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Volumn 25, Issue 3, 2009, Pages 175-179

Cholestasis and cholestatic syndromes

Author keywords

Biliary transporters; Cholestasis; Primary biliary cirrhosis; Sclerosing cholangitis

Indexed keywords

TUMOR NECROSIS FACTOR RELATED APOPTOSIS INDUCING LIGAND;

EID: 67651109156     PISSN: 02671379     EISSN: None     Source Type: Journal    
DOI: 10.1097/MOG.0b013e32832914b4     Document Type: Review
Times cited : (23)

References (32)
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    • ABCB4 Heterozygous Gene Mutations Associated with Fibrosing Cholestatic Liver Disease in Adults
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    • A mutation in the canalicular phospholipid transporter gene, ABCB4, is associated with cholestasis, ductopenia, and cirrhosis in adults
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    • Comment on biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis
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    • Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells
    • Combination therapy for PBC with UDCA and steroids has been suggested. This study elegantly identifies molecular mechanisms that could contribute biologically to such a pharmacological approach, and identifies a biliary transporter as relevant therefore to both disease etiopathogenesis and treatment
    • Arenas F, Hervias I, Uriz M, et al. Combination of ursodeoxycholic acid and glucocorticoids upregulates the AE2 alternate promoter in human liver cells. J Clin Invest 2008; 118:695-709. Combination therapy for PBC with UDCA and steroids has been suggested. This study elegantly identifies molecular mechanisms that could contribute biologically to such a pharmacological approach, and identifies a biliary transporter as relevant therefore to both disease etiopathogenesis and treatment.
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    • Arenas, F.1    Hervias, I.2    Uriz, M.3
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    • Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis
    • Until recently, there were few animal models of PBC. This knockout mouse (AE2) adds to the growing list of murine genetic changes that lead to autoimmune cholangitis
    • Salas JT, Banales JM, Sarvide S, et al. Ae2a,b-deficient mice develop antimitochondrial antibodies and other features resembling primary biliary cirrhosis. Gastroenterology 2008; 134:1482-1493. Until recently, there were few animal models of PBC. This knockout mouse (AE2) adds to the growing list of murine genetic changes that lead to autoimmune cholangitis.
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    • Salas, J.T.1    Banales, J.M.2    Sarvide, S.3
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    • Hedgehog signaling regulates epithelial-mesenchymal transition during biliary fibrosis in rodents and humans
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    • Omenetti A, Porrello A, Jung Y, et al. Hedgehog signaling regulates epithelial-mesenchymal transition during biliary fibrosis in rodents and humans. J Clin Invest 2008; 118:3331-3342. This group has championed the role of the Hh signaling pathway in biliary fibrosis. This study now links the pathway to EMT in biliary fibrosis, another pathological mechanism proposed to be relevant to cholestatic liver disease.
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    • Inhibition of integrin alphavbeta6 on cholangiocytes blocks transforming growth factor-beta activation and retards biliary fibrosis progression
    • A nice study demonstrating that inhibition of integrin alphavbeta6 may be of value to those with progressive biliary diseases
    • Patsenker E, Popov Y, Stickel F, et al. Inhibition of integrin alphavbeta6 on cholangiocytes blocks transforming growth factor-beta activation and retards biliary fibrosis progression. Gastroenterology 2008; 135:660-670. A nice study demonstrating that inhibition of integrin alphavbeta6 may be of value to those with progressive biliary diseases.
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    • Patsenker, E.1    Popov, Y.2    Stickel, F.3
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    • Interacting alleles of the coinhibitory immunoreceptor genes cytotoxic T-lymphocyte antigen 4 and programmed cell-death 1 influence risk and features of primary biliary cirrhosis
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    • Juran, B.D.1    Atkinson, E.J.2    Schlicht, E.M.3
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    • Primary biliary cirrhosis is associated with a genetic variant in the 3′ flanking region of the CTLA4 gene
    • Demonstration that PBC has a genetic association with the immunoregulatory gene CTLA-4
    • Juran BD, Atkinson EJ, Schlicht EM, et al. Primary biliary cirrhosis is associated with a genetic variant in the 3′ flanking region of the CTLA4 gene. Gastroenterology 2008; 135:1200-1206. Demonstration that PBC has a genetic association with the immunoregulatory gene CTLA-4.
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    • Juran, B.D.1    Atkinson, E.J.2    Schlicht, E.M.3
  • 23
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    • Human leukocyte antigen polymorphisms in Italian primary biliary cirrhosis: A multicenter study of 664 patients and 1992 healthy controls
    • A large study reinforcing the association between PBC and the human leukocyte antigen system. This paper additionally serves as a reminder of the appropriate study size needed to address genetic causes of multifactorial diseases, which for relatively rare diseases such as those seen in cholestatic liver disease, requires collaboration
    • Invernizzi P, Selmi C, Poli F, et al. Human leukocyte antigen polymorphisms in Italian primary biliary cirrhosis: a multicenter study of 664 patients and 1992 healthy controls. Hepatology 2008; 48:1906-1912. A large study reinforcing the association between PBC and the human leukocyte antigen system. This paper additionally serves as a reminder of the appropriate study size needed to address genetic causes of multifactorial diseases, which for relatively rare diseases such as those seen in cholestatic liver disease, requires collaboration.
    • (2008) Hepatology , vol.48 , pp. 1906-1912
    • Invernizzi, P.1    Selmi, C.2    Poli, F.3
  • 24
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    • Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis
    • A useful study for clinicians and patients identifying biochemical predictors of long-term outcome for patients with PBC. Normal liver biochemistry after 1 year of treatment with UDCA is associated with an excellent outcome for individuals.
    • Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology 2008; 48:871-877. A useful study for clinicians and patients identifying biochemical predictors of long-term outcome for patients with PBC. Normal liver biochemistry after 1 year of treatment with UDCA is associated with an excellent outcome for individuals.
    • (2008) Hepatology , vol.48 , pp. 871-877
    • Corpechot, C.1    Abenavoli, L.2    Rabahi, N.3
  • 25
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    • Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid
    • Pares A, Caballeria L, Rodes J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology 2006; 130:715-720.
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    • Pares, A.1    Caballeria, L.2    Rodes, J.3
  • 26
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    • Portal hypertension and primary biliary cirrhosis: Effect of long-term ursodeoxycholic acid treatment
    • A unique long-term study published that looked at portal hypertension serially in patients with PBC treated with UDCA. 'Responders' (stable or improved PHG and normalized AST level at 2 years) had a 15-year survival similar to healthy controls
    • Huet PM, Vincent C, Deslaurier J, et al. Portal hypertension and primary biliary cirrhosis: effect of long-term ursodeoxycholic acid treatment. Gastroenterology 2008; 135:1552-1560. A unique long-term study published that looked at portal hypertension serially in patients with PBC treated with UDCA. 'Responders' (stable or improved PHG and normalized AST level at 2 years) had a 15-year survival similar to healthy controls.
    • (2008) Gastroenterology , vol.135 , pp. 1552-1560
    • Huet, P.M.1    Vincent, C.2    Deslaurier, J.3
  • 27
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    • Prediction of clinical outcomes in primary biliary cirrhosis by serum enhanced liver fibrosis assay
    • Mayo MJ, Parkes J, Adams-Huet B, et al. Prediction of clinical outcomes in primary biliary cirrhosis by serum enhanced liver fibrosis assay. Hepatology 2008; 48:1549-1557.
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    • Mayo, M.J.1    Parkes, J.2    Adams-Huet, B.3
  • 28
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    • Mortality attributable to cholestatic liver disease in the United States
    • A reminder, if needed, that the greatest challenge in cholestatic liver disease is to change the natural history of sclerosing cholangitis. As compared with PBC in which mortality is falling, mortality for PSC remains disturbingly untouched
    • Mendes FD, Kim WR, Pedersen R, et al. Mortality attributable to cholestatic liver disease in the United States. Hepatology 2008; 47:1241-1247. A reminder, if needed, that the greatest challenge in cholestatic liver disease is to change the natural history of sclerosing cholangitis. As compared with PBC in which mortality is falling, mortality for PSC remains disturbingly untouched.
    • (2008) Hepatology , vol.47 , pp. 1241-1247
    • Mendes, F.D.1    Kim, W.R.2    Pedersen, R.3
  • 29
    • 43149104736 scopus 로고    scopus 로고
    • Incidence and mortality of primary sclerosing cholangitis in the UK: A population-based cohort study
    • Card TR, Solaymani-Dodaran M, West J. Incidence and mortality of primary sclerosing cholangitis in the UK: a population-based cohort study. J Hepatol 2008; 48:939-944.
    • (2008) J Hepatol , vol.48 , pp. 939-944
    • Card, T.R.1    Solaymani-Dodaran, M.2    West, J.3
  • 30
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    • The natural history of small-duct primary sclerosing cholangitis
    • A unique collection of patients from across the world with small-duct PSC demonstrating how about one in five progresses to large-duct disease over about 7 years. Cholangiocarcinoma was only seen in an individual who progressed
    • Bjornsson E, Olsson R, Bergquist A, et al. The natural history of small-duct primary sclerosing cholangitis. Gastroenterology 2008; 134:975-980. A unique collection of patients from across the world with small-duct PSC demonstrating how about one in five progresses to large-duct disease over about 7 years. Cholangiocarcinoma was only seen in an individual who progressed.
    • (2008) Gastroenterology , vol.134 , pp. 975-980
    • Bjornsson, E.1    Olsson, R.2    Bergquist, A.3
  • 32
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    • Immunoglobulin G4-associated cholangitis: Clinical profile and response to therapy
    • A useful clinic description of IgG4-associated autoimmune pancreatitis/sclerosing cholangitis, demonstrating therapeutic responses and clinical outcomes
    • Ghazale A, Chari ST, Zhang L, et al. Immunoglobulin G4-associated cholangitis: clinical profile and response to therapy. Gastroenterology 2008; 134:706-715. A useful clinic description of IgG4-associated autoimmune pancreatitis/sclerosing cholangitis, demonstrating therapeutic responses and clinical outcomes.
    • (2008) Gastroenterology , vol.134 , pp. 706-715
    • Ghazale, A.1    Chari, S.T.2    Zhang, L.3


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