Abstract:
BACKGROUND:Acid sphingomyelinase knock-out (ASMKO) mice are a model of types A and B Niemann-Pick disease. In the present study, we evaluated whether bone marrow transplantation (BMT) carried out on newborn ASMKO mice could prevent or alter the Niemann-Pick disease phenotype. METHODS:Previous work from our laboratory had shown that ASMKO mice were highly susceptible to irradiation-induced death. Therefore, we preconditioned 1-day-old ASMKO (n=35) mice with a "sublethal" dose of 200 cGy of total body irradiation before BMT. The transplantation effects were then analyzed by biochemical, pathological, and clinical approaches. RESULTS:Engraftment ranging from 7% to 100% was achieved in 97% of the transplanted animals. Growth of the engrafted animals was improved, and their survival was increased (from a mean of 5 months to 9 months). The onset of ataxia also was delayed in most of the engrafted animals. In accordance with these observations, biochemical and pathological analysis revealed significant changes in the transplanted group as compared with nontransplanted animals. Lipid storage was reduced in several organs, and there was evidence of histologic improvement seen throughout the reticuloendothelial system, even in animals that were engrafted as low as 14%. In the central nervous system, lipid storage also was reduced, and the Purkinje cells, which are almost absent in ASMKO mice, were present in certain areas of the transplanted animals cerebella. CONCLUSIONS:These results demonstrated that BMT could alter the pathologic phenotype in ASMKO mice, but that this procedure alone was not sufficient to elicit a complete therapeutic effect.
journal_name
Transplantationjournal_title
Transplantationauthors
Miranda SR,Erlich S,Friedrich VL Jr,Haskins ME,Gatt S,Schuchman EHdoi
10.1097/00007890-199804150-00005subject
Has Abstractpub_date
1998-04-15 00:00:00pages
884-92issue
7eissn
0041-1337issn
1534-6080journal_volume
65pub_type
杂志文章相关文献
TRANSPLANTATION文献大全abstract:BACKGROUND:New insights into mechanisms of injury in orthotopic liver transplantation (OLT) often come from experiments in small animal models. Mice are particularly suitable because of the growing availability of gene-altered animals and specific antibodies. A validated model of OLT in mice is not available and, in pa...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-200211150-00008
更新日期:2002-11-15 00:00:00
abstract:BACKGROUND:Ischemia-reperfusion (I/R) injury is a multifactorial phenomenon that occurs during the transplant event and frequently compromises early graft function after liver transplantation (LT). Current comprehension of molecular mechanisms and regulation processes of I/R injury lacks clarity. MicroRNA (miRNA) regul...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/TP.0b013e3182a20398
更新日期:2013-11-27 00:00:00
abstract:BACKGROUND:At our institution, increased kidney donation from unrelated donors accounts for a steady rise in live donor kidney transplantation rates. We compared outcomes of living related (LRT) versus living unrelated kidney transplants (LURT) and analyzed the effect of early rejection upon graft survival. METHODS:A ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/01.tp.0000135464.11616.5a
更新日期:2004-10-15 00:00:00
abstract::Islet transplantation may be optimized by islet immunoisolation to prevent direct contact between the islet graft and the host tissue. In this study, we examined the glycemia and insulin secretion in streptozotocin-diabetic rats transplanted with islets subjected to immunoisolation with Algire diffusion chamber or wit...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-199103000-00005
更新日期:1991-03-01 00:00:00
abstract::Increased QT dispersion (QTd), predicting patients with risk of malignant arrhythmia, have recently been reported in hemodialysis patients (HDp). In this prospective study, we aimed to investigate changes in QTd and signal averaged-ECG (SAECG) in HDp after transplantation. Twenty-seven HDp (M/F:18/9, mean age 30+/-8 y...
journal_title:Transplantation
pub_type: 临床试验,杂志文章
doi:10.1097/00007890-200111150-00009
更新日期:2001-11-15 00:00:00
abstract:BACKGROUND:Recent evidence has suggested that inconsistencies in human-islet yields after collagenase digestion are attributed to the activation of endogenous enzymes of the cadaveric donor pancreas. Inhibition of protease activity by Pefabloc (0.4 mM; Roche Biochemicals Inc., Indianapolis, IN) has recently been shown ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/01.TP.0000046537.47139.CE
更新日期:2003-02-27 00:00:00
abstract:BACKGROUND:Acute rejection (AR) of the transplanted kidney has been identified as the major risk factor for the development of chronic rejection and immunological graft loss. However, the clinical presentation and response to AR therapy can vary considerably between recipients. METHODS:We studied the first AR episode ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-199911150-00017
更新日期:1999-11-15 00:00:00
abstract::Suppressor cell activity in the spleen of 4-5-day-old mice is high and can be assayed in mixed lymphocyte cultures. The suppressor cells inhibit the proliferation of adult mouse spleen cells responding in vitro to an allogeneic stimulus. This suppressor cell activity can be increased by injecting the mice, on the day ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-198409000-00010
更新日期:1984-09-01 00:00:00
abstract:BACKGROUND:Long-term use of immunosuppressants is associated with significant morbidity and mortality in transplant recipients. A simple whole blood assay that has U.S. Food and Drug Administration clearance directly assesses the net state of immune function of allograft recipients for better individualization of thera...
journal_title:Transplantation
pub_type: 杂志文章,meta分析
doi:10.1097/01.tp.0000234837.02126.70
更新日期:2006-09-15 00:00:00
abstract::Methods are described for preparing large amounts of horse anti-human thymocyte globulin (ATG, ATGAM; The Upjohn Company) for clinical use. These methods have been used since 1968 to provide material for clinical trials. Characteristics of 40 lots of ATG are summarized. ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-197910000-00007
更新日期:1979-10-01 00:00:00
abstract:BACKGROUND:Most transplant centers in the United States, including our own, use creatinine clearance (Ccr) to estimate glomerular filtration rate (GFR) in potential living kidney donors. The purpose of this study was to evaluate our experience with estimation of donor GFR by Ccr and to explore other potential strategie...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-200101270-00016
更新日期:2001-01-27 00:00:00
abstract:BACKGROUND:The development of kidney dysfunction is one of the most important after liver transplantation (LT). Genetic variants of pathogenetically relevant cytokines may influence the development and course of the disease. The aim of our study was to evaluate the role of transforming growth factor-β1 (TGF-β1) polymor...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/TP.0b013e318242be0b
更新日期:2012-03-15 00:00:00
abstract::Low-affinity, cold-reactive antibodies easily removed by washing were not detected by the antiglobulin technique but killed T lymphocytes when washing was omitted, incubation was prolonged, and cytotoxic tests were incubated at room temperature or at 4 degrees C. These antibodies were present in approximately 25% of s...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-198710000-00014
更新日期:1987-10-01 00:00:00
abstract:BACKGROUND:Extracorporeal support (ECS) during organ procurement from donors after circulatory determination of death (DCDD) could increase the number of donor organs and decrease posttransplant complications. This study reports the experience of a large transplant center with controlled DCDD. METHODS:A retrospective ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/TP.0000000000000070
更新日期:2014-08-15 00:00:00
abstract::Leflunomide is a compound recently shown to reduce T and B cell-mediated responses in a number of experimental rat, mouse, and human systems. To explore its potential as an immunosuppressant, we studied leflunomide in 128 Brown-Norway/Lewis cardiac transplants and in 48 unoperated Lewis rats. At doses ranging from 0.6...
journal_title:Transplantation
pub_type: 杂志文章
doi:
更新日期:1994-04-27 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:Human islet transplantation is a great potential therapy for type I diabetes. To investigate islet graft survival and function, we recently showed the improved effects after co-culture and co-transplantation with vascular endothelial cells (ECs) in diabetic rats. METHODS:ECs were isolated, and the viability...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/TP.0b013e3182356ca7
更新日期:2011-12-15 00:00:00
abstract::A severe shortage of organs is one of the major barriers facing transplantation today. One of the proposals designed to overcome this serious problem is to increase the use of genetically unrelated living kidney donors. Excellent results have been achieved with these volunteers and cogent arguments have been made that...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-200004270-00035
更新日期:2000-04-27 00:00:00
abstract::We performed a multicenter study to investigate the problem of cadaver kidney transplants that never achieve function, or that recover after a period of delayed function, and we used multivariate techniques to model the results. Sixty-nine patients who received cadaver kidney transplants that never functioned (NF) wer...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-198801000-00027
更新日期:1988-01-01 00:00:00
abstract:BACKGROUND:Liver transplant (LT) for nonalcoholic steatohepatitis (NASH) related hepatocellular carcinoma (HCC) is not well characterized in the literature. The aim of the study was to examine characteristics and outcomes of patients who had LT for NASH-HCC (NASH) versus HCC from other liver diseases (non-NASH). METHO...
journal_title:Transplantation
pub_type: 杂志文章,多中心研究
doi:10.1097/TP.0000000000002043
更新日期:2018-04-01 00:00:00
abstract::Erythropoietin (EPO) is widely used among patients with end-stage renal disease awaiting transplantation. Data suggest that EPO therapy may be immunomodulatory. The purpose of this study was to assess the effects of pretransplant EPO therapy on renal allograft outcome. We evaluated 120 consecutive renal transplant rec...
journal_title:Transplantation
pub_type: 临床试验,杂志文章
doi:10.1097/00007890-199610150-00024
更新日期:1996-10-15 00:00:00
abstract::Healthy rat islets were encapsulated in alginate-polylysine-alginate capsules measuring 0.25-0.35 mm in diameter using a modified encapsulation technique. The encapsulated islets were transplanted intraperitoneally in nonimmunosuppressed streptozotocin-induced diabetic BALB/c mice. The diabetic condition of the experi...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-199206000-00002
更新日期:1992-06-01 00:00:00
abstract::Eight patients with chronic myeloid leukemia relapse after allogeneic BMT were treated with IFN-alpha and buffy coat transfusions (BC) of the bone marrow donor. The antileukemic effect of this treatment was directly demonstrated in 4 patients by the disappearance of Philadelphia chromosome-positive metaphases or the l...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-199311000-00013
更新日期:1993-11-01 00:00:00
abstract:BACKGROUND:The pathogenesis of acute pulmonary xenograft injury has not yet been determined. The present study evaluates the role of complement in mediating pulmonary xenograft dysfunction by using cobra venom factor (CVF) to deplete recipient complement and transgenic swine, which express human regulators of complemen...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-199804270-00013
更新日期:1998-04-27 00:00:00
abstract:BACKGROUND:Many reports of successful early withdrawal of regular maintenance steroids in transplant recipients have appeared in recent years. The question now arises whether, in the current age of powerful nonsteroidal immunosuppressants such as Neoral and Tacrolimus, routine administration of steroids posttransplant ...
journal_title:Transplantation
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00007890-199910270-00021
更新日期:1999-10-27 00:00:00
abstract:BACKGROUND:One of the main concerns after living donor liver transplantation is the risk of morbidity and/or mortality that it imposes on the donors. Respiratory postoperative complications in living liver donors have already been reported but their frequency seems to be underestimated. We designed a prospective study ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/01.tp.0000191624.70135.35
更新日期:2006-01-27 00:00:00
abstract::Sera from 56 recipients of liver or heart transplants were investigated for monoclonal immunoglobulins (mIg) by immunofixation electrophoresis (IFE) at different times during 4 years after transplantation. Transient, changing, or stable mIgs were found in 18 patients. A significantly increased mIg incidence was observ...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/00007890-198809000-00011
更新日期:1988-09-01 00:00:00
abstract::An isolated vascularized bone marrow transplant (iVBMT) model was previously developed in the rat to specifically study the role of bone marrow and its environment in a composite tissue allotransplant. An extraperitoneal model was successfully created to avoid laparotomy and cross-clamping of the great vessels. The ex...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/01.TP.0000061490.85273.29
更新日期:2003-05-15 00:00:00
abstract:BACKGROUND:Finding the best combination of immunosuppression is an important challenge in kidney transplantation. Current short-term (1- and 3-year) allograft survival is quite good, making it difficult to determine differences in therapeutic regimens without large sample sizes. Using data from the United Network for O...
journal_title:Transplantation
pub_type: 杂志文章
doi:10.1097/01.TP.0000079839.38844.09
更新日期:2003-08-15 00:00:00
abstract:BACKGROUND:Sirolimus (rapamycin) is a potent immunosuppressant with a mechanism of action different from cyclosporine (CsA) or tacrolimus. METHODS:In 11 European centers, first cadaveric renal allograft recipients were randomized to CsA (n=42) or sirolimus (n=41). Dosing of these agents was concentration-controlled an...
journal_title:Transplantation
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1097/00007890-199904150-00017
更新日期:1999-04-15 00:00:00