Abstract:
:Transplantation for Wilson's disease occupies 1/3 of the cases for metabolic diseases in Japan. At the end of 2009, 109 transplantations had been performed including three deceased donor cases in the Japanese registry. We herein discuss problems of transplantation for Wilson's disease as well as its indication, timing, and social care. We retrospectively reviewed four fulminant cases and two chronic cases who underwent living donor liver transplantation. There were two boys and two girls. Four adolescents of average age 11.3 years underwent living donor liver transplantation. Duration from onset to transplantation ranged from 10 to 23 days. Average Model for End-stage Liver Disease (MELD) score was 27.8 (range=24-31). All patients were administrated chelates prior to transplantation. MELD, New Wilson's index, Japanese scoring for liver transplantation, and liver atrophy were useful tools for transplantation decision making; however, none of them was an independent decisive tool. Clinical courses after transplantation were almost uneventful. One girl, however, developed an acute rejection episode due to noncompliance at 3 years after transplantation. All patients currently survive without a graft loss. No disease recurrence had been noted even using living related donors. Two adults evaluated for liver transplantation were listed for deceased donor liver transplantation. Both candidates developed cirrhosis despite long-term medical treatment. There were no appropriate living donors for them. There are many problems in transplantation for Wilson's disease. The indications for liver transplantation should be considered individually using some decision-making tools. The safety of the living donor should be paid the most attention.
journal_name
Transplant Procjournal_title
Transplantation proceedingsauthors
Narumi S,Umehara M,Toyoki Y,Ishido K,Kudo D,Kimura N,Kobayashi T,Sugai M,Hakamada Kdoi
10.1016/j.transproceed.2012.01.024subject
Has Abstractpub_date
2012-03-01 00:00:00pages
478-80issue
2eissn
0041-1345issn
1873-2623pii
S0041-1345(12)00058-9journal_volume
44pub_type
杂志文章abstract:BACKGROUND:Islet allograft rejection in sensitized recipients is difficult to control by costimulation blockade using anti-CD154 and cytotoxic T-lymphocyte antigen-4 immunoglobulin (CTLA4Ig). Because leukocyte function antigen (LFA) 1 is highly expressed on memory T cells, adding an LFA-1 blockade may inhibit memory T-...
journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2010.05.084
更新日期:2010-07-01 00:00:00
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pub_type: 杂志文章
doi:10.1016/j.transproceed.2010.09.001
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abstract:OBJECTIVES:The human major histocompatibility complex class I chain-related antigen A, B (MICA, B) are believed to be "cell stress sensors," which encode stress-inducible surface proteins that bind to NKG2D, an activating receptor of NK, alpha beta and gamma delta T cells. While cyclosporine (CsA) has improved patient ...
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doi:10.1016/j.transproceed.2006.06.080
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2012.10.065
更新日期:2013-06-01 00:00:00
abstract::Liver retransplantation carries a significantly higher morbidity and mortality compared with patients after single transplantations. The aim of this study was to review our outcomes in liver retransplantations. From February 1984 to February 2007, 409 liver transplantations were performed on 396 patients, including 13...
journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2008.07.067
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pub_type: 杂志文章
doi:10.1016/j.transproceed.2014.01.015
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2013.02.122
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2009.06.126
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2013.11.007
更新日期:2013-01-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2012.07.081
更新日期:2012-09-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2009.09.006
更新日期:2009-10-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 临床试验,杂志文章
doi:10.1016/j.transproceed.2005.01.043
更新日期:2005-03-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 临床试验,杂志文章
doi:10.1016/j.transproceed.2004.09.016
更新日期:2005-04-01 00:00:00
abstract::Tumor load is often underdiagnosed on radiological examination previous to liver transplantation (LT) for hepatocarcinoma (CHC). Thus, post-liver transplant explant analysis is required following transplantation to assess the risk of the recurrence of CHC. The objectives were to compare the characteristics of CHC on p...
journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2018.03.132
更新日期:2019-01-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2019.03.027
更新日期:2019-01-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/s0041-1345(03)00358-0
更新日期:2003-06-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2007.11.035
更新日期:2008-01-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2009.12.029
更新日期:2010-01-01 00:00:00
abstract::The studies discussed in this presentation have shown that CD8 T lymphocytes have a primary role in conferring resistance to MCMV in the immunocompetent BALB/c mouse. In the presence of selective CD8 deficiency, however, other mechanisms, apparently mediated by the CD4 T-cell subset, contribute in a compensatory fashi...
journal_title:Transplantation proceedings
pub_type: 杂志文章,评审
doi:
更新日期:1991-06-01 00:00:00
abstract:OBJECTIVE:The goal of this article is to present the experience of a new liver transplant team. MATERIALS AND METHODS:This review includes all patients who received a liver transplant between March 15, 2000 and March 15, 2003. RESULTS:We performed 87 transplantations on 84 patients; 39 were females and 45 were males ...
journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2004.06.020
更新日期:2004-07-01 00:00:00
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pub_type: 杂志文章,多中心研究
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更新日期:2007-01-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2011.05.048
更新日期:2011-09-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2017.12.006
更新日期:2018-01-01 00:00:00
abstract::The mechanism of the immunosuppressive effect of CyA and FK 506 can be monitored in vivo in humans. The picture emerging is of a close relationship between drug concentrations and CN inhibition. But many puzzles of the drugs remain. What is the role of CyA in the activation of transforming growth factor-beta (TGF-beta...
journal_title:Transplantation proceedings
pub_type: 杂志文章,评审
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更新日期:1998-08-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2008.11.011
更新日期:2009-06-01 00:00:00
abstract::Perfusion parameters (PP) and early transplant outcome data from 332 consecutive ECD type kidneys machine preserved on the Waters RM-3 apparatus were reviewed and analyzed to examine the validity of using suboptimal PPs (renal resistance of .41-.60) as a criterion for discarding kidneys. Overall discard rate was 23.5%...
journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2005.01.058
更新日期:2005-01-01 00:00:00
abstract::Cyclosporine elimination in a regimen including sirolimus has been shown to be a safe and effective approach to improve graft function. Nevertheless, it is still unknown whether the functional benefit of CyA withdrawal coincides with a subsequent reduction in histologic lesions of chronic damage or development of chro...
journal_title:Transplantation proceedings
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1016/s0041-1345(03)00612-2
更新日期:2003-08-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章,评审
doi:10.1016/j.transproceed.2012.05.041
更新日期:2012-07-01 00:00:00
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journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2009.09.065
更新日期:2009-12-01 00:00:00
abstract:OBJECTIVE:Persistent hyperparathyroidism (HPT) with hypercalcemia and hypophosphatemia is common after renal transplantation, resulting in the need for parathyroidectomy. Cinacalcet may be a therapeutic option for these patients. We sought to analyze the efficacy of treatment with cinacalcet for patients with hypercalc...
journal_title:Transplantation proceedings
pub_type: 杂志文章
doi:10.1016/j.transproceed.2009.06.055
更新日期:2009-07-01 00:00:00