Successful liver transplantation of two brothers with crigler-najjar syndrome type 1 using a single cadaveric organ.

Abstract:

:Crigler-Najjar type 1 disease (CNS1) is the result of a genetic defect, leading to complete functional loss of an enzyme which glucuronidates bilirubin. As a consequence, unconjugated bilirubin accumulates and may cause kernicterus, the most serious complication in adolescents. Phototherapy effectively adjusts bilirubin levels less than critical concentrations over many years but become less effective in elder children. Therefore, liver transplantation must be performed as definite therapy in these patients to avoid irreversible neurological deficits. Two brothers with CNS1, one without neurological deficits and one with moderate brain injury underwent orthotopic split liver transplantation from the same donor. The intra- and postoperative course of both patients was uneventful. Bilirubin levels normalized after transplantation in both recipients. Furthermore, mental and physical development considerably improved upon transplantation in the brother with neurological dysfunction. Therefore, orthotopic liver transplantation in CNS1 patients should be performed early enough to avoid irreversible brain damage, i.e., as a prophylactic procedure.

journal_name

Transplantation

journal_title

Transplantation

authors

Schauer R,Lang T,Zimmermann A,Stangl M,Da Silva L,Schildberg FW,Rau HG

doi

10.1097/00007890-200201150-00012

subject

Has Abstract

pub_date

2002-01-15 00:00:00

pages

67-9

issue

1

eissn

0041-1337

issn

1534-6080

journal_volume

73

pub_type

杂志文章
  • Fragility of the electron transport chain and superoxide generation in mitochondria of the liver graft after cold ischemia.

    abstract:BACKGROUND:After cold ischemia, electrons transferred in the electron transport chain may leak out of the mitochondria in proportion to the deterioration of mitochondrial oxidative phosphorylation. This seems to be one major cause of the lipid peroxidation that occurs mainly in the hepatocytes at reperfusion in liver t...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-199904270-00015

    authors: Ohkohchi N,Endoh T,Oikawa K,Seya K,Satomi S

    更新日期:1999-04-27 00:00:00

  • Effect of FK506 treatment on allocytolytic T lymphocyte induction in vivo: differential effects of FK506 on L3T4+ and Ly2+ T cells.

    abstract::Although FK506 has been widely investigated as a potent suppressor of organ allograft rejection in animals, little is known about the effect of FK506 on T cell responses to allografts in vivo. In the present study, we have studied the effect of FK506 on the induction of allocytolytic T lymphocyte using mice primed wit...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-199008000-00021

    authors: Maruyama M,Suzuki H,Yamashita N,Yano S

    更新日期:1990-08-01 00:00:00

  • Nutritional markers in liver allograft recipients.

    abstract:BACKGROUND:Malnutrition is common in patients with end-stage liver disease considered for transplantation, but it is unclear whether this affects the outcome after transplantation. AIM.: To determine whether the severity of malnutrition in liver transplant candidates affects outcome after transplantation. METHODS:We d...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/01.tp.0000150022.64564.c2

    authors: Shahid M,Johnson J,Nightingale P,Neuberger J

    更新日期:2005-02-15 00:00:00

  • Improved outcomes of combined liver and kidney transplants in small children (

    abstract:BACKGROUND:Combined liver and kidney transplantation (CLKT) is a surgical challenge in small children because of technical aspects, lack of pediatric donors, and restrictions related to the size of the abdominal cavity. We report outcomes after CLKT in this challenging group of smaller children. METHOD:A review of pro...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/TP.0b013e3181b29f0c

    authors: Perera MT,Silva MA,Sharif K,McKiernan PJ,Kelly DA,Lloyd C,Milford DV,Mayer DA,Mirza DF

    更新日期:2009-09-15 00:00:00

  • Donation after circulatory death: current practices, ongoing challenges, and potential improvements.

    abstract::Organ donation after circulatory death (DCD) has been endorsed by the World Health Organization and is practiced worldwide. This overview examines current DCD practices, identifies problems and challenges, and suggests clinical strategies for possible improvement. Although there is uniform agreement on DCD donor candi...

    journal_title:Transplantation

    pub_type: 杂志文章,评审

    doi:10.1097/01.TP.0000437178.48174.db

    authors: Morrissey PE,Monaco AP

    更新日期:2014-02-15 00:00:00

  • Cytomegalovirus serostatus pairing and deceased donor kidney transplant outcomes in adult recipients with antiviral prophylaxis.

    abstract:BACKGROUND:The objectives of this study are to investigate the impact of cytomegalovirus (CMV) donor (D)/recipient (R) serostatus on kidney transplant outcomes in recipients who received CMV prophylaxis and to investigate the association of individual antiviral agents (acyclovir, ganciclovir, and valganciclovir) with o...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/TP.0b013e3181f7c053

    authors: Kuo HT,Ye X,Sampaio MS,Reddy P,Bunnapradist S

    更新日期:2010-11-27 00:00:00

  • Differential effect of cyclosporine in vivo on the distribution of T cell subsets in the thymus, spleen, and lymph nodes.

    abstract::Cyclosporine treatment of BALB/c mice (at a dose of 20 mg/kg every other day for 3 weeks) caused a remarkable reduction in the PNA-, L3T4+Lyt-2- subset of thymocytes. A significant reduction of the L3T4+Lyt-2-subset was also observed in both the lymph node and spleen cells of CsA-treated mice, though the degree of the...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-198903000-00020

    authors: Hiramine C,Hojo K,Matsumoto H,Koseto M,Itoh M

    更新日期:1989-03-01 00:00:00

  • Pregnancy outcome after cyclosporine therapy during pregnancy: a meta-analysis.

    abstract:BACKGROUND:Cyclosporine (CsA) therapy must often be continued during pregnancy to maintain maternal health in such conditions as organ transplantation and autoimmune disease. This meta-analysis was performed to determine whether CsA exposure during pregnancy is associated with an increased risk of congenital malformati...

    journal_title:Transplantation

    pub_type: 杂志文章,meta分析

    doi:10.1097/00007890-200104270-00006

    authors: Bar Oz B,Hackman R,Einarson T,Koren G

    更新日期:2001-04-27 00:00:00

  • Levosimendan treatment after primary organ failure in heart transplantation: a direct way to recovery?

    abstract::Heart transplantation is considered nowadays the gold standard in the therapy of chronic and terminal heart insufficiency. Primary organ failure after heart transplantation is a severe complication generally related to prolonged ischemia time, poor quality of the organ, or acute rejection. All these factors can potent...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/01.tp.0000233845.15508.89

    authors: Beiras-Fernandez A,Weis FC,Fuchs H,Meiser BM,Reichart B,Weis M

    更新日期:2006-10-27 00:00:00

  • The relationship between flow cytometer crossmatch results and subsequent rejection episodes in cadaver renal allograft recipients.

    abstract::Flow cytometry (FC) T and B cell crossmatches were done retrospectively for 38 cadaver renal transplant recipients (29 first and 9 retransplants--minimum follow-up 12 months) using both current pretransplant serum and peak-reactive sera. An increase in median fluorescence intensity (channel shift) and/or an increase i...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-198803000-00012

    authors: Lazda VA,Pollak R,Mozes MF,Jonasson O

    更新日期:1988-03-01 00:00:00

  • Islet Transplantation Provides Superior Glycemic Control With Less Hypoglycemia Compared With Continuous Subcutaneous Insulin Infusion or Multiple Daily Insulin Injections.

    abstract:BACKGROUND:The aim was to compare efficacy of multiple daily injections (MDI), continuous subcutaneous insulin infusion (CSII) and islet transplantation to reduce hypoglycemia and glycemic variability in type 1 diabetes subjects with severe hypoglycemia. METHODS:This was a within-subject, paired comparison of MDI and ...

    journal_title:Transplantation

    pub_type: 杂志文章,多中心研究

    doi:10.1097/TP.0000000000001381

    authors: Holmes-Walker DJ,Gunton JE,Hawthorne W,Payk M,Anderson P,Donath S,Loudovaris T,Ward GM,Kay TW,OʼConnell PJ

    更新日期:2017-06-01 00:00:00

  • Interstitial pneumonitis following autologous bone marrow transplantation.

    abstract::The incidence of interstitial pneumonitis (IP) was reviewed in 70 consecutive patients who received autologous marrow transplants for hematologic malignancies. All patients were treated with total-body irradiation (TBI), with or without other chemotherapeutic agents. Seven patients (10%) developed IP, 3 were due to cy...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-198611000-00015

    authors: Pecego R,Hill R,Appelbaum FR,Amos D,Buckner CD,Fefer A,Thomas ED

    更新日期:1986-11-01 00:00:00

  • Rat xenograft survival in mice treated with donor-specific transfusion and anti-CD154 antibody is enhanced by elimination of host CD4+ cells.

    abstract:BACKGROUND:Treatment with a donor-specific transfusion (DST) and a brief course of anti-mouse CD154 (anti-CD40-ligand) monoclonal antibody (mAb) prolongs the survival of both allografts and rat xenografts in mice. The mechanism by which allograft survival is prolonged is incompletely understood, but depends in part on ...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-200101270-00026

    authors: Gordon EJ,Woda BA,Shultz LD,Rossini AA,Greiner DL,Mordes JP

    更新日期:2001-01-27 00:00:00

  • What clinical alternatives to whole liver transplantation? Current status of artificial devices and hepatocyte transplantation.

    abstract::Shortage of organ donors limits the number of possible liver transplantations. Alternative therapies for treatment of liver failure are currently being developed: (i) extracorporeal artificial liver devices; (ii) bioartificial liver devices using hepatocytes; and (iii) hepatocyte transplantation. The objective of thes...

    journal_title:Transplantation

    pub_type: 社论,评审

    doi:10.1097/TP.0b013e3181963ad3

    authors: Sgroi A,Serre-Beinier V,Morel P,Bühler L

    更新日期:2009-02-27 00:00:00

  • Apoptosis and the expression of Fas and Fas ligand (FasL) antigen in rejection and reinfection in liver allograft specimens.

    abstract:BACKGROUND AND METHODS:To investigate the frequency of apoptosis and the expression of Fas/Fas ligand (FasL) in liver allografts, we examined 97 biopsy specimens from 62 patients after orthotopic liver transplantation. The results of the biopsies were as follows: acute allograft rejection (n=32); hepatitis C virus (HCV...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-199904150-00027

    authors: Tannapfel A,Kohlhaw K,Ebelt J,Hauss J,Liebert U,Berr F,Wittekind C

    更新日期:1999-04-15 00:00:00

  • Assessing the efficacy of kidney paired donation--performance of an integrated three-site program.

    abstract:BACKGROUND:Kidney paired donation (KPD) has emerged as a viable option for renal transplant candidates with incompatible living donors. The aim of this study was to assess the "performance" of a three-site KPD program that allowed screening of multiple donors per recipient. METHODS:We reviewed retrospectively the acti...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/TP.0000000000000054

    authors: Li H,Stegall MD,Dean PG,Casey ET,Reddy KS,Khamash HA,Heilman RL,Mai ML,Taner CB,Kosberg CL,Bakken LL,Wozniak EJ,Giles KL,Veal LA,Gandhi MJ,Cosio FG,Prieto M

    更新日期:2014-08-15 00:00:00

  • 31P nuclear magnetic resonance spectroscopic evaluation of liver preservation solutions.

    abstract::The individual effects of verapamil and 2 antioxidants on perfused rat liver nucleoside triphosphate (NTP) recovery following cold storage in University of Wisconsin (UW) solution was assessed using 31P nuclear magnetic resonance spectroscopy. The pharmacological agents were added to UW solution and were present only ...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:

    authors: Bowers JL,Neymark E,Cook CU,Clouse ME

    更新日期:1994-06-15 00:00:00

  • A sensitive micromethod for generating and assaying allogeneically induced cytotoxic human lymphocytes.

    abstract::A sensitive micromethod for generating and assaying allogeneically induced cytotoxic human lymphocytes in vitro is described. Responding lymphocytes are cultured with mitomycin-C treated allogeneic stimulating cells in wells of replicate microtrays for both one-way mixed leukocyte culture (MLC) and cell-mediated lymph...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-197606000-00005

    authors: Zarling JM,McKeough M,Bach FH

    更新日期:1976-06-01 00:00:00

  • Rituximab, IVIG, and plasma exchange without graft local infusion treatment: a new protocol in ABO incompatible living donor liver transplantation.

    abstract:BACKGROUND:Although graft local infusion (GLI) treatment via the portal vein or the hepatic artery has been the pivotal strategy in ABO incompatible (ABOi) living donor liver transplantation (LDLT) in Japan, the procedure is associated with a high rate of catheter-associated complications. METHODS:A new ABOi-LDLT prot...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/TP.0b013e3181adcae6

    authors: Ikegami T,Taketomi A,Soejima Y,Yoshizumi T,Uchiyama H,Harada N,Iguchi T,Hashimoto N,Maehara Y

    更新日期:2009-08-15 00:00:00

  • CD4+ and B lymphocytes in transplantation immunity. II. Augmented rejection of tumor allografts by mice lacking B cells.

    abstract::To test the importance of B lymphocytes in immunity to major histocompatibility complex class I alloantigens, B cell-deficient mice were generated by reconstituting severe combined immunodeficiency mice, which lack functional B and T lymphocytes, with T cells or with both T and B cells. The reconstituted mice were cha...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:

    authors: Monach PA,Schreiber H,Rowley DA

    更新日期:1993-06-01 00:00:00

  • Sodium-hydrogen exchanger inhibition, pharmacologic ischemic preconditioning, or both for extended cardiac allograft preservation.

    abstract:BACKGROUND:The aim of this study was to determine the efficacy of cariporide (a sodium-hydrogen exchanger inhibitor), BMS180448 (a pharmacologic ischemic preconditioning agent), and the combination thereof, as adjuvant therapies for extended cardiac allograft preservation. METHODS:A porcine model of donor brain death ...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/01.TP.0000079254.81264.6D

    authors: Ryan JB,Hicks M,Cropper JR,Garlick SR,Kesteven SH,Wilson MK,Feneley MP,Macdonald PS

    更新日期:2003-09-15 00:00:00

  • Quality of life of liver transplant recipients. A pilot study.

    abstract::In a cross-sectional survey of the quality of life of 45 liver transplant recipients, physical and psychological status, physical complaints, capability to participate in daily life, social support, and global quality of life were assessed. The average time of follow-up was 9 months after transplantation. Nine patient...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-199309000-00013

    authors: Leyendecker B,Bartholomew U,Neuhaus R,Hörhold M,Blumhardt G,Neuhaus P,Klapp BF

    更新日期:1993-09-01 00:00:00

  • Inverse correlation of cyclosporine binding with sensitivity and resistance.

    abstract::The primary effects of CsA on human lymphocyte responses in vitro appear to be the inhibition of IL-2 production and the inhibition of cytotoxic T cell activation. Induction of suppressor T cell activity is resistant to the effects of CsA. These data imply two distinct subsets of lymphocytes: CsA-resistant and CsA-sen...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-198808001-00012

    authors: Hess AD,Colombani PM

    更新日期:1988-08-01 00:00:00

  • Improved results in pancreatic transplantation by avoidance of nonimmunological graft failures.

    abstract::Twenty eight consecutive combined renal and pancreatic transplantations with enteric exocrine diversion were performed between June 1984 and May 1986. The one-year actuarial patient survival and renal and pancreatic graft survival were 90%, 67%, and 69%, respectively. Nineteen pancreatic grafts and eighteen renal graf...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-198705000-00014

    authors: Tydén G,Brattström C,Lundgren G,Ostman J,Gunnarsson R,Groth CG

    更新日期:1987-05-01 00:00:00

  • Clinical presentation of hepatic artery thrombosis after liver transplantation in the cyclosporine era.

    abstract::Hepatic artery thrombosis is a dreadful complication of orthotopic liver transplantation. It should be suspected in cases of fulminant liver failure, delayed bile leak, or intermittent sepsis of unknown cause after liver transplantation. Accurate diagnosis is assisted by ultrasound and computerized tomography scans, b...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-198512000-00019

    authors: Tzakis AG,Gordon RD,Shaw BW Jr,Iwatsuki S,Starzl TE

    更新日期:1985-12-01 00:00:00

  • Anti-inflammatory effect of transforming growth factor-beta1 in myoblast transplantation.

    abstract:BACKGROUND:The inflammatory reaction that occurs during the 5 days after transplantation led at 3 days to the death of 70% of injected myoblasts. Use of anti-inflammatory agents appeared to be a possible way to increase myoblast survival. The application of gene transfer techniques to cell transplantation offers the po...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-199803270-00005

    authors: Merly F,Huard C,Asselin I,Robbins PD,Tremblay JP

    更新日期:1998-03-27 00:00:00

  • The effect of cyclosporine on agonist-stimulated glomerular and mesangial cell vasodilatory prostaglandin production.

    abstract::Cyclosporine A administration produces an increase in renal vascular resistance and a decrease in glomerular filtration rate in both human and animal models. CsA usage in humans has also been shown to alter the ability of the kidney to adapt to alterations in renal hemodynamics. CsA alters the production of prostaglan...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-199110000-00025

    authors: Bunke M,Wilder L,Martin A

    更新日期:1991-10-01 00:00:00

  • Kidney graft failure and presensitization against HLA class I and class II antigens.

    abstract:BACKGROUND:It is well known that kidney transplant recipients with preformed lymphocytotoxic antibodies against HLA antigens have an increased graft rejection rate. However, the individual contribution of anti-HLA class I and class II antibodies to this phenomenon is poorly understood. We investigated the clinical rele...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-200204270-00014

    authors: Süsal C,Opelz G

    更新日期:2002-04-27 00:00:00

  • Ranolazine--a new drug with beneficial effects on renal preservation.

    abstract::Ranolazine is a new drug with a novel mode of action as a metabolic modulator and membrane stabilizer. In this prospective randomized double-blind trial, a porcine model of renal autotransplantation was used to assess the effects of this drug during preservation and reperfusion of kidneys cold-stored for 24 hr in phos...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-199011000-00004

    authors: Lodge JP,Lam FT,Perry SL,Giles GR

    更新日期:1990-11-01 00:00:00

  • Antibodies to donor B lymphocytes and mixed lymphocyte culture blocking in cadaveric renal transplantation.

    abstract::In 33 renal allograft recipient-donor pairs, B and T lymphocyte complement-dependent cytotoxicity crossmatches and mixed lymphocyte culture (MLC) blocking experiments were performed and the results were correlated with graft outcome. MLC blocking particularly in the unidirectional culture against donor-stimulating cel...

    journal_title:Transplantation

    pub_type: 杂志文章

    doi:10.1097/00007890-197804000-00002

    authors: Ettenger RB,Opelz G,Walker J,Terasaki PI,Uittenbogaart C,Pennisi AJ,Malekzadeh MH,Fine RN

    更新日期:1978-04-01 00:00:00