Living Donor Liver Transplantation for Progressive Familial Intrahepatic Cholestasis Type 1: Two Reported Cases.


BACKGROUND:Progressive familial intrahepatic cholestasis type 1 (PFIC1) is an inherited disease characterized by cholestatic features. We report two patients with PFIC1 who underwent liver retransplantation. CASE REPORT:One patient was a 3-year-old female who underwent liver transplantation for PFIC1. She presented with severe diarrhea and fatty liver, and went into liver failure. She therefore underwent liver retransplantation and external biliary diversion 8 years after the initial liver transplantation. The explanted liver was histologically diagnosed with chronic rejection. Her intractable diarrhea stopped after the retransplantation. She was diagnosed with a fatty liver 8 months after the retransplantation and died 4 years after retransplantation due to bleeding from an ileostomy. The other patient was a 3-year-old male. This patient underwent liver retransplantation due to liver cirrhosis caused by steatohepatitis 9 years after the initial liver transplantation. The biliary tract was not diverted. He also experienced severe diarrhea after the retransplantation and requires home parenteral nutrition due to an eating disorder. CONCLUSIONS:Liver transplantation is the only treatment to resolve life-threatening issues due to PFIC1, but requires further improvement as a therapeutic modality.


Transplant Proc


Oya Y,Sugawara Y,Honda M,Yoshii D,Isono K,Hayashida S,Yamamoto H,Inomata Y




Has Abstract


2017-06-01 00:00:00














  • Effective anatomic reconstruction of the middle hepatic vein in modified right lobe graft living donor liver transplantation.

    abstract:INTRODUCTION:Adult liver transplantation using the right lobe graft without a middle hepatic vein (MHV; modified right lobe graft) has widely been used to compensate for the cadaveric organ shortage. To provide appropriate functional graft volume in the right lobe graft used for living donor liver transplantation (LDLT...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Kim BW,Park YK,Paik OJ,Lee BM,Wang HJ,Kim MW

    更新日期:2007-12-01 00:00:00

  • Contraceptive Choices and Counseling in Norwegian Female Renal Transplant Recipients.

    abstract:BACKGROUND:There are major gaps in the understanding of sexual and reproductive health in female renal transplant recipients. METHODS:In this Norwegian multicenter retrospective observational study, 118 female renal transplant recipients aged 22 to 49 years responded to a questionnaire on fertility, contraceptive use,...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章,多中心研究


    authors: Eide IA,Rashidi F,Lønning K,Oldereid NB,Reisæter AV,Åsberg A,Midtvedt K

    更新日期:2019-03-01 00:00:00

  • Improved method of porcine pancreas procurement with arterial flush and ductal injection enhances islet isolation outcome.

    abstract:BACKGROUND:Several pancreas procurement procedures have been used for porcine islet isolation; however, their impact on outcomes has not been extensively studied. We evaluated an advanced procurement technique for porcine islet isolation designed to reduce warm ischemia, to remove blood content, and enhance cooling of ...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Anazawa T,Balamurugan AN,Papas KK,Avgoustiniatos ES,Ferrer J,Matsumoto S,Sutherland DE,Hering BJ

    更新日期:2010-07-01 00:00:00

  • Successful Ultrasound-Guided Percutaneous Drainage of Multiple Splenic Abscesses in a Kidney Transplant Patient With Encapsulated Sclerosing Peritonitis: A Case Report.

    abstract::Differential diagnosis of post-transplant infections should include rare/uncommon foci and pathogens. We present a rare case of life-threatening infection, a splenic abscess in a 53-year-old woman who was transplanted with a cadaveric kidney 5 months previously. The patient was admitted to our clinic with chills, shiv...

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    pub_type: 杂志文章


    authors: Tuğcu M,Ruhi Ç,Boynueğri B,Kasapoğlu U,Can Ö,Kiliçoğlu G,Şahin G,Titiz M

    更新日期:2015-06-01 00:00:00

  • A Retrospective Evaluation of 728 Kidney Donors Who Applied for Organ Donation.

    abstract:OBJECTIVE:The aim of this study was to determine the sociodemographic characteristics of people who applied to be kidney donors at an organ transplantation center. MATERIAL AND METHODS:A total of 728 participants in the kidney donor program were included in the study between 2015 and 2018 at Istanbul Yeniyüzyıl Univer...

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    pub_type: 杂志文章


    authors: Bozkurt O,Erdoğan B,Uyar M

    更新日期:2019-05-01 00:00:00

  • Preliminary Study of Bone Marrow-Derived Mesenchymal Stem Cells Pretreatment With Erythropoietin in Preventing Acute Rejection After Rat Renal Transplantation.

    abstract::Renal transplantation is currently the most effective treatment for end-stage kidney diseases, and acute allograft rejection is well treated with high dose prednisolone and immunosuppressive agents, but long-term use of immunosuppressants will be detrimental to the long-term survival of the transplanted kidney and wil...

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    authors: Zhang Y,Zhou S,Hu JM,Chen H,Liu D,Li M,Guo Y,Fan LP,Li LY,Liu YG,Zhao M

    更新日期:2018-12-01 00:00:00

  • Efficacy of intravenous immunoglobulin in the treatment of persistent BK viremia and BK virus nephropathy in renal transplant recipients.

    abstract::BK virus-associated nephropathy (BKVN) can cause clinically significant viral infection in renal transplant recipients, leading to allograft dysfunction and loss. The usual management of BKVN involves the reduction of immunosuppression and the addition of leflunomide, quinolones, and cidofovir, but the rate of graft l...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Vu D,Shah T,Ansari J,Naraghi R,Min D

    更新日期:2015-03-01 00:00:00

  • Computed Tomography as Primary Screening for Appraisal of Pulmonary Small Nodules in Liver Transplant Candidates.

    abstract:BACKGROUND:Chest computed tomography (CT) as a primary screening method in candidates for living donor liver transplantation (LDLT) is not yet a standardized procedure. The aim of this study is to present our methods and result of evaluation of pulmonary small nodules (PSN) after CT as a primary screening tool. PATIEN...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Wu YJ,Lin CC,Chang YM,Wang SH,Lin YH,Lu HI,Concejero AM,Chen CL,Lin TL

    更新日期:2016-05-01 00:00:00

  • Initial clinical effect of intraportal insulin administration on liver graft regeneration in adult patients underwent living donor right lobe liver transplantation.

    abstract:OBJECTIVE:Insulin is one factor responsible for hepatotrophic regeneration in animal models. This study assessed the clinical effects of intraportal administration of insulin on liver graft regeneration in adult patients undergoing right lobe living donor liver transplantation (LDLT). METHODS:Between July 2005 and Sep...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Xu MQ,Yan LN,Li B,Wen TF,Zeng Y,Zhao JC,Wang WT,Yang JY,Ma YK,Chen ZY,Zhang ZW

    更新日期:2009-06-01 00:00:00

  • Optimized technique in needle biopsy protocol shown to be of greater sensitivity and accuracy compared to wedge biopsy.

    abstract:BACKGROUND:Kidney biopsy is an important tool in determining allograft suitability for transplantation. Most deceased-donor renal biopsies performed today in the United States are wedge biopsies (WBs), with core needle biopsies being performed only by a minority of organ procurement organizations (OPOs). The lack of a ...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Yushkov Y,Dikman S,Alvarez-Casas J,Giudice A,Hoffman A,Goldstein MJ

    更新日期:2010-09-01 00:00:00

  • Molecular Adsorbent Recirculating System Treatment Can Reduce Blood Levels of N-Acetylcysteine in Patients With Acetaminophen Overdose: Case Reports.

    abstract:BACKGROUND:Acetaminophen poisoning continues to be a major cause of liver failure that can lead to liver transplantation. N-acetylcysteine (NAC) is the cornerstone of treatment. Some authors use a Molecular Adsorbent Recirculating System (MARS) system in acetaminophen poisoning. It is reported that the MARS system elim...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Lopez-Lopez V,Ros J,Ferreras D,Sanmartin J,Martinez M,Pons Miñano JA,Sanchez-Bueno F,Robles-Campos R,Ramirez-Romero P,Parrilla-Paricio P

    更新日期:2018-03-01 00:00:00

  • Do we have to treat subclinical rejections in early protocol renal allograft biopsies?

    abstract::The aim of the present study was to evaluate whether treatment of subclinical, borderline rejections (SR/BR) or histological findings of chronic allograft nephropathy (CAN) in protocol biopsies in the first month posttransplantation after living related kidney transplantation has a beneficial effect on graft histology...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Masin-Spasovska J,Spasovski G,Dzikova S,Petrusevska G,Lekovski Lj,Ivanovski N,Popov Z

    更新日期:2007-10-01 00:00:00

  • Quality of life of living kidney donors: a single-center experience.

    abstract::Renal transplantation improves the quality of life (QoL) of patients with end-stage renal disease. The preservation of QoL of living kidney donors is paramount. The aim of this study was to assess the QoL pre- and postdonation using Medical Outcome Survey Short Form-36 (SF-36) and to compare with a control group of po...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Shrestha A,Shrestha A,Vallance C,McKane WS,Shrestha BM,Raftery AT

    更新日期:2008-06-01 00:00:00

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    pub_type: 杂志文章


    authors: Interewicz B,Olszewski WL,Stanislawska J,Sikora M,Durlik M,Maksymowicz M

    更新日期:2005-01-01 00:00:00

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    journal_title:Transplantation proceedings

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    authors: Server S,Sabet S,Sahin T,Guven K,Aydın U,Tokat Y,Inan N

    更新日期:2019-09-01 00:00:00

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    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Oh CK,Lee BM,Kim H,Kim SI,Kim YS

    更新日期:2008-09-01 00:00:00

  • Taurine protects against ischemia-reperfusion injury in rabbit livers.

    abstract::Ischemia-reperfusion injury is of major clinical relevance during liver transplantation. In the present study, the therapeutic efficacy of taurine to protect against ischemia-reperfusion injury was investigated in rabbit livers. We demonstrated that intravenous injection of taurine attenuated liver dysfunction as evid...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Tong L,Li J,Qiao H,Jiang H,Meng F,Sun X

    更新日期:2006-06-01 00:00:00

  • Liver ischemic preconditioning: a new strategy for the prevention of ischemia-reperfusion injury.

    abstract::Ischemic preconditioning renders the liver more tolerant to ischemia-reperfusion injury in warm and cold ischemia-reperfusion models. In general, the application of a 5 to 10-minute period of ischemia followed by 10 minutes of reperfusion confers early effective protection to the liver. Mechanisms responsible for this...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章,评审


    authors: Peralta C,Serafin A,Fernández-Zabalegui L,Wu ZY,Roselló-Catafau J

    更新日期:2003-08-01 00:00:00

  • Pericardial Effusion Associated With Sirolimus Use After Renal Transplantation: A Single-Center Case Series.

    abstract::Pericardial effusion and cardiac tamponade following renal transplantation have been recognized as a potentially serious complications associated with the use of sirolimus for immunosuppression. Our study aims to analyze the development of sirolimus-associated pericardial effusion. Patients who underwent renal transpl...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Lopez-Soler RI,Patel SK,Lyubarova R,Pashkovetsky E,Wang S,Schuster D,Chandolias N,Conti D

    更新日期:2019-01-01 00:00:00

  • Tacrolimus and Mycophenolic Acid Blood Concentration and Cellular Rejection After Heart Transplantation in First Endomyocardial Biopsy.

    abstract:BACKGROUND:Tacrolimus and mycophenolic acid (MPA) are the most important immunosuppressive drugs in modern heart transplantation. The pharmacokinetics of tacrolimus are best described by a 2-compartment model. MPA has very variable pharmacokinetics. The aim of this research was to compare kinetics of the immunosuppress...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Nadziakiewicz P,Grochla M,Krauchuk A,Szyguła-Jurkiewicz B,Lorek J,Barańska A,Zembala MO,Przybyłowski P

    更新日期:2020-09-01 00:00:00

  • C0h/C2h monitoring of the pharmacodynamics of cyclosporin plus mycophenolate mofetil in human heart transplant recipients.

    abstract:UNLABELLED:Pharmacokinetic (PK) parameters like C2h have improved efficacy of immunosuppressive therapy. However, drug interactions, toxicities, and individual differences to drug effects still remain challenging. Therefore, this study was designed to assess pharmacodynamic (PD) effects of the combination cyclosporin (...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Barten MJ,Rahmel A,Garbade J,Richter M,Bittner HB,Dhein S,Mohr FW,Gummert JF

    更新日期:2005-03-01 00:00:00

  • Semen collection requirement from multiple-organs brain-dead donors: report of two cases and analysis of the available Brazilian legislation.

    abstract::Along with developments in transplantation there have been major breakthroughs in the techniques of assisted reproduction. The areas of common interest include requesting semen collection from organ donors with a diagnosis of brain death. After the recent report of two cases in the Search Service of Organs and Tissues...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Sardinha LA,Dantas Filho VP,Montone EB,Athayde MV,Rodrigues SL,Panunto MR,Araújo S,Zambelli HJ

    更新日期:2013-04-01 00:00:00

  • C/D Ratio in Long-Term Renal Function.

    abstract:BACKGROUND/AIMS:Tacrolimus is an immunosuppressive drug. Its C0 concentration, commonly used for monitoring, does not always correspond to its pharmacologic effect. Thölking et al developed an indicator, the C/D ratio, that describes the drug's metabolism rate. Our purpose was to determine whether the points dividing t...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Kwiatkowska E,Kwiatkowski S,Wahler F,Gryczman M,Domańki L,Marchelk-Myśliwiec M,Ciechanowski K,Drozd-Dabrowska M

    更新日期:2019-12-01 00:00:00

  • Experience with Alemtuzumab (Campath-1H) as induction agent in renal transplantation followed by steroid-free immunosuppression.

    abstract:BACKGROUND:The purpose of this study was to describe the initial experience with alemtuzumab as induction followed by steroid-free immunosuppression in kidney transplantation. METHODS:One hundred patients who received renal transplants from living and deceased donors were followed for a median period of 12 months (ran...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Baez Y,Giron F,Niño-Murcia A,Rodríguez J,Salcedo S

    更新日期:2008-04-01 00:00:00

  • Kidney dimensions at sonography are correlated with glomerular filtration rate in renal transplant recipients and in kidney donors.

    abstract::The gold standard to assess renal function is the measurement of glomerular filtration rate (GFR). For practical reasons, renal function is often evaluated from serum creatinine (S Cr) or cystatin C (S Cys), and GFR is predicted from SCr. Ultrasound scanning of the kidneys is used only to evaluate renal morphology. Th...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Paleologo G,Abdelkawy H,Barsotti M,Basha A,Bernabini G,Bianchi A,Caprio F,Emad A,Grassi G,Nerucci B,Tregnaghi C,Rizzo G,Donadio C

    更新日期:2007-07-01 00:00:00

  • Calcineurin-free protocols with basiliximab induction allow patients included in "old to old" programs achieve standard kidney transplant function.

    abstract:INTRODUCTION:The EuroTransplant "old to old" program establishes that patients older than 60 years can receive offers of organs from donors older than 60 years. The compromised function of these organs makes it a priority to preserve their initial kidney function. HYPOTHESIS:Calcineurin-sparing protocols using anti-IL...

    journal_title:Transplantation proceedings

    pub_type: 临床试验,杂志文章


    authors: Emparan C,Laukötter M,Wolters H,Dame C,Heidenreich S,Senninger N

    更新日期:2003-06-01 00:00:00

  • Vascularized anal autotransplantation model in rats: preliminary report.

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    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Araki J,Mihara M,Narushima M,Iida T,Sato T,Koshima I

    更新日期:2011-11-01 00:00:00

  • Disseminated nocardiosis: a rare infectious complication following non-heart-beating donor liver transplantation.

    abstract::Nocardiosis is an infrequent disease that affects patients who display a cellular immunodeficiency, such as transplant recipients on immunosuppressive treatment, but uncommonly associated with high morbidity and mortality rates. Disseminated Nocardiosis affecting the central nervous system (CNS), abdomen, skin, and lu...

    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Jimenez-Galanes Marchan S,Meneu Díaz JC,Caso Maestro O,Perez Saborido B,Moreno Elola-Olaso A,Abradelo Usera M,Fundora Suarez Y,Gimeno Calvo A,Moreno Molinero V,Garcia Reyne A,Barra Valencia V,San Juan Garrido R,Moreno Gonzalez E

    更新日期:2009-07-01 00:00:00

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    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Osorio JM,Pérez Marfil A,Ferreyra C,Pérez Abud R,Ruiz Fuentes MC,Galindo P,de Gracia MC,Osuna A

    更新日期:2012-09-01 00:00:00

  • Impact of Secondary Insults in Brain Death After Traumatic Brain Injury.

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    journal_title:Transplantation proceedings

    pub_type: 杂志文章


    authors: Heppekcan D,Ekin S,Çivi M,Aydın Tok D

    更新日期:2019-09-01 00:00:00