Semin Liver Dis 2010; 30(2): 205-209
DOI: 10.1055/s-0030-1253523
DIAGNOSTIC PROBLEMS IN HEPATOLOGY

© Thieme Medical Publishers

A Patient with Persistent Pruritus

Lawrence U. Liu1 , Lihui Qin2 , A.S. Knisely3
  • 1Division of Liver Diseases, The Mount Sinai Medical Center, New York, New York
  • 2Department of Pathology, The Mount Sinai Medical Center, New York, New York
  • 3Institute of Liver Studies/Histopathology, King's College Hospital, London, United Kingdom
Further Information

Publication History

Publication Date:
26 April 2010 (online)

ABSTRACT

We present a patient with an initial and acute presentation of jaundice and marked persistent pruritus. Laboratory and radiology test results eliminated the possibility of acute hepatitis A/B/C viral infections, primary biliary cirrhosis, autoimmune hepatitis, Wilson disease, paraneoplastic cholestasis, and obstructive biliary disease. Centrilobular cholestasis was prominent in a liver biopsy specimen. Benign recurrent intrahepatic cholestasis (BRIC) was diagnosed through a review of the clinical history, available data, and the subsequent exclusion of other possible etiologies. The patient's clinical features resolved within 3 months of medical treatment.

REFERENCES

  • 1 Luketic V A, Shiffman M L. Benign recurrent intrahepatic cholestasis.  Clin Liver Dis. 2004;  8(1) 133-149, vii
  • 2 Summerskill W HJ, Walshe J M. Benign recurrent intrahepatic “obstructive” jaundice.  Lancet. 1959;  2(7105) 686-690
  • 3 Brenard R, Geubel A P, Benhamou J P. Benign recurrent intrahepatic cholestasis. A report of 26 cases.  J Clin Gastroenterol. 1989;  11(5) 546-551
  • 4 Da Silva L C, de Brito T. Benign recurrent intrahepatic cholestasis in two brothers.  Ann Intern Med. 1966;  65 330-341
  • 5 Kuhn H A. Intrahepatic cholestasis in two brothers.  Ger Med Mon. 1963;  8 185-188
  • 6 Stathers G, Reed C SH, Hirst E. Idiopathic recurrent cholestasis.  Gastroenterology. 1967;  52(3) 536-543
  • 7 Tygstrup N. Intermittent possibly familial intrahepatic cholestatic jaundice.  Lancet. 1960;  1(7135) 1171-1172
  • 8 Schapiro R H, Isselbacher K J. Benign recurrent intrahepatic cholestasis.  N Engl J Med. 1963;  268 708-711
  • 9 De Koning T J, Sandkuijl L A, De Schryver J EAR, Hennekam E A, Beemer F A, Houwen R H. Autosomal-recessive inheritance of benign recurrent intrahepatic cholestasis.  Am J Med Genet. 1995;  57(3) 479-482
  • 10 Paulusma C C, Oude Elferink R PJ, Jansen P LM. Progressive familial intrahepatic cholestasis type 1.  Semin Liver Dis. 2010;  30(2) 115-122
  • 11 van Mil S W, van der Woerd W L, van der Brugge G et al.. Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11.  Gastroenterology. 2004;  127(2) 379-384
  • 12 Davit-Spraul A, Gonzales E, Baussan C, Jacquemin E. The spectrum of liver diseases related to ABCB4 gene mutations: pathophysiology and clinical aspects.  Semin Liver Dis. 2010;  30(2) 132-144
  • 13 de Pagter A GF, van Berge Henegouwen G P, ten Bokkel Huinink J A, Brandt K H. Familial benign recurrent intrahepatic cholestasis. Interrelation with intrahepatic cholestasis of pregnancy and from oral contraceptives?.  Gastroenterology. 1976;  71(2) 202-207
  • 14 Lesser P B. Benign familial recurrent intrahepatic cholestasis.  Am J Dig Dis. 1973;  18(4) 259-264
  • 15 Tang X, Halleck M S, Schlegel R A, Williamson P. A subfamily of P-type ATPases with aminophospholipid transporting activity.  Science. 1996;  272(5267) 1495-1497
  • 16 Biempica L, Gutstein S, Arias I M. Morphological and biochemical studies of benign recurrent cholestasis.  Gastroenterology. 1967;  52(3) 521-535
  • 17 Endo T, Uchida K, Amuro Y, Higashino K, Yamamura Y. Bile acid metabolism in benign recurrent intrahepatic cholestasis. Comparative studies on the icteric and anicteric phases of a single case.  Gastroenterology. 1979;  76(5 Pt 1) 1002-1006
  • 18 Spiegel E L, Schubert W, Perrin E, Schiff L. Benign recurrent intrahepatic cholestasis, with response to cholestyramine.  Am J Med. 1965;  39(4) 682-688
  • 19 van Berge Henegouwen G P, Brandt K-H, de Pagter A GF. Is an acute disturbance in hepatic transport of bile-acids the primary cause of cholestasis in benign recurrent intrahepatic cholestasis?.  Lancet. 1974;  1(7869) 1249-1251
  • 20 Freedman M R, Holzbach R T, Ferguson D R. Pruritus in cholestasis: no direct causative role for bile acid retention.  Am J Med. 1981;  70(5) 1011-1016
  • 21 Ghent C N, Bloomer J R, Klatskin G. Elevations in skin tissue levels of bile acids in human cholestasis: relation to serum levels and topruritus.  Gastroenterology. 1977;  73(5) 1125-1130
  • 22 Bijleveld C MA, Vonk R J, Kuipers F et al.. Benign recurrent intrahepatic cholestasis: altered bile acid metabolism.  Gastroenterology. 1989;  97(2) 427-432
  • 23 Tygstrup N, Jensen B. Intermittent intrahepatic cholestasis of unknown etiology in five young males from the Faroe Islands.  Acta Med Scand. 1969;  185(6) 523-530
  • 24 Davit-Spraul A, Gonzales E, Baussan C, Jacquemin E. Progressive familial intrahepatic cholestasis.  Orphanet J Rare Dis. 2009;  4 1-12
  • 25 Strautnieks S S, Byrne J A, Pawlikowska L et al.. Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families.  Gastroenterology. 2008;  134(4) 1203-1214
  • 26 Paulusma C C, Groen A, Kunne C et al.. Atp8b1 deficiency in mice reduces resistance of the canalicular membrane to hydrophobic bile salts and impairs bile salt transport.  Hepatology. 2006;  44(1) 195-204
  • 27 Carlton V EH, Knisely A S, Freimer N B. Mapping of a locus for progressive familial intrahepatic cholestasis (Byler disease) to 18q21-q22, the benign recurrent intrahepatic cholestasis region.  Hum Mol Genet. 1995;  4(6) 1049-1053
  • 28 Oude Elferink R PJ, van Berge Henegouwen G P. Cracking the genetic code for benign recurrent and progressive familial intrahepatic cholestasis.  J Hepatol. 1998;  29(2) 317-320
  • 29 Shiffman M L, Heuman D H. Pathophysiology and pharmacology of cholestasis. In: McCallum RW, Friedman G, Jacobson ED Gastrointestinal Pharmacology and Therapeutics. New York; Raven Press 1996: 401-416
  • 30 Summerskill W HJ. The syndrome of benign recurrent cholestasis.  Am J Med. 1965;  38 298-305
  • 31 Williams R, Cartter M A, Sherlock S, Scheuer P J, Hill K R. Idiopathic recurrent cholestasis: a study of the functional and pathological lesions in four cases.  Q J Med. 1964;  33 387-399
  • 32 Stapelbroek J M, van Erpecum K J, Klomp L WJ et al.. Nasobiliary drainage induces long-lasting remission in benign recurrent intrahepatic cholestasis.  Hepatology. 2006;  43(1) 51-53

Lawrence U LiuM.D. 

Division of Liver Diseases, The Mount Sinai Medical Center

One Gustave L. Levy Place, Box 1104, New York, NY 10029

Email: Lawrence.Liu@mountsinai.org

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