutant Sprague-Dawley rat. Hepatology 1991;14:352–360.

    Article  PubMed  CAS  Google Scholar 

  79. Abei M, Matsuzaki Y, Tanaka N, et al. Immunohistochemical investigation of hepatic ligandin in patients with congenital hyperbilirubinemia (rapid communication, in Japanese). Acta Hepatol Jpn 1989;30:1040.

    Google Scholar 

  80. Kondo T, Kuchiba K, Shimizu Y. Coproporphyrin isomers in Dubin-Johnson syndrome. Gastroenterology 1976;70:1117–1120.

    PubMed  CAS  Google Scholar 

  81. Gilbert A, Lereboullet P, Herscher M: Les trois cholémies congénitales. Bull Soc Med Hosp Paris 1907;24:1203.

    Google Scholar 

  82. Owens D, Scherlock S. Diagnosis of Gilbert's syndrome: Role of reduced caloric intake test. British Med J 1973;3:559–563.

    CAS  Google Scholar 

  83. Fleischner G, Kamisaka K, Habig W, et al. Human ligandin: Characterization and quantitation. Gastroenterology 1975;69: 821.

    Google Scholar 

  84. Sandberg DH, Sjövall J, Sjövall K, et al. Measurement of human serum bile acid by gas-liquid chromatography. J Lipid Res 1965;6:182–192.

    PubMed  CAS  Google Scholar 

  85. Nambu M, Yoshida K, Komatsu I, et al. Serum bile acid levels in patients with constitutional hyperbilirubinemia and constitutional ICG excretory defect (in Japanese). Acta Hepatol Jpn 1978;19:770–778.

    CAS  Google Scholar 

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  1. Department of Medicine, Urayasu Hospital of Juntendo University School of Medicine, Tomioka 2-1-1, 279, Urayasu, Chiba, Japan

    Masaji Nambu & Toshihiko Namihisa

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  1. Masaji Nambu
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Nambu, M., Namihisa, T. Hepatic transport and metabolism of various organic anions in patients with congentital non-hemolytic hyperbilirubinemia, including constitutional indocyanine green excretory defect. J Gastroenterol 29, 228–240 (1994). https://doi.org/10.1007/BF02358689

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