Abstract:
:Citrullinaemia is a rare autosomal recessive disorder due to a deficiency of argininosuccinate synthetase (ASS). While the clinical course of the adult onset form (Type II) is unpredictable, many patients undergo sudden deterioration with progressive cerebral oedema. Two patients with Type II citrullinaemia were referred for liver transplantation. One patient was successfully transplanted, and his plasma citrulline level decreased from 300 to 69 mumol/l (normal range 20-60 mumol/l); Fisher's ratio increased to a normal range within 24 h of transplantation. The other patient died from cerebral oedema, despite Optimal pharmacological measures, while awaiting a suitable donor organ. Dietary and pharmacological treatment is vital before liver transplantation. The level of serum arginine requires regulation, as it can rise secondary to citrullinaemia in Type II disease. An immunocytochemical study of the liver in both patients showed a clustered distribution of ASS which is associated with a dismal prognosis compared with patients who have a homogenous distribution. Distribution of ASS was normal in the transplanted liver. Liver transplantation is effective therapy for adult-onset Type II citrullinaemia. A clustered pattern of ASS distribution in a liver biopsy is a significant feature to activate early referral for liver transplantation.
journal_name
Clin Transplantjournal_title
Clinical transplantationauthors
Kawamoto S,Strong RW,Kerlin P,Lynch SV,Steadman C,Kobayashi K,Nakagawa S,Matsunami H,Akatsu T,Saheki Tsubject
Has Abstractpub_date
1997-10-01 00:00:00pages
453-8issue
5 Pt 1eissn
0902-0063issn
1399-0012journal_volume
11pub_type
杂志文章abstract:BACKGROUND:The survival of patients returning to hemodialysis (HD) following kidney transplant failure is unfavorable. However, the factors responsible for this poor outcome are largely unknown; chronic inflammation due to failed allograft and malnutrition may contribute to morbidity and mortality. We aimed to compare ...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2009.01097.x
更新日期:2010-07-01 00:00:00
abstract:BACKGROUND:One severe side effect of calcineurin inhibitors (CNIs: such as cyclosporine [CsA] and tacrolimus [FK506]) is neurotoxicity. CNIs are substrates for CYP3A5 and P-glycoprotein (P-gp), encoded by ABCB1 gene. In the present study, we hypothesized that genetic variability in CYP3A5 and ABCB1 genes may be associa...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2009.01181.x
更新日期:2010-11-01 00:00:00
abstract:INTRODUCTION:Previously, increasing age has been a part of the exclusion criteria used when determining eligibility for a pancreas transplant. However, the analysis of pancreas transplantation outcomes based on age groupings has largely been based on single-center reports. METHODS:A UNOS database review of all adult p...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/ctr.12407
更新日期:2014-09-01 00:00:00
abstract::Endothelial dysfunction is of vital importance, as it may cause ischemia and dysfunction in various organs. Despite, this problem has been well documented in patients with end-stage renal disease (ESRD), there is not enough data considering this issue following renal transplantation. One of the potential causes of end...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-0012.2003.00030.x
更新日期:2003-06-01 00:00:00
abstract::The aim of this study was to improve outcomes in living donor liver transplantation (LDLT) patients with portal vein thrombosis (PVT). Of 246 adult patients who underwent LDLT with a right lobe graft between January 2000 and May 2007, PVT was diagnosed in 50 patients (20.3%), who were further subdivided into partial (...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2010.01217.x
更新日期:2011-01-01 00:00:00
abstract:INTRODUCTION:The impact of induction immunosuppression on long-term survival in lung transplant recipients remains unclear. We sought to evaluate the effect of contemporary induction immunosuppression agents in lung transplant recipients' survival, utilizing national registry data. METHODS:We queried the United Networ...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/ctr.12339
更新日期:2014-04-01 00:00:00
abstract::Micafungin has been approved for the prophylaxis of Candida infections in patients undergoing allogeneic hematopoietic stem cell transplantation (allo-HSCT). Here, we report a single-center experience of three different dose levels regarding efficacy, toxicity, and colonization with Candida ssp. in clinical practice. ...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/ctr.12310
更新日期:2014-03-01 00:00:00
abstract::Changes to the United States kidney allocation system targeted at reducing organ discard have failed to improve organ utilization. High Kidney Donor Profile Index kidneys continue to be discarded at high rates as a result of the regulatory and financial barriers to widespread utilization of these organs. However, ther...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/ctr.13990
更新日期:2020-09-01 00:00:00
abstract:BACKGROUND:The aim of this study was to see whether lessons could be learned from the prospectively maintained nationwide database on solitary pancreas transplantation (SPTx) performed in the UK. METHODS:Two hundred and forty-five SPTx were utilized from the 2004-2013 period (113 pancreas transplant alone and 132 panc...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/ctr.12650
更新日期:2015-12-01 00:00:00
abstract::Post-transplant lymphoproliferative disorders (PTLDs) are a heterogeneous set of complications of organ transplantation associated with poor patient prognosis. We analyzed the clinicopathological features of PTLDs in 43 adult and pediatric recipients of solid organ or bone marrow transplantation at a large transplant ...
journal_title:Clinical transplantation
pub_type: 临床试验,杂志文章
doi:10.1111/j.1399-0012.2010.01392.x
更新日期:2012-01-01 00:00:00
abstract::Acute renal failure is a significant risk factor for death in patients with liver failure. The goal of this study was to analyze the impact of peri-transplant dialysis on the long-term mortality of liver transplant recipients. We performed a single-center, retrospective cohort study of 743 adult liver transplants; pat...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2010.01238.x
更新日期:2011-03-01 00:00:00
abstract::There is limited information regarding the role of flow cytometry crossmatching (FCXM) in primary cadaver kidney allografting and even less about B cell reactivity and graft survival (GS). Furthermore, there is little or no published data concerning reaction strength (cutoff value), the effect of historic sera reactio...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:
更新日期:1996-12-01 00:00:00
abstract::Measurement of indocyanine green plasma disappearance rate (PDR(ICG)) has been suggested as a meaningful liver function parameter. However, there are only very limited data concerning its value in the monitoring of graft dysfunction (GDF) and primary non-function (PNF) especially during molecular absorbent recirculati...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2007.00706.x
更新日期:2007-11-01 00:00:00
abstract::Kidney dysfunction is a recognized complication after non-renal solid organ transplantation, particularly after intestinal transplant. In our study, we reviewed data on 33 multivisceral transplant (MVT)- and 15 isolated small bowel (ISB)-transplant patients to determine risk factors for kidney dysfunction. Kidney func...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2012.01672.x
更新日期:2012-07-01 00:00:00
abstract::The objective of this meta-analysis was to review the evidence for the use of immune globulin in the prevention of symptomatic cytomegalovirus (CMV) disease in transplant recipients. A computerized search of Medline from 1980 to October 1991 was conducted using the textword "cytomegalo:" and the subheading "prevention...
journal_title:Clinical transplantation
pub_type: 杂志文章,meta分析
doi:
更新日期:1994-02-01 00:00:00
abstract::Subclinical acute rejection (SAR) occurs in about 30% of stable renal transplant patients and may be a risk factor for a poor allograft outcome. In the present study, the prevalence and clinical features of subclinical rejection, and the expression of immune activation markers in surveillance graft biopsies were asses...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2004.00170.x
更新日期:2004-08-01 00:00:00
abstract::The transplantation of vascularized organs between species stimulates an intense rejection reaction by the recipient of the graft. The classification of the xenograft rejection reaction on the basis of the degree of genetic disparity between donor and recipient species and the presence or absence of preformed antibody...
journal_title:Clinical transplantation
pub_type: 杂志文章,评审
doi:
更新日期:1994-04-01 00:00:00
abstract::According to the EU Directive 2006/17/EC, European tissue banks are required to perform a physical examination on every potential tissue donor. The directive itself, however, does not specify the content of this examination. The aim of this study was to investigate the current practice of physical examination in Europ...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2009.01089.x
更新日期:2010-03-01 00:00:00
abstract::Omega-3 fatty acids have been shown to play a role in preventing development of both acute and chronic rejection. New evidence also suggests that HMGCoA reductase inhibitors may have an immunomodulatory effect and may decrease the incidence of rejection in both animal and human transplant models. Omega-3 fatty acids a...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:
更新日期:1995-06-01 00:00:00
abstract::Hepatic allograft rejection still remains an important problem following liver transplantation. Early acute rejection, occurring within three months of transplant, is a common event and usually of lesser significance with respect to prognosis than other non-immune-related post-transplant morbidities. However, little i...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2007.00788.x
更新日期:2008-05-01 00:00:00
abstract::With the increased number of long-term survivors after liver transplantation, new-onset diabetes after transplantation (NODAT) is becoming more significant in patient follow-up. However, the incidence of new-onset diabetes after living-donor liver transplantation (LDLT) has not been well elucidated. The aim of this st...
journal_title:Clinical transplantation
pub_type: 临床试验,杂志文章
doi:10.1111/ctr.12103
更新日期:2013-05-01 00:00:00
abstract::ABO-incompatible kidney transplantation (ABO-ILKT) has been reported to have a higher rate of early complications and higher medical costs than ABO-compatible kidney transplantation (ABO-CLKT). We aimed to compare the clinical outcomes, complications, and medical costs between ABO-ILKTs and ABO-CLKTs at 2 years post-t...
journal_title:Clinical transplantation
pub_type: 临床试验,杂志文章
doi:10.1111/ctr.13591
更新日期:2019-06-01 00:00:00
abstract::The study included 110 consecutive patients with hematological malignancies receiving fludarabine-based reduced intensity conditioning (RIC) and hematopoietic stem cell transplantation (HSCT) from matched unrelated donors. The median age was 55 yr (range 11-68) and all but 15 patients received peripheral blood stem ce...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/ctr.12131
更新日期:2013-07-01 00:00:00
abstract:BACKGROUND:The shortage of grafts for kidney transplantation (Tx) has resulted in an expansion of criteria for pediatric donors. However, the limitations of pediatric donors for kidney Tx are not yet clearly established. We investigated the outcomes of recipients with pediatric kidney grafts from Colorado to elucidate ...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:
更新日期:1998-12-01 00:00:00
abstract::Clinical small bowel transplantation (SBTx) has been associated with a high rate of infectious complications. Laparotomy, preservation injury, abnormal motility, lymphatic disruption, aberrant systemic venous drainage, rejection and antibiotic therapy could all be implicated in the etiology of these complications. In ...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-0012.2000.140609.x
更新日期:2000-12-01 00:00:00
abstract:BACKGROUND:Arterial stiffness and left ventricular hypertrophy (LVH) are major independent risk factors for cardiovascular disease in healthy and renal population. In this study, we aimed to investigate comparative long-term effects of renal transplantation (RTx) and of hemodialysis (HD) on both arterial stiffness and ...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2008.00794.x
更新日期:2008-05-01 00:00:00
abstract::The study explores the prevalence of unrecognized diabetes mellitus (DM), the incidence and risk factors new onset diabetes mellitus (NODM) and determines whether patient survival differs between patients with transient (NODM that resolves) compared with those with fixed NODM. This is a single center review of solitar...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2006.00529.x
更新日期:2006-09-01 00:00:00
abstract:BACKGROUND:The accurate estimation of liver volume and right/left ratio in donor candidates is critical, but there is no method using portal vein (PV) flow. METHODS:Of 125 donor candidates, right/left liver volume ratio was estimated using ultrasound (US)-PV area ratio and Doppler US-PV flow ratio, and the results wer...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/ctr.13050
更新日期:2017-09-01 00:00:00
abstract::The progression of HCV-related disease is particularly aggressive in the post-transplantation setting. Recipients with recurrent HCV infection undergo repeated liver biopsies in order to estimate disease progression. A strong association was found between serum immunoglobulins levels and hepatic fibrosis in non-transp...
journal_title:Clinical transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-0012.2007.00657.x
更新日期:2007-05-01 00:00:00
abstract:BACKGROUND:There are limited published data concerning the effects of different immunosuppressive regimens on the development of polyomavirus (BKV) viremia. We examined the risk of developing BKV viremia in kidney transplant recipients receiving everolimus (EVR) or mycophenolic acid (MPA) as maintenance therapy. METHO...
journal_title:Clinical transplantation
pub_type: 临床试验,杂志文章
doi:10.1111/ctr.12151
更新日期:2013-07-01 00:00:00