Role of biopsy in Benign recurrent intrahepatic cholestasis
Report of two rare cases
Keywords:
Benign recurrent intrahepatic cholestasis, canalicular cholestasis, gamma glutamyl transferase, jaundice
Abstract
Benign recurrent intrahepatic cholestasis (BRIC) is an inherited self limiting cholestatic disorder. It is very rare and less common than its severe form, Progressive familial intrahepatic cholestasis (PFIC). Due to impaired canalicular biliary excretion, patients develop recurrent episodes of jaundice and intense pruritus with characteristic symptom free intervals. Most of the cases have a benign course and are managed symptomatically. Histopathological examination of liver biopsy is essential in cases of recurrent intrahepatic cholestasis to confirm the diagnosis. Here we report two cases of biopsy confirmed Benign recurrent intrahepatic cholestasis for its rarity.References
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2) Summerskill WHJ. The syndrome of benign recurrent cholestasis. Am J Med 1965;38:298-305.
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4) Walensi M, Canbay A, Oliver Witzke, Guido Gerken, Alisan Kahraman. Long term therapy of a patient with Summerskill- Walshe-Tygstrup syndrome by applying Prometheus liver dialysis: A case report. Case Rep Gastroenterol. 2012;6(2):550-556.
5) Geethalakshmi S, Mageshukumar S. Benign recurrent intrahepatic cholestasis: A rare case report. Int J Sci Stud. 2014;2(7):222-224.
6) Kochhar R, Bhasker RV, Mittal PK, Malik AK, Mehta SK. Benign recurrent intrahepatic cholestasis associated with retinitis pigmentosa. Indian J Gastroenterol.1988;7(1):51-2.
7) Gao H, Lin S, LvX, Ma H, Wang X,et al. The identification of two new ABCB11 mutations and the treatment of outcome in a young adult with Benign recurrent intrahepatic cholestasis: A case report. Hepat Mon. 2017; 17(10):e55087.
8) Niaz MK, Ahmed DS, Jennet SNE, Masum QAA et al. A rare case report on Benign recurrent intrahepatic cholestasis. Faridpur Med.Coll.J.2014; 9(2):108-110.
9) De Pagter AG, Van Berge Henegouwen GP, Ten Bokkel Huinink JA, Brandt KH. Familial benign recurrent cholestasis. Interrelation with intrahepatic cholestasis of pregnancy and from oral contraceptives? Gastroenterology. 1976;71:202-207.
10) Putterman C, Keidar S, Brook JG. Benign recurrent intrahepatic cholestasis- 25 years of follow-up. Postgraduate medical journal.1987;63:295-296.
11) Luketic VA, Schiffman ML. Benign recurrent intrahepatic cholestasis. Clin Liver Dis 1999;3:5509-28.
12) Van Ooteghem NA, Klomp LW, van Berge-Henegouwen GP, Houwen RH Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum. J Hepatol 2002;36:439-43.
13) Chhetri D, Gupta R, Duseja A, Dhiman RK, Chawla Y, Das A. Benign recurrent intrahepatic cholestasis in an adult. Trop Gastroenterol. 2007; 28(4): 186-7.
2) Summerskill WHJ. The syndrome of benign recurrent cholestasis. Am J Med 1965;38:298-305.
3) Tygstrup N, Jensen B. Intermittent intrahepatic cholestasis of unknown etiology in five young males from the Faroe islands. Acta Med Scand 1969;185:523-30.
4) Walensi M, Canbay A, Oliver Witzke, Guido Gerken, Alisan Kahraman. Long term therapy of a patient with Summerskill- Walshe-Tygstrup syndrome by applying Prometheus liver dialysis: A case report. Case Rep Gastroenterol. 2012;6(2):550-556.
5) Geethalakshmi S, Mageshukumar S. Benign recurrent intrahepatic cholestasis: A rare case report. Int J Sci Stud. 2014;2(7):222-224.
6) Kochhar R, Bhasker RV, Mittal PK, Malik AK, Mehta SK. Benign recurrent intrahepatic cholestasis associated with retinitis pigmentosa. Indian J Gastroenterol.1988;7(1):51-2.
7) Gao H, Lin S, LvX, Ma H, Wang X,et al. The identification of two new ABCB11 mutations and the treatment of outcome in a young adult with Benign recurrent intrahepatic cholestasis: A case report. Hepat Mon. 2017; 17(10):e55087.
8) Niaz MK, Ahmed DS, Jennet SNE, Masum QAA et al. A rare case report on Benign recurrent intrahepatic cholestasis. Faridpur Med.Coll.J.2014; 9(2):108-110.
9) De Pagter AG, Van Berge Henegouwen GP, Ten Bokkel Huinink JA, Brandt KH. Familial benign recurrent cholestasis. Interrelation with intrahepatic cholestasis of pregnancy and from oral contraceptives? Gastroenterology. 1976;71:202-207.
10) Putterman C, Keidar S, Brook JG. Benign recurrent intrahepatic cholestasis- 25 years of follow-up. Postgraduate medical journal.1987;63:295-296.
11) Luketic VA, Schiffman ML. Benign recurrent intrahepatic cholestasis. Clin Liver Dis 1999;3:5509-28.
12) Van Ooteghem NA, Klomp LW, van Berge-Henegouwen GP, Houwen RH Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum. J Hepatol 2002;36:439-43.
13) Chhetri D, Gupta R, Duseja A, Dhiman RK, Chawla Y, Das A. Benign recurrent intrahepatic cholestasis in an adult. Trop Gastroenterol. 2007; 28(4): 186-7.
Published
2020-01-31
Issue
Section
Case Report
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