RELAPSING FEATURES OF BILE SALT EXPORT PUMP (BSEP) DEFICIENCY IN A PATIENT SUCCESSFULLY TRANSPLANTED FOR PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS TYPE 2 (PFIC2)

被引:0
|
作者
Centenari, C. [1 ]
Fabre, M. [2 ]
Sciveres, M. [3 ]
Jacquemin, E. [4 ]
Maggiore, G. [1 ]
机构
[1] Azienda Osped Univ Pisana, Clin Pediat IIa Gastroenterol & Epatol, Pisa, Italy
[2] Univ Paris Sud 11, Univ Hosp Bicetre, AP HP, Dept Pathol, Paris, France
[3] UPMC Ismett, Pediat Hepatol & Pediat Liver Transplantat, Palermo, Italy
[4] Univ Paris Sud 11, Univ Hosp Bicetre, AP HP, Pediat Hepatol, Paris, France
关键词
D O I
10.1016/S1590-8658(10)60561-0
中图分类号
R57 [消化系及腹部疾病];
学科分类号
摘要
CO18
引用
收藏
页码:S329 / S329
页数:1
相关论文
共 48 条
  • [21] PATERNAL ISODISOMY FOR CHROMOSOME 2 AS CAUSE OF BILE SALT EXPORT PUMP (BSEP) DEFICIENCY
    Giovannoni, I.
    Terracciano, A.
    David, E.
    Francalanci, P.
    Gennari, F.
    Santorelli, F. M.
    JOURNAL OF HEPATOLOGY, 2009, 50 : S105 - S105
  • [22] MORPHOLOGY OF TRANSPLANTED LIVER IN RECURRENT PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS TYPE 2
    Iljinsky, I. M.
    Mozheiko, N. P.
    Tsirulnikova, O. M.
    VESTNIK TRANSPLANTOLOGII I ISKUSSTVENNYH ORGANOV, 2020, 22 (04): : 192 - 196
  • [23] Mutations in bile salt export pump (ABCB11) in two children with progressive familial intrahepatic cholestasis and cholangiocarcinoma
    Scheimann, A. O.
    Strautnieks, S. S.
    Knisely, A. S.
    Byrne, J. A.
    Thompson, R. J.
    Finegold, M. J.
    JOURNAL OF PEDIATRICS, 2007, 150 (05): : 556 - 559
  • [24] Human iPSC-derived hepatocyte-like cells generated from patients with bile salt export pump deficiency recapitulate the phenotype of progressive familial intrahepatic cholestasis type 2
    Imagawa, Kazuo
    Takayama, Kazuo
    Isoyama, Shigemi
    Tanikawa, Ken
    Shinkai, Masato
    Kage, Masayoshi
    Kawabata, Kenji
    Sumazaki, Ryo
    Mizuguchi, Hiroyuki
    HEPATOLOGY, 2015, 62 : 1032A - 1033A
  • [25] A Rare BSEP Mutation Associated with a Mild Form of Progressive Familial Intrahepatic Cholestasis Type 2
    Waisbourd-Zinman, Orith
    Surrey, Lea F.
    Schwartz, Anna E.
    Russo, Pierre A.
    Wen, Jessica
    ANNALS OF HEPATOLOGY, 2017, 16 (03) : 465 - 468
  • [26] Progressive Familial Intrahepatic Cholestasis (PFIC) Type 1, 2, and 3: A Review of the Liver Pathology Findings
    Morotti, Raffaella A.
    Suchy, Frederick J.
    Magid, Margret S.
    SEMINARS IN LIVER DISEASE, 2011, 31 (01) : 3 - 10
  • [27] PLASMAPHARESIS, INTRAVENOUS IMMUNOGLOBULIN AND RITUXIMAB SUCCESSFULLY TREAT RECURRENT PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS TYPE 2 (PFIC-2) AFTER LIVER TRANSPLANTATION
    Shagrani, Mohammad
    Abaalkhail, Faisal
    Al Sebayel, Mohammad
    Alhussaini, Hessah
    Broring, Dieter
    Elsiesy, Hussien
    TRANSPLANT INTERNATIONAL, 2015, 28 : 596 - 596
  • [28] Low serum concentrations of γGT activity in progressive familial intrahepatic cholestasis, evidence for different mechanisms in PFIC, type I (FIC1 disease), and in PFIC, type 2 (BSEP disease).
    Hanigan, MH
    Bull, LN
    Strautnieks, SS
    Thompson, RJ
    Bove, KE
    Dahms, BB
    Finegold, MJ
    Hart, J
    Jaffe, R
    Roskams, T
    Portmann, BC
    Knisely, AS
    HEPATOLOGY, 2002, 36 (04) : 310A - 310A
  • [29] Plasmapharesis, Intravenous Immunoglobulin and Rituximab Successfully Treat Recurrent Progressive Familial Intrahepatic Cholestasis Type 2 (PFIC-2) After Liver Transplantation
    Elsiesy, H.
    Abaalkhail, F.
    Alhamoudi, W.
    Alhussaini, H.
    Alsebayel, M.
    Broring, D.
    Shagrani, M.
    AMERICAN JOURNAL OF TRANSPLANTATION, 2015, 15
  • [30] In vitro and in vivo effects of chaperone therapy with 4-phenylbutyrate on ABCB11 missense mutations involved in progressive familial intrahepatic cholestasis type 2 (PFIC2)
    Gonzales, Emmanuel
    Grosse, Brigitte
    Davit-Spraul, Anne
    Schuller, Brice
    Fabre, Monique
    Cassio, Doris
    Jacquemin, Emmanuel
    HEPATOLOGY, 2012, 56 : 204A - 204A