Orthotopic liver transplantation for adult-onset type II citrullinaemia.

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 Transplant

journal_title

Clinical transplantation

authors

Kawamoto S,Strong RW,Kerlin P,Lynch SV,Steadman C,Kobayashi K,Nakagawa S,Matsunami H,Akatsu T,Saheki T

subject

Has Abstract

pub_date

1997-10-01 00:00:00

pages

453-8

issue

5 Pt 1

eissn

0902-0063

issn

1399-0012

journal_volume

11

pub_type

杂志文章
  • Comparison of nutritional status in hemodialysis patients with and without failed renal allografts.

    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

    authors: Yelken BM,Gorgulu N,Caliskan Y,Yazici H,Turkmen A,Yildiz A,Sever MS

    更新日期:2010-07-01 00:00:00

  • Influence of CYP3A5 and ABCB1 gene polymorphisms on calcineurin inhibitor-related neurotoxicity after hematopoietic stem cell transplantation.

    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

    authors: Yanagimachi M,Naruto T,Tanoshima R,Kato H,Yokosuka T,Kajiwara R,Fujii H,Tanaka F,Goto H,Yagihashi T,Kosaki K,Yokota S

    更新日期:2010-11-01 00:00:00

  • An analysis of pancreas transplantation outcomes based on age groupings--an update of the UNOS database.

    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

    authors: Siskind E,Maloney C,Akerman M,Alex A,Ashburn S,Barlow M,Siskind T,Bhaskaran M,Ali N,Basu A,Molmenti E,Ortiz J

    更新日期:2014-09-01 00:00:00

  • The effect of calcineurin inhibitors on endothelial function in renal transplant recipients.

    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

    authors: Oflaz H,Turkmen A,Kazancioglu R,Kayacan SM,Bunyak B,Genchallac H,Erol B,Mercanoglu F,Umman S,Sever MS

    更新日期:2003-06-01 00:00:00

  • Clinical analysis of living donor liver transplantation in patients with portal vein thrombosis.

    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

    authors: Kim SJ,Kim DG,Park JH,Moon IS,Lee MD,Kim JI,Yoon YC,Yoo YK

    更新日期:2011-01-01 00:00:00

  • To induce or not to induce: a 21st century evaluation of lung transplant immunosuppression's effect on survival.

    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

    authors: Whitson BA,Lehman A,Wehr A,Hayes D Jr,Kirkby S,Pope-Harman A,Kilic A,Higgins RS

    更新日期:2014-04-01 00:00:00

  • Micafungin as antifungal prophylaxis in recipients of allogeneic hematopoietic stem cell transplantation: results of different dosage levels in clinical practice.

    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

    authors: Langebrake C,Rohde H,Lellek H,Wolschke C,Kröger NM

    更新日期:2014-03-01 00:00:00

  • A call to action: Feasible strategies to reduce the discard of transplantable kidneys in the United States.

    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

    authors: Stewart ZA,Shah SA,Formica RN,Kandaswamy R,Paramesh AS,Friedman J,Squires R,Cooper M,Axelrod DA

    更新日期:2020-09-01 00:00:00

  • Solitary pancreas transplantation: a review of the UK experience over a period of 10 yr.

    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

    authors: Vrakas G,Arantes RM,Gerlach U,Reddy S,Friend P,Vaidya A

    更新日期:2015-12-01 00:00:00

  • Post-transplant lymphoproliferative disorders: clinicopathological analysis of 43 cases in a single center, 1990-2009.

    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

    authors: Yoon SO,Yu E,Cho YM,Suh C,Kim KM,Han DJ,Lee SG,Huh J

    更新日期:2012-01-01 00:00:00

  • High mortality in orthotopic liver transplant recipients who require hemodialysis.

    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

    authors: Zand MS,Orloff MS,Abt P,Patel S,Tsoulfas G,Kashyap R,Jain A,Safadjou S,Bozorgzadeh A

    更新日期:2011-03-01 00:00:00

  • Flow cytometry crossmatching and primary cadaver kidney graft outcome: relevance of T and B cell targets, historic sera and autologous controls.

    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:

    authors: LeFor WM,Ackermann JR,Alveranga DY,Bowers VD,Heinrichs DF,Kahana L,Shires DL Jr,Weinstein SS,Wright CE

    更新日期:1996-12-01 00:00:00

  • Indocyanine green elimination but not bilirubin indicates improvement of graft function during MARS therapy.

    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

    authors: Scheingraber S,Richter S,Igna D,Girndt M,Flesch S,Kleinschmidt S,Schilling MK

    更新日期:2007-11-01 00:00:00

  • Risk factors for native kidney dysfunction in patients with abdominal multivisceral/small bowel transplantation.

    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

    authors: Suzuki M,Mujtaba MA,Sharfuddin AA,Yaqub MS,Mishler DP,Faiz S,Vianna RM,Mangus RS,Tector JA,Taber TE

    更新日期:2012-07-01 00:00:00

  • Use of immune globulin to prevent symptomatic cytomegalovirus disease in transplant recipients--a meta-analysis.

    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:

    authors: Glowacki LS,Smaill FM

    更新日期:1994-02-01 00:00:00

  • Prevalence and immunohistochemical findings of subclinical kidney allograft rejection and its association with graft outcome.

    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

    authors: Veronese FV,Noronha IL,Manfro RC,Edelweiss MI,Goldberg J,Gonçalves LF

    更新日期:2004-08-01 00:00:00

  • The pathogenesis of xenograft rejection.

    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:

    authors: Makowka L,Cramer DV

    更新日期:1994-04-01 00:00:00

  • Physical examination of the potential tissue donor, what do European tissue banks do?

    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

    authors: Van Geyt C,Van Wijk M,Bokhorst A,Beele H

    更新日期:2010-03-01 00:00:00

  • HMGCoA reductase inhibitors and omega-3 fatty acids as adjunctive agents in maintenance immunosuppression after solid organ transplantation.

    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:

    authors: Katznelson S,Wilkinson AH

    更新日期:1995-06-01 00:00:00

  • Late acute rejection after liver transplantation impacts patient survival.

    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

    authors: Uemura T,Ikegami T,Sanchez EQ,Jennings LW,Narasimhan G,McKenna GJ,Randall HB,Chinnakotla S,Levy MF,Goldstein RM,Klintmalm GB

    更新日期:2008-05-01 00:00:00

  • Incidence and risk factors for new-onset diabetes in living-donor liver transplant recipients.

    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

    authors: Honda M,Asonuma K,Hayashida S,Suda H,Ohya Y,Lee KJ,Yamamoto H,Takeichi T,Inomata Y

    更新日期:2013-05-01 00:00:00

  • Outcomes, complications, and economic impact of ABO-incompatible living kidney transplantation: A single-center Japanese cohort study.

    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

    authors: Kakuta Y,Okumi M,Unagami K,Iizuka J,Takagi T,Ishida H,Tanabe K

    更新日期:2019-06-01 00:00:00

  • A high antithymocyte globulin dose increases the risk of relapse after reduced intensity conditioning HSCT with unrelated donors.

    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

    authors: Remberger M,Ringdén O,Hägglund H,Svahn BM,Ljungman P,Uhlin M,Mattsson J

    更新日期:2013-07-01 00:00:00

  • Limitations of pediatric donor kidneys for transplantation.

    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:

    authors: Yagisawa T,Kam I,Chan L,Springer JW,Dunn S

    更新日期:1998-12-01 00:00:00

  • Low infectious complications in segmental living related small bowel transplantation in adults.

    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

    authors: Cicalese L,Sileri P,Asolati M,Rastellini C,Abcarian H,Benedetti E

    更新日期:2000-12-01 00:00:00

  • Comparative effects of renal transplantation and maintenance dialysis on arterial stiffness and left ventricular mass index.

    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

    authors: Keven K,Calayoglu R,Sengul S,Dincer I,Kutlay S,Erturk S,Erbay B,Nergizoglu G

    更新日期:2008-05-01 00:00:00

  • New onset diabetes mellitus post-kidney transplantation.

    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

    authors: Kiberd M,Panek R,Kiberd BA

    更新日期:2006-09-01 00:00:00

  • Measurement of liver volumes by portal vein flow by Doppler ultrasound in living donor liver transplantation.

    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

    authors: Choi SH,Kwon JH,Kim KW,Jang HY,Kim JH,Kwon HJ,Lee J,Song GW,Lee SG

    更新日期:2017-09-01 00:00:00

  • Serum globulin levels in predicting the extent of hepatic fibrosis in patients with recurrent post-transplant hepatitis C infection.

    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

    authors: Schmilovitz-Weiss H,Cohen M,Pappo O,Sulkes J,Braun M,Tur-Kaspa R,Ben-Ari Z

    更新日期:2007-05-01 00:00:00

  • Everolimus leads to a lower risk of BKV viremia than mycophenolic acid in de novo renal transplantation patients: a single-center experience.

    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

    authors: Moscarelli L,Caroti L,Antognoli G,Zanazzi M,Di Maria L,Carta P,Minetti E

    更新日期:2013-07-01 00:00:00