-
1
-
-
0020607713
-
Immaturity of the enterohepatic circulation in early life: Factors predisposing to "physiologic" maldigestion and cholestasis
-
6348232 1:STN:280:DyaL3s3msl2ntA%3D%3D
-
WF Balistreri JE Heubi FJ Suchy 1983 Immaturity of the enterohepatic circulation in early life: factors predisposing to "physiologic" maldigestion and cholestasis J Pediatr Gastroenterol Nutr 2 346 354 6348232 1:STN:280:DyaL3s3msl2ntA%3D%3D
-
(1983)
J Pediatr Gastroenterol Nutr
, vol.2
, pp. 346-354
-
-
Balistreri, W.F.1
Heubi, J.E.2
Suchy, F.J.3
-
2
-
-
29244446225
-
Whatever happened to "neonatal hepatitis"?
-
DOI 10.1016/j.cld.2005.10.008, PII S1089326105000966, Pediatric Hepatology: Progress in Deciphering Mechanisms and Advancing Therapy
-
WF Balistreri JA Bezerra 2006 Whatever happened to "neonatal hepatitis"? Clin Liver Dis 10 27 53 10.1016/j.cld.2005.10.008 16376793 (Pubitemid 41821376)
-
(2006)
Clinics in Liver Disease
, vol.10
, Issue.1
, pp. 27-53
-
-
Balistreri, W.F.1
Bezerra, J.A.2
-
3
-
-
68049131203
-
New molecular insights into the mechanisms of cholestasis
-
10.1016/j.jhep.2009.05.012 19595470 1:CAS:528:DC%2BD1MXpsFyhs7g%3D
-
M Wagner G Zollner M Trauner 2009 New molecular insights into the mechanisms of cholestasis J Hepatol 51 565 580 10.1016/j.jhep.2009.05.012 19595470 1:CAS:528:DC%2BD1MXpsFyhs7g%3D
-
(2009)
J Hepatol
, vol.51
, pp. 565-580
-
-
Wagner, M.1
Zollner, G.2
Trauner, M.3
-
4
-
-
67651047020
-
Prevalence of subclinical vitamin k deficiency in cholestatic liver disease
-
10.1097/MPG.0b013e31819a61ff 19502999 1:CAS:528:DC%2BD1MXptFCrsLs%3D
-
J Strople G Lovell J Heubi 2009 Prevalence of subclinical vitamin k deficiency in cholestatic liver disease J Pediatr Gastroenterol Nutr 49 78 84 10.1097/MPG.0b013e31819a61ff 19502999 1:CAS:528:DC%2BD1MXptFCrsLs%3D
-
(2009)
J Pediatr Gastroenterol Nutr
, vol.49
, pp. 78-84
-
-
Strople, J.1
Lovell, G.2
Heubi, J.3
-
5
-
-
7644235252
-
High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis
-
DOI 10.1016/S0140-6736(04)17356-X, PII S014067360417356X
-
PF Whitington JU Hibbard 2004 High-dose immunoglobulin during pregnancy for recurrent neonatal haemochromatosis Lancet 364 1690 1698 10.1016/S0140-6736(04)17356-X 15530630 1:CAS:528:DC%2BD2cXpsVKht7w%3D (Pubitemid 39458145)
-
(2004)
Lancet
, vol.364
, Issue.9446
, pp. 1690-1698
-
-
Whitington, P.F.1
Hibbard, J.U.2
-
6
-
-
48949090831
-
Outcome of pregnancies at risk for neonatal hemochromatosis is improved by treatment with high-dose intravenous immunoglobulin
-
10.1542/peds.2007-3107 18474533 This article reports the impact of IV Ig administered during pregnancy on the outcome of neonates with iron storage disease. IV Ig was administered weekly to women from 18 weeks until the end of at-risk pregnancies. Gestational therapy reduced the recurrence rate of liver disease and promoted survival in 98% of neonates
-
PF Whitington S Kelly 2008 Outcome of pregnancies at risk for neonatal hemochromatosis is improved by treatment with high-dose intravenous immunoglobulin Pediatrics 121 e1615 e1621 10.1542/peds.2007-3107 18474533 This article reports the impact of IV Ig administered during pregnancy on the outcome of neonates with iron storage disease. IV Ig was administered weekly to women from 18 weeks until the end of at-risk pregnancies. Gestational therapy reduced the recurrence rate of liver disease and promoted survival in 98% of neonates
-
(2008)
Pediatrics
, vol.121
-
-
Whitington, P.F.1
Kelly, S.2
-
7
-
-
67651154714
-
Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase i modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency
-
10.1002/hep.22974 19444872 1:CAS:528:DC%2BD1MXpt1Shtr0%3D
-
S Pan L Huang J McPherson, et al. 2009 Single nucleotide polymorphism-mediated translational suppression of endoplasmic reticulum mannosidase I modifies the onset of end-stage liver disease in alpha1-antitrypsin deficiency Hepatology 50 275 281 10.1002/hep.22974 19444872 1:CAS:528:DC%2BD1MXpt1Shtr0%3D
-
(2009)
Hepatology
, vol.50
, pp. 275-281
-
-
Pan, S.1
Huang, L.2
McPherson, J.3
-
8
-
-
49149090637
-
Hepatocyte-specific ablation of Foxa2 alters bile acid homeostasis and results in endoplasmic reticulum stress
-
10.1038/nm.1853 18660816 1:CAS:528:DC%2BD1cXptlKltLo%3D This report describes the biologic consequences of the inactivation of the Foxa2 gene in mice. Loss of Foxa2 resulted in intrahepatic cholestasis associated with a decreased expression of genes involved in bile acid transport at the basolateral and canalicular membranes. Foxa2 was also found to be decreased in children and adults with cholestatic syndromes
-
IM Bochkis NE Rubins P White, et al. 2008 Hepatocyte-specific ablation of Foxa2 alters bile acid homeostasis and results in endoplasmic reticulum stress Nat Med 14 828 836 10.1038/nm.1853 18660816 1:CAS:528:DC%2BD1cXptlKltLo%3D This report describes the biologic consequences of the inactivation of the Foxa2 gene in mice. Loss of Foxa2 resulted in intrahepatic cholestasis associated with a decreased expression of genes involved in bile acid transport at the basolateral and canalicular membranes. Foxa2 was also found to be decreased in children and adults with cholestatic syndromes
-
(2008)
Nat Med
, vol.14
, pp. 828-836
-
-
Bochkis, I.M.1
Rubins, N.E.2
White, P.3
-
9
-
-
68349139310
-
Allograft steatohepatitis in progressive familial intrahepatic cholestasis type 1 after living donor liver transplantation
-
10.1002/lt.21686 19479804 This article reports the development of macrovesicular steatosis in 8 of 11 patients who received living-donor liver transplantation for progressive familial intrahepatic cholestasis. Patients developed steatohepatitis (7 of 8), bridging fibrosis (6 of 8), or cirrhosis (2 of 8) in the liver graft. Posttransplant diarrhea occurred in all eight patients with hepatic steatosis
-
A Miyagawa-Hayashino H Egawa T Yorifuji M Hasegawa, et al. 2009 Allograft steatohepatitis in progressive familial intrahepatic cholestasis type 1 after living donor liver transplantation Liver Transpl 15 610 618 10.1002/lt.21686 19479804 This article reports the development of macrovesicular steatosis in 8 of 11 patients who received living-donor liver transplantation for progressive familial intrahepatic cholestasis. Patients developed steatohepatitis (7 of 8), bridging fibrosis (6 of 8), or cirrhosis (2 of 8) in the liver graft. Posttransplant diarrhea occurred in all eight patients with hepatic steatosis
-
(2009)
Liver Transpl
, vol.15
, pp. 610-618
-
-
Miyagawa-Hayashino, A.1
Egawa, H.2
Yorifuji, T.3
Hasegawa, M.4
-
10
-
-
38649113883
-
ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity
-
DOI 10.1002/hep.21950
-
CC Paulusma DE Folmer KS Ho-Mok, et al. 2008 ATP8B1 requires an accessory protein for endoplasmic reticulum exit and plasma membrane lipid flippase activity Hepatology 47 268 278 10.1002/hep.21950 17948906 1:CAS:528: DC%2BD1cXhtlChsbs%3D (Pubitemid 351171064)
-
(2008)
Hepatology
, vol.47
, Issue.1
, pp. 268-278
-
-
Paulusma, C.C.1
Folmer, D.E.2
Ho-Mok, K.S.3
De Waart, D.R.4
Hilarius, P.M.5
Verhoeven, A.J.6
Oude Elferink, R.P.J.7
-
11
-
-
72949094009
-
Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type 1 mutations on canalicular localization of ATP8B1
-
10.1002/hep.23158 19731236 1:CAS:528:DC%2BD1MXhsV2js7rL
-
DE Folmer VA van der Mark KS Ho-Mok, et al. 2009 Differential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type 1 mutations on canalicular localization of ATP8B1 Hepatology 50 1597 1605 10.1002/hep.23158 19731236 1:CAS:528:DC%2BD1MXhsV2js7rL
-
(2009)
Hepatology
, vol.50
, pp. 1597-1605
-
-
Folmer, D.E.1
Van Der Mark, V.A.2
Ho-Mok, K.S.3
-
12
-
-
58149401293
-
The membrane protein ATPase class i type 8B member 1 signals through protein kinase C zeta to activate the farnesoid X receptor
-
10.1002/hep.22431 18668687 1:CAS:528:DC%2BD1cXhsFamsrfF This article reports the functional relationship between ATP8B1 (FIC1), FXR, and BSEP promoter using a cell-culture system. The investigators found that the overexpression of FIC1in a cell culture system promoted phosphorylation and nuclear localization of FXR, which was blocked by protein kinase C zeta inhibitors. By expressing FIC1 mutants based on sequence results from patients with PFIC-1 and BRIC-1, they found that severe mutations in FIC1 were unable to induce FXR-dependent
-
T Frankenberg T Miloh FY Chen, et al. 2008 The membrane protein ATPase class I type 8B member 1 signals through protein kinase C zeta to activate the farnesoid X receptor Hepatology 48 1896 1905 10.1002/hep.22431 18668687 1:CAS:528:DC%2BD1cXhsFamsrfF This article reports the functional relationship between ATP8B1 (FIC1), FXR, and BSEP promoter using a cell-culture system. The investigators found that the overexpression of FIC1in a cell culture system promoted phosphorylation and nuclear localization of FXR, which was blocked by protein kinase C zeta inhibitors. By expressing FIC1 mutants based on sequence results from patients with PFIC-1 and BRIC-1, they found that severe mutations in FIC1 were unable to induce FXR-dependent
-
(2008)
Hepatology
, vol.48
, pp. 1896-1905
-
-
Frankenberg, T.1
Miloh, T.2
Chen, F.Y.3
-
13
-
-
60449097716
-
ATP8B1 deficiency disrupts the bile canalicular membrane bilayer structure in hepatocytes, but FXR expression and activity are maintained
-
10.1053/j.gastro.2008.10.025 19027009 1:CAS:528:DC%2BD1MXjslSju7s%3D This article investigated whether ATP8B1 deficiency produces cholestasis by affecting FXR activity or by impairing the structure of the canalicular membrane. Knocking down the ATP8B1 gene using a siRNA-based approach, the authors found no changes in the expression of FXR or FXR-dependent membrane transporters. In contrast, cells with suppressed ATP8B1 had focal areas of canalicular disruption by electron microscopy when exposed to bile acids
-
SY Cai S Gautam T Nguyen, et al. 2009 ATP8B1 deficiency disrupts the bile canalicular membrane bilayer structure in hepatocytes, but FXR expression and activity are maintained Gastroenterology 136 1060 1069 10.1053/j.gastro.2008.10. 025 19027009 1:CAS:528:DC%2BD1MXjslSju7s%3D This article investigated whether ATP8B1 deficiency produces cholestasis by affecting FXR activity or by impairing the structure of the canalicular membrane. Knocking down the ATP8B1 gene using a siRNA-based approach, the authors found no changes in the expression of FXR or FXR-dependent membrane transporters. In contrast, cells with suppressed ATP8B1 had focal areas of canalicular disruption by electron microscopy when exposed to bile acids
-
(2009)
Gastroenterology
, vol.136
, pp. 1060-1069
-
-
Cai, S.Y.1
Gautam, S.2
Nguyen, T.3
-
14
-
-
56149102929
-
Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II
-
10.1002/hep.22499 18798335 1:CAS:528:DC%2BD1cXhsVKmt77M
-
L Wang H Dong CJ Soroka, et al. 2008 Degradation of the bile salt export pump at endoplasmic reticulum in progressive familial intrahepatic cholestasis type II Hepatology 48 1558 1569 10.1002/hep.22499 18798335 1:CAS:528: DC%2BD1cXhsVKmt77M
-
(2008)
Hepatology
, vol.48
, pp. 1558-1569
-
-
Wang, L.1
Dong, H.2
Soroka, C.J.3
-
15
-
-
61949292897
-
Missense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing
-
10.1002/hep.22683 19101985 1:CAS:528:DC%2BD1MXivFWht7s%3D
-
JA Byrne SS Strautnieks G Ihrke, et al. 2009 Missense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicing Hepatology 49 553 567 10.1002/hep.22683 19101985 1:CAS:528:DC%2BD1MXivFWht7s%3D
-
(2009)
Hepatology
, vol.49
, pp. 553-567
-
-
Byrne, J.A.1
Strautnieks, S.S.2
Ihrke, G.3
-
16
-
-
41549123313
-
Severe Bile Salt Export Pump Deficiency: 82 Different ABCB11 Mutations in 109 Families
-
DOI 10.1053/j.gastro.2008.01.038, PII S0016508508001121
-
SS Strautnieks JA Byrne L Pawlikowska, et al. 2008 Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families Gastroenterology 134 1203 1214 10.1053/j.gastro.2008.01.038 18395098 1:CAS:528:DC%2BD1cXls1GlsLY%3D This article describes the clinical phenotype and biallelic mutations in most children diagnosed with deficiency of BSEP. About 93% of the subjects had abnormal or absent BSEP on liver biopsies. Fifteen percent of the patients also developed hepatocellular carcinoma/ cholangiocarcinoma, with the incidence further increasing to 38% if the mutations produced truncated proteins (Pubitemid 351459603)
-
(2008)
Gastroenterology
, vol.134
, Issue.4
, pp. 1203-1214
-
-
Strautnieks, S.S.1
Byrne, J.A.2
Pawlikowska, L.3
Cebecauerova, D.4
Rayner, A.5
Dutton, L.6
Meier, Y.7
Antoniou, A.8
Stieger, B.9
Arnell, H.10
Ozcay, F.11
Al-Hussaini, H.F.12
Bassas, A.F.13
Verkade, H.J.14
Fischler, B.15
Nemeth, A.16
Kotalova, R.17
Shneider, B.L.18
Cielecka-Kuszyk, J.19
McClean, P.20
Whitington, P.F.21
Sokal, E.22
Jirsa, M.23
Wali, S.H.24
Jankowska, I.25
Pawlowska, J.26
Mieli-Vergani, G.27
Knisely, A.S.28
Bull, L.N.29
Thompson, R.J.30
more..
-
17
-
-
33747032310
-
Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency
-
DOI 10.1002/hep.21287
-
AS Knisely SS Strautnieks Y Meier, et al. 2006 Hepatocellular carcinoma in ten children under five years of age with bile salt export pump deficiency Hepatology 44 478 486 10.1002/hep.21287 16871584 1:CAS:528:DC%2BD28XptVKmtLc%3D (Pubitemid 44214888)
-
(2006)
Hepatology
, vol.44
, Issue.2
, pp. 478-486
-
-
Knisely, A.S.1
Strautnieks, S.S.2
Meier, Y.3
Stieger, B.4
Byrne, J.A.5
Portmann, B.C.6
Bull, L.N.7
Pawlikowska, L.8
Bilezikci, B.9
Ozcay, F.10
Laszlo, A.11
Tiszlavicz, L.12
Moore, L.13
Raftos, J.14
Arnell, H.15
Fischler, B.16
Nemeth, A.17
Papadogiannakis, N.18
Cielecka-Kuszyk, J.19
Jankowska, I.20
Pawlowska, J.21
Melin-Aldana, H.22
Emerick, K.M.23
Whitington, P.F.24
Mieli-Vergani, G.25
Thompson, R.J.26
more..
-
18
-
-
34247177532
-
Mutations in Bile Salt Export Pump (ABCB11) in Two Children with Progressive Familial Intrahepatic Cholestasis and Cholangiocarcinoma
-
DOI 10.1016/j.jpeds.2007.02.030, PII S0022347607001345
-
AO Scheimann SS Strautnieks AS Knisely, et al. 2007 Mutations in bile salt export pump (ABCB11) in two children with progressive familial intrahepatic cholestasis and cholangiocarcinoma J Pediatr 150 556 559 10.1016/j.jpeds.2007. 02.030 17452236 1:CAS:528:DC%2BD2sXksFahsL0%3D (Pubitemid 46602338)
-
(2007)
Journal of Pediatrics
, vol.150
, Issue.5
, pp. 556-559
-
-
Scheimann, A.O.1
Strautnieks, S.S.2
Knisely, A.S.3
Byrne, J.A.4
Thompson, R.J.5
Finegold, M.J.6
-
19
-
-
68949190850
-
De novo bile salt transporter antibodies as a possible cause of recurrent graft failure after liver transplantation: A novel mechanism of cholestasis
-
10.1002/hep.23083 19642168 1:CAS:528:DC%2BD1MXhtVKrtrrK This article reports the clinical features and outcome of a child who developed recurrence of cholestatic liver disease following transplantation for complications of BSEP deficiency. The patient's serum was reactive to a domain of the extracellular loop of BSEP and inhibited its transport activity
-
V Keitel M Burdelski Z Vojnisek, et al. 2009 De novo bile salt transporter antibodies as a possible cause of recurrent graft failure after liver transplantation: a novel mechanism of cholestasis Hepatology 50 510 517 10.1002/hep.23083 19642168 1:CAS:528:DC%2BD1MXhtVKrtrrK This article reports the clinical features and outcome of a child who developed recurrence of cholestatic liver disease following transplantation for complications of BSEP deficiency. The patient's serum was reactive to a domain of the extracellular loop of BSEP and inhibited its transport activity
-
(2009)
Hepatology
, vol.50
, pp. 510-517
-
-
Keitel, V.1
Burdelski, M.2
Vojnisek, Z.3
-
20
-
-
70349626199
-
Recurrence of bile salt export pump deficiency after liver transplantation
-
10.1056/NEJMoa0901075 19797282 1:CAS:528:DC%2BD1MXht1CksrzK This article reports the clinical features and outcome of three children who developed recurrence of cholestatic liver disease following transplantation for end-stage liver disease due to BSEP deficiency. All patients had circulating high-titer antibodies that recognized BSEP and inhibited its transport properties
-
P Jara L Hierro P Martinez-Fernandez, et al. 2009 Recurrence of bile salt export pump deficiency after liver transplantation N Engl J Med 361 1359 1367 10.1056/NEJMoa0901075 19797282 1:CAS:528:DC%2BD1MXht1CksrzK This article reports the clinical features and outcome of three children who developed recurrence of cholestatic liver disease following transplantation for end-stage liver disease due to BSEP deficiency. All patients had circulating high-titer antibodies that recognized BSEP and inhibited its transport properties
-
(2009)
N Engl J Med
, vol.361
, pp. 1359-1367
-
-
Jara, P.1
Hierro, L.2
Martinez-Fernandez, P.3
-
21
-
-
65449136273
-
A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature
-
10.1002/hep.22775 19185004 1:CAS:528:DC%2BD1MXkslWjsr0%3D
-
JL Delaunay AM Durand-Schneider D Delautier, et al. 2009 A missense mutation in ABCB4 gene involved in progressive familial intrahepatic cholestasis type 3 leads to a folding defect that can be rescued by low temperature Hepatology 49 1218 1227 10.1002/hep.22775 19185004 1:CAS:528: DC%2BD1MXkslWjsr0%3D
-
(2009)
Hepatology
, vol.49
, pp. 1218-1227
-
-
Delaunay, J.L.1
Durand-Schneider, A.M.2
Delautier, D.3
-
22
-
-
0040284751
-
Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis
-
DOI 10.1073/pnas.95.1.282
-
JM de Vree E Jacquemin E Sturm, et al. 1998 Mutations in the MDR3 gene cause progressive familial intrahepatic cholestasis Proc Natl Acad Sci U S A 95 282 287 10.1073/pnas.95.1.282 9419367 (Pubitemid 28103945)
-
(1998)
Proceedings of the National Academy of Sciences of the United States of America
, vol.95
, Issue.1
, pp. 282-287
-
-
De Vree, J.M.L.1
Jacquemin, E.2
Sturm, E.3
Cresteil, D.4
Bosma, P.J.5
Aten, J.6
Deleuze, J.-F.7
Desrochers, M.8
Burdelski, M.9
Bernard, O.10
Oude Elferink, R.P.J.11
Hadchouel, M.12
-
23
-
-
0029990296
-
Defect of multidrug-resistance 3 gene expression in a subtype of progressive familial intrahepatic cholestasis
-
10.1002/hep.510230435 8666348 1:CAS:528:DyaK28XisFygtL0%3D
-
JF Deleuze E Jacquemin C Dubuisson, et al. 1996 Defect of multidrug-resistance 3 gene expression in a subtype of progressive familial intrahepatic cholestasis Hepatology 23 904 908 10.1002/hep.510230435 8666348 1:CAS:528:DyaK28XisFygtL0%3D
-
(1996)
Hepatology
, vol.23
, pp. 904-908
-
-
Deleuze, J.F.1
Jacquemin, E.2
Dubuisson, C.3
-
24
-
-
33847064667
-
MDR3 (ABCB4) defects: A paradigm for the genetics of adult cholestatic syndromes
-
10.1055/s-2006-960172 17295178 1:CAS:528:DC%2BD2sXjtlWrsLg%3D
-
M Trauner P Fickert M Wagner 2007 MDR3 (ABCB4) defects: a paradigm for the genetics of adult cholestatic syndromes Semin Liver Dis 27 77 98 10.1055/s-2006-960172 17295178 1:CAS:528:DC%2BD2sXjtlWrsLg%3D
-
(2007)
Semin Liver Dis
, vol.27
, pp. 77-98
-
-
Trauner, M.1
Fickert, P.2
Wagner, M.3
-
25
-
-
46049094386
-
ABCB4 Heterozygous Gene Mutations Associated With Fibrosing Cholestatic Liver Disease in Adults
-
DOI 10.1053/j.gastro.2008.03.044, PII S0016508508005386
-
M Ziol V Barbu O Rosmorduc, et al. 2008 ABCB4 heterozygous gene mutations associated with fibrosing cholestatic liver disease in adults Gastroenterology 135 131 141 10.1053/j.gastro.2008.03.044 18482588 1:CAS:528:DC%2BD1cXps1Wlt7g%3D This article reports the mutation analysis of the ABCB4 gene in 32 adults with anicteric cholestasis of unknown etiology. The authors found heterozygous mutations in 34% of patients, with decreased or absent MDR3 staining (Pubitemid 351899020)
-
(2008)
Gastroenterology
, vol.135
, Issue.1
, pp. 131-141
-
-
Ziol, M.1
Barbu, V.2
Rosmorduc, O.3
Frassati-Biaggi, A.4
Barget, N.5
Hermelin, B.6
Scheffer, G.L.7
Bennouna, S.8
Trinchet, J.9
Beaugrand, M.10
Ganne-Carrie, N.11
-
26
-
-
62149139054
-
Congenital biliary atresia: Liver injury begins at birth
-
10.1016/j.jpedsurg.2008.10.069 19302872
-
E Makin A Quaglia N Kvist, et al. 2009 Congenital biliary atresia: liver injury begins at birth J Pediatr Surg 44 630 633 10.1016/j.jpedsurg.2008.10.069 19302872
-
(2009)
J Pediatr Surg
, vol.44
, pp. 630-633
-
-
Makin, E.1
Quaglia, A.2
Kvist, N.3
-
27
-
-
66049112396
-
Impact of age at Kasai operation on its results in late childhood and adolescence: A rational basis for biliary atresia screening
-
10.1542/peds.2008-1949 19403492
-
MO Serinet BE Wildhaber P Broue, et al. 2009 Impact of age at Kasai operation on its results in late childhood and adolescence: a rational basis for biliary atresia screening Pediatrics 123 1280 1286 10.1542/peds.2008-1949 19403492
-
(2009)
Pediatrics
, vol.123
, pp. 1280-1286
-
-
Serinet, M.O.1
Wildhaber, B.E.2
Broue, P.3
-
28
-
-
42249098059
-
Universal screening for biliary atresia using an infant stool color card in Taiwan
-
DOI 10.1002/hep.22182
-
CH Hsiao MH Chang HL Chen, et al. 2008 Universal screening for biliary atresia using an infant stool color card in Taiwan Hepatology 47 1233 1240 10.1002/hep.22182 18306391 (Pubitemid 351547909)
-
(2008)
Hepatology
, vol.47
, Issue.4
, pp. 1233-1240
-
-
Hsiao, C.-H.1
Chang, M.-H.2
Chen, H.-L.3
Lee, H.-C.4
Wu, T.-C.5
Lin, C.-C.6
Yang, Y.-J.7
Chen, A.-C.8
Tiao, M.-M.9
Lau, B.-H.10
Chu, C.-H.11
Lai, M.-W.12
Fang, S.-B.13
Wen, W.-H.14
Wang, Y.-H.15
Wang, K.-S.16
Shih, C.-S.17
Yu, C.-H.18
Lee, K.-M.19
Lee, I.-H.20
Lin, L.-H.21
Lin, W.-T.22
Lin, Y.-C.23
Chou, Y.-K.24
Shih, H.-H.25
Chang, P.-F.26
Fan, C.-H.27
Hsu, Y.-C.28
Huang, C.-F.29
Huang, I.-F.30
Huang, M.-L.31
Tang, K.-T.32
Yeung, C.-Y.33
Yeh, J.-N.34
Chen, M.-H.35
Chen, H.-W.36
Chen, S.-M.37
Chen, T.-J.38
Lai, M.-K.39
Tsai, Y.-H.40
Hsieh, T.-K.41
more..
-
29
-
-
56049102133
-
Biliary atresia: We still operate too late
-
10.2223/JPED.1825
-
CO Kieling JL Santos SM Vieira, et al. 2008 Biliary atresia: we still operate too late J Pediatr (Rio J) 84 436 441 10.2223/JPED.1825
-
(2008)
J Pediatr (Rio J)
, vol.84
, pp. 436-441
-
-
Kieling, C.O.1
Santos, J.L.2
Vieira, S.M.3
-
30
-
-
34548319067
-
Screening and outcomes in biliary atresia: Summary of a national institutes of health workshop
-
DOI 10.1002/hep.21790
-
RJ Sokol RW Shepherd R Superina, et al. 2007 Screening and outcomes in biliary atresia: summary of a National Institutes of Health workshop Hepatology 46 566 581 10.1002/hep.21790 17661405 1:CAS:528:DC%2BD2sXhtVSisbzL (Pubitemid 47344793)
-
(2007)
Hepatology
, vol.46
, Issue.2
, pp. 566-581
-
-
Sokol, R.J.1
Shepherd, R.W.2
Superina, R.3
Bezerra, J.A.4
Robuck, P.5
Hoofnagle, J.H.6
-
31
-
-
0034235859
-
Early prediction of long-term survival and the timing of liver transplantation after the Kasai operation
-
DOI 10.1053/jpsu.2000.7765
-
Y Ohhama M Shinkai S Fujita, et al. 2000 Early prediction of long-term survival and the timing of liver transplantation after the Kasai operation J Pediatr Surg 35 1031 1034 10.1053/jpsu.2000.7765 10917290 1:STN:280: DC%2BD3M%2FmslOruw%3D%3D (Pubitemid 30432184)
-
(2000)
Journal of Pediatric Surgery
, vol.35
, Issue.7
, pp. 1031-1034
-
-
Ohhama, Y.1
Shinkai, M.2
Fujita, S.3
Nishi, T.4
Yamamoto, H.5
-
32
-
-
33646086401
-
A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000
-
10.1016/j.jpeds.2005.12.054 16647406
-
BL Shneider MB Brown B Haber, et al. 2006 A multicenter study of the outcome of biliary atresia in the United States, 1997 to 2000 J Pediatr 148 467 474 10.1016/j.jpeds.2005.12.054 16647406
-
(2006)
J Pediatr
, vol.148
, pp. 467-474
-
-
Shneider, B.L.1
Brown, M.B.2
Haber, B.3
-
33
-
-
69949100743
-
Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia
-
10.1002/lt.21711 19642116
-
L Pape K Olsson C Petersen, et al. 2009 Prognostic value of computerized quantification of liver fibrosis in children with biliary atresia Liver Transpl 15 876 882 10.1002/lt.21711 19642116
-
(2009)
Liver Transpl
, vol.15
, pp. 876-882
-
-
Pape, L.1
Olsson, K.2
Petersen, C.3
-
34
-
-
63649152113
-
The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis
-
10.1016/j.jpedsurg.2008.09.013 19361628
-
JL Santos CO Kieling L Meurer, et al. 2009 The extent of biliary proliferation in liver biopsies from patients with biliary atresia at portoenterostomy is associated with the postoperative prognosis J Pediatr Surg 44 695 701 10.1016/j.jpedsurg.2008.09.013 19361628
-
(2009)
J Pediatr Surg
, vol.44
, pp. 695-701
-
-
Santos, J.L.1
Kieling, C.O.2
Meurer, L.3
-
35
-
-
33745736207
-
The next challenge in pediatric cholestasis: Deciphering the pathogenesis of biliary atresia
-
16819397 1:CAS:528:DC%2BD28XmsVGjtL0%3D
-
JA Bezerra 2006 The next challenge in pediatric cholestasis: deciphering the pathogenesis of biliary atresia J Pediatr Gastroenterol Nutr 43 Suppl 1 S23 S29 16819397 1:CAS:528:DC%2BD28XmsVGjtL0%3D
-
(2006)
J Pediatr Gastroenterol Nutr
, vol.43
, Issue.SUPPL 1
-
-
Bezerra, J.A.1
-
36
-
-
0027158027
-
Reversal of left-right asymmetry: A situs inversus mutation
-
10.1126/science.8480178 8480178 1:CAS:528:DyaK3sXktVaht7w%3D
-
T Yokoyama NG Copeland NA Jenkins, et al. 1993 Reversal of left-right asymmetry: a situs inversus mutation Science 260 679 682 10.1126/science.8480178 8480178 1:CAS:528:DyaK3sXktVaht7w%3D
-
(1993)
Science
, vol.260
, pp. 679-682
-
-
Yokoyama, T.1
Copeland, N.G.2
Jenkins, N.A.3
-
37
-
-
37549045669
-
CFC1 gene involvement in biliary atresia with polysplenia syndrome
-
10.1097/01.mpg.0000304465.60788.f4 18162845 1:CAS:528: DC%2BD2sXhsVOgtr%2FE
-
A Davit-Spraul C Baussan B Hermeziu, et al. 2008 CFC1 gene involvement in biliary atresia with polysplenia syndrome J Pediatr Gastroenterol Nutr 46 111 112 10.1097/01.mpg.0000304465.60788.f4 18162845 1:CAS:528:DC%2BD2sXhsVOgtr%2FE
-
(2008)
J Pediatr Gastroenterol Nutr
, vol.46
, pp. 111-112
-
-
Davit-Spraul, A.1
Baussan, C.2
Hermeziu, B.3
-
38
-
-
75749099706
-
Genetic variation in the vascular endothelial growth factor gene is associated with biliary atresia
-
(Epub ahead of print)
-
Lee HC, Chang TY, Yeung CY, et al.: Genetic variation in the vascular endothelial growth factor gene is associated with biliary atresia. J Clin Gastroenterol 2009 (Epub ahead of print).
-
(2009)
J Clin Gastroenterol
-
-
Lee, H.C.1
Chang, T.Y.2
Yeung, C.Y.3
-
39
-
-
67650246360
-
Sox17 regulates organ lineage segregation of ventral foregut progenitor cells
-
10.1016/j.devcel.2009.05.012 19619492 1:CAS:528:DC%2BD1MXpsV2msbs%3D This article reports the anatomic consequences of under-and overexpression of Sox17, a gene involved in determination of endoderm lineage. Loss of Sox17 in mice resulted in the absence of biliary structures, while the overexpression suppressed pancreatic development and facilitated the expansion of ectopic biliary-like tissue
-
JR Spence AW Lange SC Lin, et al. 2009 Sox17 regulates organ lineage segregation of ventral foregut progenitor cells Dev Cell 17 62 74 10.1016/j.devcel.2009.05.012 19619492 1:CAS:528:DC%2BD1MXpsV2msbs%3D This article reports the anatomic consequences of under-and overexpression of Sox17, a gene involved in determination of endoderm lineage. Loss of Sox17 in mice resulted in the absence of biliary structures, while the overexpression suppressed pancreatic development and facilitated the expansion of ectopic biliary-like tissue
-
(2009)
Dev Cell
, vol.17
, pp. 62-74
-
-
Spence, J.R.1
Lange, A.W.2
Lin, S.C.3
-
40
-
-
63849165695
-
Defective development of the gallbladder and cystic duct in Lgr4-hypomorphic mice
-
10.1002/dvdy.21900 19301403 1:CAS:528:DC%2BD1MXltFynsL0%3D This article reports the absence of the gallbladder and cystic duct in mice carrying the targeted inactivation of the Lgr4 gene. The remainder of the extrahepatic bile ducts was normal
-
R Yamashita Y Takegawa M Sakumoto, et al. 2009 Defective development of the gallbladder and cystic duct in Lgr4-hypomorphic mice Dev Dyn 238 993 1000 10.1002/dvdy.21900 19301403 1:CAS:528:DC%2BD1MXltFynsL0%3D This article reports the absence of the gallbladder and cystic duct in mice carrying the targeted inactivation of the Lgr4 gene. The remainder of the extrahepatic bile ducts was normal
-
(2009)
Dev Dyn
, vol.238
, pp. 993-1000
-
-
Yamashita, R.1
Takegawa, Y.2
Sakumoto, M.3
-
41
-
-
34249794593
-
Maternal microchimerism in biliary atresia
-
discussion 991
-
Kobayashi H, Tamatani T, Tamura T, et al.: Maternal microchimerism in biliary atresia. J Pediatr Surg 2007, 42:987-991; discussion 991.
-
(2007)
J Pediatr Surg
, vol.42
, pp. 987-991
-
-
Kobayashi, H.1
Tamatani, T.2
Tamura, T.3
-
42
-
-
10444234973
-
Maternal microchimerism in the livers of patients with biliary atresia
-
DOI 10.1186/1471-230X-4-14
-
DL Suskind P Rosenthal MB Heyman, et al. 2004 Maternal microchimerism in the livers of patients with biliary atresia BMC Gastroenterol 4 14 10.1186/1471-230X-4-14 15285784 1:CAS:528:DC%2BD2cXns1egsr4%3D (Pubitemid 39637362)
-
(2004)
BMC Gastroenterology
, vol.4
, pp. 14
-
-
Suskind, D.L.1
Rosenthal, P.2
Heyman, M.B.3
Kong, D.4
Magrane, G.5
Baxter-Lowe, L.-A.6
Muench, M.O.7
-
43
-
-
40949131851
-
Maternal microchimerism in underlying pathogenesis of biliary atresia: Quantification and phenotypes of maternal cells in the liver
-
10.1542/peds.2007-0568 18310200
-
T Muraji N Hosaka N Irie, et al. 2008 Maternal microchimerism in underlying pathogenesis of biliary atresia: quantification and phenotypes of maternal cells in the liver Pediatrics 121 517 521 10.1542/peds.2007-0568 18310200
-
(2008)
Pediatrics
, vol.121
, pp. 517-521
-
-
Muraji, T.1
Hosaka, N.2
Irie, N.3
-
44
-
-
36749026566
-
Evidence for the epithelial to mesenchymal transition in biliary atresia fibrosis
-
DOI 10.1016/j.humpath.2007.05.021, PII S0046817707002985
-
R Diaz JW Kim JJ Hui, et al. 2008 Evidence for the epithelial to mesenchymal transition in biliary atresia fibrosis Hum Pathol 39 102 115 10.1016/j.humpath.2007.05.021 17900655 1:CAS:528:DC%2BD2sXhsVSls77O (Pubitemid 350216166)
-
(2008)
Human Pathology
, vol.39
, Issue.1
, pp. 102-115
-
-
Diaz, R.1
Kim, J.W.2
Hui, J.-J.3
Li, Z.4
Swain, G.P.5
Fong, K.S.K.6
Csiszar, K.7
Russo, P.A.8
Rand, E.B.9
Furth, E.E.10
Wells, R.G.11
-
45
-
-
64549142000
-
Epithelial-mesenchymal transition induced by biliary innate immunity contributes to the sclerosing cholangiopathy of biliary atresia
-
10.1002/path.2488 19116990 1:CAS:528:DC%2BD1MXkslWntr4%3D
-
K Harada Y Sato H Ikeda, et al. 2009 Epithelial-mesenchymal transition induced by biliary innate immunity contributes to the sclerosing cholangiopathy of biliary atresia J Pathol 217 654 664 10.1002/path.2488 19116990 1:CAS:528:DC%2BD1MXkslWntr4%3D
-
(2009)
J Pathol
, vol.217
, pp. 654-664
-
-
Harada, K.1
Sato, Y.2
Ikeda, H.3
-
46
-
-
51149113802
-
Cholangiocyte expression of alpha2beta1-integrin confers susceptibility to rotavirus-induced experimental biliary atresia
-
10.1152/ajpgi.00442.2007 18436621 1:CAS:528:DC%2BD1cXoslyhu7c%3D This article reports the increased expression of α2β1-integrin in cholangiocytes using cell culture and in vivo assays. The expression of this integrin was important for the susceptibility of cholangiocytes to rotavirus infection. Blocking of the integrin using specific antibodies minimized symptoms and improved survival in an experimental model of rotavirus-induced biliary atresia
-
M Jafri B Donnelly S Allen, et al. 2008 Cholangiocyte expression of alpha2beta1-integrin confers susceptibility to rotavirus-induced experimental biliary atresia Am J Physiol Gastrointest Liver Physiol 295 G16 G26 10.1152/ajpgi.00442.2007 18436621 1:CAS:528:DC%2BD1cXoslyhu7c%3D This article reports the increased expression of α2β1-integrin in cholangiocytes using cell culture and in vivo assays. The expression of this integrin was important for the susceptibility of cholangiocytes to rotavirus infection. Blocking of the integrin using specific antibodies minimized symptoms and improved survival in an experimental model of rotavirus-induced biliary atresia
-
(2008)
Am J Physiol Gastrointest Liver Physiol
, vol.295
-
-
Jafri, M.1
Donnelly, B.2
Allen, S.3
-
47
-
-
70350041702
-
Cholangiocytes as immune modulators in rotavirus-induced murine biliary atresia
-
10.1111/j.1478-3231.2008.01921.x 19040538 1:CAS:528:DC%2BD1MXhtFGht7nL This article describes the expression of markers of antigen-presenting cells (MHC-I and II, CD40) in cholangiocyte cell lines and freshly isolated cells. However, cultured cholangiocytes were unable to function as competent antigen-presenting cells in T-cell proliferation assays
-
BH Barnes RM Tucker F Wehrmann, et al. 2009 Cholangiocytes as immune modulators in rotavirus-induced murine biliary atresia Liver Int 29 1253 1261 10.1111/j.1478-3231.2008.01921.x 19040538 1:CAS:528:DC%2BD1MXhtFGht7nL This article describes the expression of markers of antigen-presenting cells (MHC-I and II, CD40) in cholangiocyte cell lines and freshly isolated cells. However, cultured cholangiocytes were unable to function as competent antigen-presenting cells in T-cell proliferation assays
-
(2009)
Liver Int
, vol.29
, pp. 1253-1261
-
-
Barnes, B.H.1
Tucker, R.M.2
Wehrmann, F.3
-
48
-
-
68849092071
-
Neonatal NK cells target the mouse duct epithelium via Nkg2d and drive tissue-specific injury in experimental biliary atresia
-
10.1172/JCI38879 19662681 1:CAS:528:DC%2BD1MXpsFClu70%3D The investigators analyzed the population of mononuclear cells in extrahepatic bile ducts of neonatal mice and found that NK cells are the most abundant cells. Using a rotavirus-induced model of biliary atresia, they also found an increase in number and activation status of NK cells, which used the Nkg2d receptor to attach to and kill cholangiocytes. The loss of NK cells or blocking of Nkg2d using antibodies prevented injury to the epithelium of extrahepatic bile ducts, decreased symptoms, and
-
P Shivakumar GE Sabla P Whitington, et al. 2009 Neonatal NK cells target the mouse duct epithelium via Nkg2d and drive tissue-specific injury in experimental biliary atresia J Clin Invest 119 2281 2290 10.1172/JCI38879 19662681 1:CAS:528:DC%2BD1MXpsFClu70%3D The investigators analyzed the population of mononuclear cells in extrahepatic bile ducts of neonatal mice and found that NK cells are the most abundant cells. Using a rotavirus-induced model of biliary atresia, they also found an increase in number and activation status of NK cells, which used the Nkg2d receptor to attach to and kill cholangiocytes. The loss of NK cells or blocking of Nkg2d using antibodies prevented injury to the epithelium of extrahepatic bile ducts, decreased symptoms, and
-
(2009)
J Clin Invest
, vol.119
, pp. 2281-2290
-
-
Shivakumar, P.1
Sabla, G.E.2
Whitington, P.3
-
49
-
-
37749039519
-
Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia
-
10.1002/hep.21873 17935230 1:CAS:528:DC%2BD2sXhsVGiur%2FK This article describes the first prospective, randomized, double-blind trial of corticosteroids following portoenterostomy in infants with biliary atresia. The investigators found no difference in biliary flow or transplant-free survival between infants treated with corticosteroids and those receiving placebo, except for improvement in serum bilirubin when corticosteroids were administered to infants younger than 70 days of age at the time of portoenterostomy
-
M Davenport MD Stringer SA Tizzard, et al. 2007 Randomized, double-blind, placebo-controlled trial of corticosteroids after Kasai portoenterostomy for biliary atresia Hepatology 46 1821 1827 10.1002/hep.21873 17935230 1:CAS:528:DC%2BD2sXhsVGiur%2FK This article describes the first prospective, randomized, double-blind trial of corticosteroids following portoenterostomy in infants with biliary atresia. The investigators found no difference in biliary flow or transplant-free survival between infants treated with corticosteroids and those receiving placebo, except for improvement in serum bilirubin when corticosteroids were administered to infants younger than 70 days of age at the time of portoenterostomy
-
(2007)
Hepatology
, vol.46
, pp. 1821-1827
-
-
Davenport, M.1
Stringer, M.D.2
Tizzard, S.A.3
-
50
-
-
50849107342
-
Wong K, Cheun Leung Lan L, et al.: Evaluation of a standardized protocol in the use of steroids after Kasai operation
-
10.1007/s00383-008-2200-2 18679691
-
HY Chung 2008 Wong K, Cheun Leung Lan L, et al.: Evaluation of a standardized protocol in the use of steroids after Kasai operation Pediatr Surg Int 24 1001 1004 10.1007/s00383-008-2200-2 18679691
-
(2008)
Pediatr Surg Int
, vol.24
, pp. 1001-1004
-
-
Chung, H.Y.1
-
51
-
-
40949118564
-
Postoperative high-dose steroids do not improve mid-term survival with native liver in biliary atresia
-
DOI 10.1111/j.1572-0241.2007.01721.x
-
C Petersen D Harder M Melter, et al. 2008 Postoperative high-dose steroids do not improve mid-term survival with native liver in biliary atresia Am J Gastroenterol 103 712 719 10.1111/j.1572-0241.2007.01721.x 18177447 1:CAS:528:DC%2BD1cXkslKmtbg%3D (Pubitemid 351406001)
-
(2008)
American Journal of Gastroenterology
, vol.103
, Issue.3
, pp. 712-719
-
-
Petersen, C.1
Harder, D.2
Melter, M.3
Becker, T.4
Wasielewski, R.V.5
Leonhardt, J.6
Ure, B.M.7
-
52
-
-
58249091189
-
Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia
-
10.1542/peds.2008-0986 19029197
-
S Willot S Uhlen L Michaud, et al. 2008 Effect of ursodeoxycholic acid on liver function in children after successful surgery for biliary atresia Pediatrics 122 e1236 e1241 10.1542/peds.2008-0986 19029197
-
(2008)
Pediatrics
, vol.122
-
-
Willot, S.1
Uhlen, S.2
Michaud, L.3
|