The curative treatment of familial hypercholesterolemia: Liver transplantation.

Abstract:

BACKGROUND:Familial hypercholesterolemia (FH) is an autosomal dominant genetic disorder characterized by premature mortal cardiovascular complications. Liver transplantation (LT) is the only curative treatment option. In this study, the long-term clinical follow-up data of 8 patients who underwent LT with a diagnosis of FH in our center are presented. MATERIALS AND METHODS:A total of 638 LT were performed between December 1985 and June 2019 at Baskent University, of which 8 patients underwent LT with a diagnosis of FH and were evaluated retrospectively. RESULTS:Of the 8 patients, 4 underwent deceased donor and 4 living donor transplantation. Five patients had preoperative cardiovascular disease and consequent interventional operations. There was significant reduction in postoperative LDL-C and TC levels starting from the first week, and stabilizing at the first month and first year. The median survival time of patients was 5 years (2-12 years). All patients are still alive. None of the complications of patients with preoperative cardiovascular complications had progressed. CONCLUSION:Liver transplantation is the preferred curative treatment for the pathophysiology of FH. In our study, LDL-C levels were brought under control with LT performed on patients with FH. Median 5-year follow-up of patients showed that the progression of cardiac complications was abated.

journal_name

Clin Transplant

journal_title

Clinical transplantation

authors

Gulsoy Kirnap N,Kirnap M,Bascil Tutuncu N,Moray G,Haberal M

doi

10.1111/ctr.13730

subject

Has Abstract

pub_date

2019-12-01 00:00:00

pages

e13730

issue

12

eissn

0902-0063

issn

1399-0012

journal_volume

33

pub_type

杂志文章
  • Heparin-induced thrombocytopenia following pancreatectomy and islet auto-transplantation.

    abstract::We report the case of a female with chronic pancreatitis who underwent multiple surgical procedures leading to total resection of the pancreas with successful islet auto-transplantation. Postoperatively, she developed heparin-induced thrombocytopenia. Heparinization prior to islet infusion is currently recommended to ...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2005.00457.x

    authors: Rastellini C,Brown ML,Cicalese L

    更新日期:2006-03-01 00:00:00

  • Monitoring of cyclosporine levels in transplant recipients using self-administered fingerprick sampling.

    abstract::Use of C(2) monitoring for cyclosporine A (CsA) microemulsion results in improved clinical outcomes vs. trough (C(0)) monitoring. Logistical issues include accurate timing of the C(2) sample; requirement for sample dilution with most standard assay techniques; and inconvenience for patients. Recently, it has been show...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2005.00472.x

    authors: Yonan N,Martyszczuk R,Machaal A,Baynes A,Keevil BG

    更新日期:2006-03-01 00:00:00

  • The impact of intraoperative fluid management during laparoscopic donor nephrectomy on donor and recipient outcomes.

    abstract:BACKGROUND:Intraoperative fluid management during laparoscopic donor nephrectomy (LDN) may have a significant effect on donor and recipient outcomes. We sought to quantify variability in fluid management and investigate its impact on donor and recipient outcomes. METHODS:A retrospective review of patients who underwen...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.13542

    authors: Williams AM,Kumar SS,Bhatti UF,Biesterveld BE,Kathawate RG,Sung RS,Woodside KJ,Englesbe MJ,Alameddine MB,Waits SA

    更新日期:2019-06-01 00:00:00

  • Immunization by blood-type antigen in human immunoglobulin products before ABO-incompatible kidney transplantation.

    abstract::A 29-year-old man wanted to receive an ABO-incompatible kidney transplant. His blood type was O, and the donor, his father, was A1. After endoscopic splenectomy performed before kidney transplantation, the recipient developed a high fever and leukocytosis, and he was treated with antibiotics and 5 g of human immunoglo...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1046/j.1399-0012.2003.00144.x

    authors: Sawada T,Ando T,Sato S,Kubota K,Fuchinoue S,Teraoka S

    更新日期:2004-04-01 00:00:00

  • Clinical outcomes of liver transplantation for HBV-related hepatocellular carcinoma: data from the NIH HBV OLT study.

    abstract:BACKGROUND:Hepatitis B virus (HBV)-related hepatocellular carcinoma (HCC) is an indication for orthotopic liver transplantation (OLT) in patients with tumor stage within the United Network for Organ Sharing criteria. The number of patients listed for HBV-related HCC is increasing, while the number of patients listed fo...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2010.01349.x

    authors: Han SH,Reddy KR,Keeffe EB,Soldevila-Pico C,Gish R,Chung RT,Degertekin B,Lok A,NIH HBV OLT Study Group.

    更新日期:2011-03-01 00:00:00

  • Microemulsion technology in the reformulation of cyclosporine: the reason behind the pharmacokinetic properties of Neoral.

    abstract::Management of transplant patients receiving cyclosporine therapy is complicated by interpatient and intrapatient variability in pharmacokinetic parameters caused by the drug's unpredictable bioavailability. Cyclosporine, a highly lipophilic cyclic polypeptide, has recently been reformulated using a microemulsion deliv...

    journal_title:Clinical transplantation

    pub_type: 杂志文章,评审

    doi:

    authors: Ritschel WA

    更新日期:1996-08-01 00:00:00

  • Urinary nitrate excretion is increased in cardiac transplanted patients with acute graft rejection.

    abstract::Acute allograft rejection in patients after cardiac transplantation is associated with intense infiltration of inflammatory cells. It has been shown in animal studies that this inflammatory process is accompanied by an induction of nitric oxide syndrome (NOS) enzyme within the grafted organ. We sought to test the hypo...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:

    authors: Mügge A,Kurucay S,Böger RH,Bode-Böger SM,Schäfers HJ,Wahlers T,Frölich JC,Lichtlen PR

    更新日期:1996-06-01 00:00:00

  • Conversion from sirolimus to everolimus in kidney transplant recipients receiving a calcineurin-free regimen.

    abstract:BACKGROUND:Everolimus (EVL) and sirolimus (SRL) were introduced into immunosuppressive regimens, in an attempt to replace or reduce the dose of the nephrotoxic calcineurin inhibitors (CNI). In our institution, due to an administrative decision, conversion from SRL to EVL, was carried out, providing us the opportunity t...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2011.01446.x

    authors: Carvalho C,Coentrão L,Bustorff M,Patrício E,Sampaio S,Santos J,Oliveira G,Pestana M

    更新日期:2011-07-01 00:00:00

  • Parvovirus B19 infection in a liver transplant recipient: case report and review in organ transplant recipients.

    abstract::We report an adult liver transplant recipient with parvovirus B19 infection, presenting as chronic transfusion-dependent anemia ensuing 1 month post-transplantation. Both serum and bone marrow cells were positive for parvovirus B19 DNA detected by polymerase chain reaction. The bone marrow biopsy revealed erythroid hy...

    journal_title:Clinical transplantation

    pub_type: 杂志文章,评审

    doi:

    authors: Chang FY,Singh N,Gayowski T,Marino IR

    更新日期:1996-06-01 00:00:00

  • Quality of life assessment in renal transplant: review and future directions.

    abstract::We performed a systematic search of the literature using the MEDLINE (through 2006) database to identify the patient-reported outcome measures of quality of life (QOL) used most often in the renal transplant literature. After applying limits and reviewing references, we identified 338 renal transplant articles with a ...

    journal_title:Clinical transplantation

    pub_type: 杂志文章,评审

    doi:10.1111/j.1399-0012.2007.00784.x

    authors: Butt Z,Yount SE,Caicedo JC,Abecassis MM,Cella D

    更新日期:2008-05-01 00:00:00

  • Extracorporeal membrane oxygenation as a bridge vs. non-bridging for lung transplantation: A systematic review and meta-analysis.

    abstract::Whether extracorporeal membrane oxygenation (ECMO) as a bridge to lung transplantation (BTT) can achieve a similar survival to non-BTT remains controversial. We conducted this meta-analysis to compare the outcomes between ECMO BTT and non-BTT to facilitate better clinical decision-making. Seven databases were searched...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.14157

    authors: Wan X,Bian T,Ye S,Cai P,Yu Z,Zhu J,Zhang W

    更新日期:2020-11-22 00:00:00

  • Molecular assessment of antibody-mediated rejection in human pancreas allograft biopsies.

    abstract::Pancreas transplant longevity is limited by immune rejection, which is diagnosed by graft biopsy using the Banff Classification. The histological criteria for antibody-mediated rejection (AMR) are poorly reproducible and inconsistently associated with outcome. We hypothesized that a 34-gene set associated with antibod...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.14065

    authors: Roufosse C,Drachenberg C,Renaudin K,Willicombe M,Toulza F,Dominy K,McLean A,Simmonds N,de Kort H,Cantarovitch D,Scalea J,Mengel M,Adam B

    更新日期:2020-11-01 00:00:00

  • Efficacy and side-effects of cyclosporine dose monitoring with levels 6 h after the morning dose in heart transplant patients.

    abstract::The purpose of this study was to compare CsA dose monitoring with trough levels (T0) vs. levels obtained 6 h after the morning dose of CsA (T6), with respect to the incidence of acute rejection and renal dysfunction, and the cumulative dose, as well as the cost of CsA after heart transplantation. Twenty consecutive ad...

    journal_title:Clinical transplantation

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:

    authors: Cantarovich M,Besner JG,Fitchett DH,Latter DA

    更新日期:1997-10-01 00:00:00

  • Mothers of children evaluated for transplantation: stress, coping resources, and perceptions of family functioning.

    abstract::The stress, coping resources, and family functioning of 36 mothers of children awaiting transplantation were evaluated. Using standardized assessment techniques, we found that, overall, 20% of mothers reported clinically elevated stress responses and that parenting stress was higher for mothers of children evaluated f...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:

    authors: Rodrigue JR,Hoffmann RG 3rd,MacNaughton K,Graham-Pole J,Andres JM,Novak DA,Fennell RS

    更新日期:1996-10-01 00:00:00

  • De novo donor-specific HLA antibodies in heart transplantation: Do transient de novo DSA confer the same risk as persistent de novo DSA?

    abstract:BACKGROUND:It is estimated that 25%-35% of heart transplant recipients develop de novo donor-specific antibodies (dnDSA). One factor that appears to play a role in clinical outcomes is DSA persistence. The objective of this study was to determine the incidence of transient and persistent dnDSA in a Canadian heart trans...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.13416

    authors: Moayedi Y,Fan CS,Tinckam KJ,Ross HJ,McCaughan JA

    更新日期:2018-11-01 00:00:00

  • Rescue therapy with mycophenolate mofetil. The Mycophenolate Mofetil Renal Refractory Rejection Study Group.

    abstract::Research with human transplant recipients has shown that mycophenolate mofetil (MMF) is a powerful and selective immunosuppressant for maintenance therapy following renal transplantation. An additional body of work suggests that it might also be a valuable tool for arresting ongoing rejection episodes. One randomized,...

    journal_title:Clinical transplantation

    pub_type: 临床试验,杂志文章,多中心研究,随机对照试验

    doi:

    authors:

    更新日期:1996-02-01 00:00:00

  • Older living donors provide excellent quality kidneys: a single center experience (older living donors).

    abstract:BACKGROUND:The role of advanced age live donors remains controversial because of decline in glomerular filtration rate and perceived increased risks of perioperative complications. METHODS:A retrospective review of all live donor transplants performed from January 2000 to December 2003. RESULTS:Seventy-eight live don...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2004.00328.x

    authors: Johnson SR,Khwaja K,Pavlakis M,Monaco AP,Hanto DW

    更新日期:2005-10-01 00:00:00

  • Intra-operative cardiac arrests during liver transplantation - a retrospective review of the first 15 yr in Asian population.

    abstract::Although many report intra-operative cardiac arrests (ICAs) in liver transplantation (LT), the incidence, major causes, and outcome remain unclear. We aimed to investigate retrospectively, the incidence, nature, and outcome of ICA in Asian population and to identify risk factors for ICA. Consecutive 1071 LTs in an ins...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.12085

    authors: Lee SH,Gwak MS,Choi SJ,Shin YH,Ko JS,Kim GS,Lee SY,Kim MH,Park HG,Lee SK,Jeon HJ

    更新日期:2013-03-01 00:00:00

  • Foot problems in the diabetic transplant recipient.

    abstract::Diabetic transplant recipients are at a high risk for foot pathology leading to amputation. This retrospective study from 1/85 to 2/95 examines the risk of foot complications in a population (n = 340) of diabetic renal and combined renal/pancreas transplant recipients. All groups suffered high lower extremity amputati...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:

    authors: Kalker AJ,Pirsch JD,Heisey D,Sollinger HW,Belzer FO,Knechtle SJ,D'Alessandro AM

    更新日期:1996-12-01 00:00:00

  • Combination of pancreas volume and HbA1c level predicts islet yield in patients undergoing total pancreatectomy and islet autotransplantation.

    abstract::Islet yield is an important predictor of acceptable glucose control after total pancreatectomy with islet autotransplantation (TP-IAT). We assessed if pancreas volume calculated with preoperative MRI could assess islet yield and postoperative outcomes. We reviewed dynamic MRI studies from 154 adult TP-IAT patients (20...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.14008

    authors: Nanno Y,Wilhelm JJ,Heller D,Schat R,Freeman ML,Trikudanathan G,Kirchner VA,Pruett TL,Beilman GJ,Hering BJ,Bellin MD

    更新日期:2020-08-01 00:00:00

  • Sequential anastomosis of accessory renal artery to inferior epigastric artery in the management of multiple arteries in live related renal transplantation: a critical appraisal.

    abstract::In live related renal transplant program, management of multiple renal arteries (MRA) is technically demanding and used to be considered a relative contraindication because of increased risk of vascular and urologic complications. We present a retrospective analysis of the outcome of grafts with MRA and suggest certai...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1034/j.1399-0012.2001.150209.x

    authors: Kumar A,Gupta RS,Srivastava A,Bansal P

    更新日期:2001-04-01 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

  • High-dose busulfan and the risk of pulmonary mortality after autologous stem cell transplant.

    abstract::The non-relapse mortality of autologous stem cell transplant is low enough that the procedure has been extended to older patients with non-Hodgkin's lymphoma. We treated 537 non-Hodgkin's lymphoma patients with high-dose chemotherapy consisting of busulfan, cyclophosphamide, and etoposide followed by autologous stem c...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2006.00581.x

    authors: Kalaycio M,Pohlman B,Kuczkowski E,Rybicki L,Andresen S,Sobecks R,Bolwell B

    更新日期:2006-11-01 00:00:00

  • Training, work, and lifestyle of transplant physicians and surgeons in Germany.

    abstract:BACKGROUND:International data on training, work, and lifestyle of transplant physicians and surgeons are scarce. Such data might help in development of uniform education paths and provide insights for young clinicians interested in this field. This study aimed at the evaluation of these data in all transplant-associate...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.12786

    authors: Hoyer DP,Kaiser GM,Cicinnati V,Radunz S,Braun F,Greif-Higer G,Schulze M,Schmidt HJ,Paul A,Beckebaum S

    更新日期:2016-09-01 00:00:00

  • Social support and immunosuppressant therapy adherence among adult renal transplant recipients.

    abstract:BACKGROUND:The purpose of the study was to assess the relationship between social support and immunosuppressant therapy adherence among adult renal transplant recipients. METHODS:A cross-sectional, survey design was employed. Mailed questionnaires were used to collect data from 81 recipients, and included the Immunosu...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2009.01060.x

    authors: Chisholm-Burns MA,Spivey CA,Wilks SE

    更新日期:2010-05-01 00:00:00

  • Improving graft survival for patients undergoing liver transplantation.

    abstract::Liver transplant (LT) outcomes are reported to be improving in non-HCV recipients but not for those infected with HCV. Our aim was to evaluate graft survival and predictors of outcome in HCV and non-HCV patients before and after 2003. Patients with primary LT between February 1, 1998, and December 31, 2005, were inclu...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2011.01428.x

    authors: Dickson RC,Pungpapong S,Keaveny AP,Taner CB,Ghabril M,Aranda-Michel J,Satyanarayana R,Bonatti H,Kramer DJ,Nguyen JH

    更新日期:2011-05-01 00:00:00

  • Cardiac diastolic dysfunction in renal-transplant recipients is associated with increased circulating Adrenomedullin.

    abstract:BACKGROUND:Renal transplantation is an excellent therapeutic alternative for end-stage renal diseases. Nevertheless, the cardiac function is often impaired in renal-transplant patients (RTR) and importantly determines their prognosis. Adrenomedullin (ADM), a peptide involved in cardiovascular homeostasis, is believed t...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/j.1399-0012.2005.00486.x

    authors: Geny B,Ellero B,Chakfé N,Kretz JG,Brandenberger G,Piquard F

    更新日期:2006-05-01 00:00:00

  • Liver allocation: urgency of need or prospect of success? Ethical considerations.

    abstract::In German legislation and in Eurotransplant's practice of liver allocation, urgency of need is considered as the primary distribution criterion. However, at a certain stage, the "sickest-first" principle is regarded as counterproductive as the performance status of these patients receiving an organ is on average criti...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1111/ctr.12154

    authors: Bobbert M,Ganten TM

    更新日期:2013-07-01 00:00:00

  • Interstitial alterations in renal cortex in acute tubular necrosis (ATN) post-renal transplantation and in patients with ATN not related to renal transplant.

    abstract:BACKGROUND:Abnormalities of renal function with long-term implications can persist after acute tubular necrosis (ATN), probably because of permanent loss of nephrons. Residual areas of fibrosis are also observed in the renal cortex post-ATN. In this study, we investigate the interstitial alterations post-ATN using hist...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1046/j.1399-0012.2003.00140.x

    authors: Moyses Neto M,Costa RS,Volpini RA,Garcia TM,Rodrigues FF,Coimbra TM

    更新日期:2004-04-01 00:00:00

  • Influence of dialysis on post-transplant events.

    abstract:INTRODUCTION:We examined the effect of haemodialysis (HD) or peritoneal dialysis (PD) on acute rejection, delayed graft function (DGF), graft and patient survival after cadaveric renal transplantation. MATERIALS AND METHODS:We carried out a retrospective analysis of 325 patients (cyclosporin [CyA]-based therapy) who h...

    journal_title:Clinical transplantation

    pub_type: 杂志文章

    doi:10.1034/j.1399-0012.2002.00069.x

    authors: Joseph JT,Jindal RM

    更新日期:2002-02-01 00:00:00