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
Transplantationjournal_title
Transplantationauthors
Schauer R,Lang T,Zimmermann A,Stangl M,Da Silva L,Schildberg FW,Rau HGdoi
10.1097/00007890-200201150-00012subject
Has Abstractpub_date
2002-01-15 00:00:00pages
67-9issue
1eissn
0041-1337issn
1534-6080journal_volume
73pub_type
杂志文章相关文献
TRANSPLANTATION文献大全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
更新日期:1999-04-27 00:00:00
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
更新日期:1990-08-01 00:00:00
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
更新日期:2005-02-15 00:00:00
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
更新日期:2009-09-15 00:00:00
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
更新日期:2014-02-15 00:00:00
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
更新日期:2010-11-27 00:00:00
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
更新日期:1989-03-01 00:00:00
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
更新日期:2001-04-27 00:00:00
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
更新日期:2006-10-27 00:00:00
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
更新日期:1988-03-01 00:00:00
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
更新日期:2017-06-01 00:00:00
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
更新日期:1986-11-01 00:00:00
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
更新日期:2001-01-27 00:00:00
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
更新日期:2009-02-27 00:00:00
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
更新日期:1999-04-15 00:00:00
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
更新日期:2014-08-15 00:00:00
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:
更新日期:1994-06-15 00:00:00
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
更新日期:1976-06-01 00:00:00
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
更新日期:2009-08-15 00:00:00
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:
更新日期:1993-06-01 00:00:00
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
更新日期:2003-09-15 00:00:00
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
更新日期:1993-09-01 00:00:00
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
更新日期:1988-08-01 00:00:00
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
更新日期:1987-05-01 00:00:00
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
更新日期:1985-12-01 00:00:00
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
更新日期:1998-03-27 00:00:00
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
更新日期:1991-10-01 00:00:00
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
更新日期:2002-04-27 00:00:00
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
更新日期:1990-11-01 00:00:00
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
更新日期:1978-04-01 00:00:00