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Biliary lipid metabolism in children with chronic intrahepatic cholestasis

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Abstract

Biliary lipid composition, standard liver function tests, serum lipids and faecal fat excretion were studied in 15 children with chronic intrahepatic cholestasis (severe intrahepatic cholestasis, n=6; paucity of intralobular bile ducts, n=4; benign recurrent cholestasis, n=5) and compared to 15 children without gastrointestinal diseases. Severe and benign intrahepatic cholestasis were associated with normal or moderately elevated serum lipids. Biliary lipid concentrations were extremely reduced, bile acid concentrations were below the critical micellar concentration. This may account for the high incidence of gallstone formation in these patients. Remission periods in patients with benign recurrent cholestasis were not followed by complete normalisation of biliary lipid concentrations, indicating a primary defect in hepatic excretory function. Children with paucity of intralobular bile ducts showed markedly increased serum lipids, but only a two-fold reduction in biliary lipid concentrations. Cholic acid was the predominant bile acid in bile of all cholestatic children even during remission. Neither increased levels of monohydroxy bile acids nor unusual bile acids could be identified in notable amounts.

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References

  1. Alagille D, Odievre M, Gautier M, Dommergues JP (1975) Hepatic ductular hypoplasia associated with characteristic facies, vertebral malformations, retarded physical, mental and sexual development and cardiac murmur. J Pediatr 86:63–71

    Google Scholar 

  2. Alagille D, Odievre M (1979) Liver and biliary tract disease in children. John Wiley and Sons, New York

    Google Scholar 

  3. Bartlett GR (1959) Phosphorus assay in column chromatography. J Biol Chem 234:466–468

    Google Scholar 

  4. Beaudoin M, Feldmann G, Erlinger S, Benhamou J-P (1973) Benign recurrent cholestasis. Digestion 9:49–65

    Google Scholar 

  5. Becker M, Ruoff H-J, Rotthauwe HW (1980) Cyclic AMP in pancreatic and biliary secretion of children with chronic intrahepatic cholestasis and cystic fibrosis. Eur J Pediatr 134:217–225

    Google Scholar 

  6. Becker M, von Bergmann K, Rotthauwe HW, Leiss O (1984) Effects of phenobarbital on biliary lipid metabolism in children with chronic intrahepatic cholestasis. Eur J Pediatr 143:41–44

    Google Scholar 

  7. Collins DM, Shannon FT, Campbell CB (1981) Bile acid metabolism in mild arteriohepatic dysplasia. Aust NZ J Med 11:48–51

    Google Scholar 

  8. Day RC, Harry DS, Owen JS, Foo AY, McIntyre N (1979) Lecithin-cholesterol acyltransferase and the lipoprotein abnormalities of parenchymal liver disease. Clinical Science 56:575–583

    Google Scholar 

  9. Deleze G, Paumgartner G (1977) Bile acids in serum and bile of infants with cholestatic syndromes. Helv Paediatr Acta 32:29–38

    Google Scholar 

  10. Einarsson K, Hellstrom K, Schersten T (1975) The formation of bile acids in patients with liver cirrhosis. Scand J Gastroenterol 10:299–304

    Google Scholar 

  11. Endo T, Uchida K, Amuro Y, Higashino K, Yamamura Y (1979) Bile acid metabolism in benign recurrent intrahepatic cholestasis. Gastroenterology 76:1002–1006

    Google Scholar 

  12. Eyssen H, Parmentier G, Compernolle F, Boon J, Eggermont E (1972) Trihydroxycoprostanic acid in the duodenal fluid of two children with intrahepatic bile duct abnormalities. Biochim Biophys Acta 273:212–221

    Google Scholar 

  13. Grundy SM, Metzger AL (1972) A physiological method for estimation of hepatic secretion of biliary lipids in man. Gastroenterology 62:1200–1217

    Google Scholar 

  14. Grundy SM, Ahrens EH jr, Miettinen TA (1965) Quantitative isolation and gas-liquid chromatographic analysis of total fecal bile acids. J Lipid Res 6:397–410

    Google Scholar 

  15. Javitt NB (1975) Current status of cholestasis induced by monohydroxy bile acids. In: Goresky CA, Fisher MM (eds) Jaundice. Plenum Press, New York, pp 401–409

    Google Scholar 

  16. Javitt NB, Morrissey KP, Siegel E, Goldberg H, Gartner LM, Hollander M, Kok E (1973) Cholestatic syndromes in infancy: diagnostic value of serum bile acid pattern and cholestyramine administration. Pediatr Res 7:110–125

    Google Scholar 

  17. Kesaniemi YA, Salaspuro MP, Vuoristo M, Miettinen TA (1982) Biliary lipid secretion in chronic cholestatic liver disease. Gut 23:931–938

    Google Scholar 

  18. Linarelli LG, Williams CN, Phillips MJ (1972) Byler's disease: fatal intrahepatic cholestasis. J Pediatr 81:484–492

    Google Scholar 

  19. Linarelli LG, Hengstenberg FH, Drash AL (1973) Effect of phenobarbital on hyperlipemia in patients with intrahepatic and extrahepatic cholestasis. J Pediatr 83:291–299

    Google Scholar 

  20. Mashige F, Imai K, Osuga T (1976) A simple and sensitive assay of total serum bile acids. Clin Chim Acta 70:79–86

    Google Scholar 

  21. McIntyre N, Calandra S, Pearson AJG (1974) Lipid and lipo-protein abnormalities in liver disease: the possible role of lecithin: cholesterol acyltransferase deficiency. Scand J Clin Lab Invest [Suppl 33] 137:115–120

    Google Scholar 

  22. McIntyre N, Harry DS, Pearson AJG (1975) The hypercholesterolaemia of obstructive jaundice. Gut 16:379–391

    Google Scholar 

  23. Miettinen TA, Siurula M (1971) Micellar solubilization of intestinal lipids and sterols in gluten enteropathy and liver cirrhosis. Scand J Gastroenterol 6:527–535

    Google Scholar 

  24. Mok HYI, von Bergmann K, Grundy SM (1978) Effects of interruption of enterohepatic circulation on biliary lipid secretion in man. Dig Dis Sci 28:1067–1075

    Google Scholar 

  25. Nair PP, Gordon M, Reback J (1967) The enzymatic cleavage of the carbonhydrogen bound in 3-alpha, 7-alpha, 12-alpha-trihydroxy-5-beta-cholan-25-olylglycine. J Biol Chem 242:7–11

    Google Scholar 

  26. Nilsson S, Schersten LS (1969) Importance of bile acids for phospholipid secretion into human hepatic bile. Gastroenterology 57:525–532

    Google Scholar 

  27. Odievre M, Gautier M, Hadchouel M, Alagille D (1973) Severe familial intrahepatic cholestasis. Arch Dis Child 48:806–812

    Google Scholar 

  28. Paumgartner G, Horak W, Probst P, Grabner G (1971) Effect of phenobarbital on bile flow and bile salt excretion in the rat. Naunyn Schmiedeberg's Arch Pharmacol 270:98–102

    Google Scholar 

  29. Poley JR (1975) Fat digestion and absorption in lipase and bile acid deficiency. In: Rommel K, Boehmer R (eds) Lipid absorption: biochemical and clinical aspects. MTP Press Ltd, Lancester, pp 151–199

    Google Scholar 

  30. Quarfordt SH, Jakoi L (1973) Alterations of hepatic cholesterol synthesis in rats by lecithin and lecithin cholesterol mesophase infusions (abstract). Gastroenterology 65:566

    Google Scholar 

  31. Rotthauwe HW, Becker M, Bosch Ch (1981) Progressive intrahepatische Cholestase. Monatsschr Kinderheilkd 129:515–520

    Google Scholar 

  32. Sabesin SM (1982) Cholestatic lipoproteins—their pathogenesis and significance. Gastroenterology 83:704–709

    Google Scholar 

  33. Sachs L (1974) Angewandte Statistik. Springer, Heidelberg New York

    Google Scholar 

  34. Salen G, Nicolau G, Shefer S, Mosbach EH (1975) Hepatic cholesterol metabolism in patients with gallstones. Gastroenterology 69:676–684

    Google Scholar 

  35. Schlenk H, Gellermann JL (1960) Esterification of fatty acids with diazomethane on a small scale. Anal Chem 32:1412–1414

    Google Scholar 

  36. Schwartz CC, Almond HR, Vlahcevic ZR, Swell L (1979) Bile acid metabolism in cirrhosis. V. Determination of biliary lipid secretion rates in patients with advanced cirrhosis. Gastroenterology 77:1177–1182

    Google Scholar 

  37. Stark H (1968) Benign recurrent cholestasis in a child. Pediatrics 41:636–639

    Google Scholar 

  38. Stiehl A, Thaler MM, Admirand WH (1972) The effects of phenobarbital on bile salts and bilirubin in patients with intrahepatic and extrahepatic cholestasis. New Engl J Med 286:858–861

    Google Scholar 

  39. Summerfield JA, Scott J, Berman M, Ghent C, Bloomer JR, Berk PD, Sherlock S (1980) Benign recurrent intrahepatic cholestasis: studies of bilirubin kinetics, bile acids and cholangiography. Gut 21:154–160

    Google Scholar 

  40. Switzer S (1967) Plasma lipoproteins in liver disease. I. Immunologically distinct low-density lipoproteins in patients with biliary obstruction. J Clin Invest 46:1855–1866

    Google Scholar 

  41. Talalay P (1960) Enzymatic analysis of steroid hormones. Methods Biochem Anal 8:114–143

    Google Scholar 

  42. Van Berge Henegouwen GP, Allan RN, Hofmann AF, Yu PYS (1977) A facile hydrolysis-solvolysis procedure for conjugated bile acid sulfates. J Lipid Res 18:118–122

    Google Scholar 

  43. Van Berge Henegouwen GP, Ferguson DR, Hofmann AF, de Pagter AGF (1978) Familial and non-familial benign recurrent cholestasis distinguished by plasma disappearance of indocyanine green but not cholylglycine. Gut 19:345–350

    Google Scholar 

  44. Van Berge Henegouwen GP, Brandt K-H, Ruben AT, Schalm SW, Taal B (1982) High frequency of lithogenic bile in primary biliary cirrhosis. Neth J Med 25:89–93

    Google Scholar 

  45. Van de Kamer JH, ten Bokkel Huinink H, Weijers HA (1949) A rapid method for the determination of fat in faeces. J Biol Chem 177:347–355

    Google Scholar 

  46. Vlahcevic YR, Bell CC, Juttijudata P, Swell L (1972) Bile-rich duodenal fluid as an indicator of biliary lipid composition and its applicability to detection of lithogenic bile. Am J Dig Dis 9:797–802

    Google Scholar 

  47. Vlahcevic YR, Yoshida T, Juttijudata P, Bell CC, Swell L (1973) Bile acid metabolism in cirrhosis. III. Biliary lipid secretion in patients with cirrhosis and its relevance to gallstone formation. Gastroenterology 64:298–303

    Google Scholar 

  48. Watson GH, Miller B (1973) Arteriohepatic dysplasia: familial pulmonary arterial stenosis with neonatal liver disease. Arch Dis Child 48:459–466

    Google Scholar 

  49. Weber A, Roy C (1972) The malabsorption associated with chronic liver disease in children. Pediatrics 50:73–83

    Google Scholar 

  50. Williams CN, Kaye R, Baker L, Hurwitz R, Senior JR (1972) Progressive familial cholestatic cirrhosis and bile acid metabolism. J Pediatr 81:493–500

    Google Scholar 

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Authors and Affiliations

  1. Department of Pediatrics, University of Bonn, D-5300, Bonn, Federal Republic of Germany

    M. Becker & H. W. Rotthauwe

  2. Department of Medicine, University of Bonn, D-5300, Bonn, Federal Republic of Germany

    K. von Bergmann & O. Leiss

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  1. M. Becker
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  2. K. von Bergmann
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  3. H. W. Rotthauwe
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  4. O. Leiss
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Becker, M., von Bergmann, K., Rotthauwe, H.W. et al. Biliary lipid metabolism in children with chronic intrahepatic cholestasis. Eur J Pediatr 143, 35–40 (1984). https://doi.org/10.1007/BF00442745

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  • DOI: https://doi.org/10.1007/BF00442745

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