Benign recurrent intrahepatic cholestasis type-II - A rare cause of direct hyperbilirubinemia exacerbations with hepatic fibrosis

Harefuah

Volumn 147, Issue 5, 2008, Pages 381-383

  Mizrahi, Meir ^a   Lalazar, Gadi ^a   Horwich, Yuval ^a   Adar, Tomer ^a   Safadi, Rifaat ^a  

^a HADASSAH HEBREW UNIVERSITY MEDICAL CENTER   (Israel)

Author keywords

Benign recurrent intrahepatic cholestasis (BRIC); Bile Salt Export Pump (BSEP); Biliary bypass liver transplantation; Canalicular cholestasis; Hyperbilirubinemia

Indexed keywords

ADULT; ARTICLE; CASE REPORT; DISEASE COURSE; FEMALE; HUMAN; HYPERBILIRUBINEMIA; INTRAHEPATIC CHOLESTASIS; LIVER DISEASE; OBSTRUCTIVE JAUNDICE; PATHOLOGY; RECURRENT DISEASE; REMISSION;

ADULT; CHOLESTASIS, INTRAHEPATIC; DISEASE PROGRESSION; FEMALE; HUMANS; HYPERBILIRUBINEMIA; JAUNDICE, OBSTRUCTIVE; LIVER DISEASES; RECURRENCE; REMISSION, SPONTANEOUS;

EID: 52449103249     PISSN: 00177768     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (20)

1. Johnson F & Dubin I, Chronic idiopathic jaundice with undentifiefd pigment in liver cells, a new clinicopathologic entity with a report of 12 cases. Medicine, 1954, 33: 155-159.
2. Rotor AB, A familial nonhemolytic jaundice with direct van den Bergh reaction. Acta Med, 1948; 5: 37-40.
3. Summerskill W & Walshe J, Benign recurrent intrahepatic "obstractive" jaundice. Lancet, 1959; 2: 686-689.
4. Summerskill W, The syndrome of benign recurrent cholestasis. Am J Med, 1965; 38: 298-302.
5. Tygstrup N & Jensen B, Intermittent intrahepatic cholestasis of unknown etiology in five young males from the Faroe Islands. Acta Med Scand, 1969; 185: 523-530.
6. Biempica L, Gutstein S & Arias I, Morphological and biochemical studies of benign recurrent cholestasis. Gastroenterology, 1967; 52: 521-525.
7. Tygstrup N, Steig BA, Juijn JA & al, Recurrent familial intrahepatic cholestasis in the Faeroe Islands. Phenotypic heterogeneity but genetic homogeneity. Hepatol, 1999; 29: 506-8.
8. 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.
9. Pawlikowska L, Groen A, Eppens EF & al, A mouse genetic model for familial cholestasis caused by ATP8B1 mutations reveals perturbed bile salt homeostasis but no impairment in bile secretion. Hum Mol Genet, 2004; 13: 881-92.
10. Jansen PL, Strautnieks SS, Jacquemin E & al, Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis. Gastroenterology, 1999; 117: 1370-9.
11. Wang L, Soroka CJ & Boyer JL, The role of bile salt export pump mutations in progressive familial intrahepatic cholestasis type II. J Clin Invest, 2002; 110: 965-72.
12. Kagawa T, Mochizuki K, Harris MJ & al, Bile acid transport activity and intracellualr distribution of PFIC2 mutants in MDCKII cells coexpressing NTCP and BSEP. Hepatology, 2003; 38: 475-480.
13. Schapiro RH & Isselbacher KJ, Benign recurrent intrahepatic cholestasis. N Engl J Med, 1963; 268: 708-11.
14. Spiegel EL, Schubert W, Perrin E & Schiff L, Benign recurrent intrahepatic cholestasis with response to cholestyramine. Am J Med, 1965; 39: 682-688.
15. Crosignani A, Podda M, Berolini E & al, Failure of ursodeoxycholic acid to prevent a cholestatic episode in a patient with benign recurrent intrahepatic cholestasis. Hepatology, 1990; 13: 1076-1083.
16. Bircher J, Correspondence: treatment of patients with benign recurrent intrahepatic cholestasis. Hepatology, 1989; 10: 1030-1033.
17. Balsells F, Wyllie R, Steffen R & Kay M, Benign recurrent intrahepatic cholestasis: improvement of pruritis and shortening of the symptomatic phase with rifampicin therapy: a case report. Clin Ped, 1997: 483-485.
18. Yerushalmi B, Sokol RJ, Narkewicz MR & al, Use of rifampicin for severe pruritis in children with chronic cholestasis. J Pediatr Gastroenterol Nutr, 1999; 29: 442-447.
19. Cancado ELR, Leitao RMC, Carrilho FJ & Laudanna AA, Unexpected clinical remission of cholestasis after rifampicin therapy in patients with normal or slightly increased levels of gamma-glutamyl transpeptidase. Am J Gastroenterol, 1998; 93: 1510-1517.
20. Ismail H, Kalianski P, Markiewicz M & al, Treatment of progressive familial intrahepatic cholestasis: liver transplantation or partial external biliary diversion. Pediatr Transplant, 1999; 3: 219-224.