Abstract:
:The aim of this study was to re-evaluate the indications and timing of LT for WD. From 2000 to 2009, eight patients with WD who had been referred to our institution for LT were enrolled in this study. The mean patient age was 15.9 yr (range, 7-37 yr). Four patients could not receive LT, because there were no available donors. All four patients were treated with chelating agent medication. Three of them (two of two patients with fulminant WD and one of two with cirrhotic WD) who did not undergo LT are still alive and doing well with stable liver functional tests. Only one of the patients with cirrhotic WD who did not undergo LT died of hepatic failure. Even among the four patients who underwent LT, one with fulminant WD recovered from hepatic encephalopathy with apheresis therapy and chelating agent. He later required LT because of severe neutropenia from d-penicillamine. The other three patients who underwent LT recovered and have been doing well. Some of the patients with WD can recover and avoid LT with medical treatment. Even when WD has progressed liver cirrhosis and/or fulminant hepatic failure at the time of diagnosis, medical treatment should be tried before considering LT.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Ohya Y,Okajima H,Honda M,Hayashida S,Suda H,Matsumoto S,Lee KJ,Yamamoto H,Takeichi T,Mitsubuchi H,Asonuma K,Endo F,Inomata Ydoi
10.1111/petr.12085subject
Has Abstractpub_date
2013-06-01 00:00:00pages
369-73issue
4eissn
1397-3142issn
1399-3046journal_volume
17pub_type
杂志文章abstract:INTRODUCTION:Myeloablative conditioning before allogeneic HSCT during childhood exposes to serious long-term complications, especially gonadal dysfunction. Pubertal issues are less described than other post-HSCT sequelae in childhood. METHODS:Pubertal development and biological gonadal parameters were assessed in a re...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13773
更新日期:2020-09-01 00:00:00
abstract::AML with the RAM immunophenotype is associated with extremely poor prognosis. We report a rare case of monozygotic twins presenting simultaneously at the age of 2 years with RAM AML. Each twin underwent a myeloablative 7/10 unrelated umbilical cord blood transplant. Pretransplant Twin A's bone marrow was negative for ...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13291
更新日期:2018-12-01 00:00:00
abstract::Graft-vs-host disease (GVHD) is one of the most difficult problems in stem cell transplantation. It is best considered in the context of the normal immune response. The role of the immune system is to identify and eliminate foreign antigens. In the case of GVHD, the antigens are minor histocompatibility antigens (mHA)...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2005.00441.x
更新日期:2005-12-01 00:00:00
abstract::Clinical grading of GI involvement during acute GVHD remains a challenging issue, especially in children. Plasma citrulline, a non-protein amino acid selectively produced and released by enterocytes, is a suitable surrogate endpoint for small intestinal epithelial cell mass, irrespective of the underlying cause of cel...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12128
更新日期:2013-11-01 00:00:00
abstract::Post-transplantation biliary strictures occur in 5-15% of the pediatric liver transplant patients and are conventionally managed by interventional radiological techniques. Failure of this treatment leads to reoperation and sometimes to retransplantation. Herein, we describe a surgical approach and interventional radio...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00212.x
更新日期:2004-10-01 00:00:00
abstract::ADV is frequently seen in our pediatric SOT population. It presents in a variety of clinical presentation and can cause severe disease. In this population, there are very few studies to determine the safety of CDV as a potential therapeutic agent. We present the findings of our retrospective study evaluating the effic...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13231
更新日期:2018-06-03 00:00:00
abstract::Secondary failure of platelet engraftment occurs in 20% of patients undergoing allogeneic HSCT and is associated with poor outcome. Currently, there are no guidelines for treatment of late thrombocytopenia and platelet transfusion is the mainstay of treatment. Here, we describe the use of Eltrombopag to treat secondar...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13017
更新日期:2017-09-01 00:00:00
abstract::The examination of predictors of non-adherence in transplantation populations offers the potential to identify high-risk individuals and concomitantly generate effective adherence-promoting interventions. The present paper aggregated quantitative estimates of various correlates of treatment adherence in studies of ped...
journal_title:Pediatric transplantation
pub_type: 杂志文章,meta分析
doi:10.1111/j.1399-3046.2007.00864.x
更新日期:2008-09-01 00:00:00
abstract:BACKGROUND/AIM:Several PK studies have shown that most pediatric patients may require higher doses on a mg/kg basis compared to adults to attain similar therapeutic trough concentrations. The aim of this study was to compare the efficacy and safety of three times daily to twice a day dosing of tacrolimus in pediatric k...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13733
更新日期:2020-09-01 00:00:00
abstract::HSCT-associated thrombotic microangiopathy (TA-TMA) is a severe complication with a poor prognosis. Recently, it has been reported that complement system dysregulation, such as CFH autoantibodies and deletions CFH-related genes 3 and 1, induced TA-TMA. In addition, C4d-positive renal arterioles are both a good marker ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12450
更新日期:2015-06-01 00:00:00
abstract::This retrospective case series reviews our center's experience with sirolimus and a CNI as alternative therapy for the treatment of PTAH. It also characterizes regulatory T cells (Tregs) in PTAH. LT recipients with PTAH who had received or were receiving treatment with sirolimus were retrospectively identified (n = 12...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2012.01648.x
更新日期:2012-03-01 00:00:00
abstract::Genetic variation has been the mainstay of hematopoietic stem cell transplantation since the first transplants were attempted. A significant expansion of genetic knowledge is under way, as the draft sequence of the human genome is annotated. Hematopoietic stem cell transplantation is a field that will greatly benefit ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.7.s3.4.x
更新日期:2003-01-01 00:00:00
abstract::We report the occurrence of epididymitis and orchitis 1 week after the onset of Listeriosis in an 11-month-old boy receiving an orthotopic liver transplantation for biliary atresia. Immunologic implications of Listeria monocytogenes-induced testicular inflammation are discussed, and the potential role of immunosuppres...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2000.00113.x
更新日期:2000-05-01 00:00:00
abstract::Antibody-mediated rejection (AMR) has recently been recognized as a significant and unique form of rejection that is not amenable to treatment with standard immunosuppressive medications aimed at modification of T-cell function. Recent interest in AMR and the role of B cells in rejection has been aided by the concomit...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2005.00363.x
更新日期:2005-06-01 00:00:00
abstract::The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR® ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was review...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13048
更新日期:2017-12-01 00:00:00
abstract::Much is still unknown about LUT function after receiving renal graft. Graft function was the main focus of different studies discussing the same issue. However, these studies ignored the effects of the graft on lower tract function and more demand for bladder cycling and growth of the child. Therefore, we aimed at eva...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13735
更新日期:2020-09-01 00:00:00
abstract::In the pediatric population, little is known on de novo DSA development, its impact on graft function, and association with suboptimal IS. We assessed the prevalence of de novo DSA in the Vienna cohort of 40 renal transplanted children and adolescents and prospectively followed its association with clinical parameters...
journal_title:Pediatric transplantation
pub_type: 临床试验,杂志文章
doi:10.1111/petr.12707
更新日期:2016-06-01 00:00:00
abstract::Pharmacokinetic monitoring of CsA is unsatisfactory, because at comparable CsA blood concentrations, the frequency and severity of adverse effects vary considerably among patients. We have therefore recently developed a precise, reliable, and robust whole-blood pharmacodynamic assay that measures the suppression of Cs...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01354.x
更新日期:2010-11-01 00:00:00
abstract::This study evaluated changes in growth parameters after pediatric heart transplantation and identified factors associated with the changes after pediatric heart transplantation (OHT). We retrospectively evaluated the somatic growth of 46 children <11 yr of age who underwent OHT for changes in weight, height, and BMI. ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00826.x
更新日期:2008-06-01 00:00:00
abstract::Mortality is the highest of any solid organ in pediatric patients awaiting heart transplantation. Strategies to increase the donor pool are needed if survival to transplant is to improve. There can be reluctance to accept pediatric hearts for transplantation if the donor has received cardiopulmonary resuscitation (CPR...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2011.01565.x
更新日期:2011-11-01 00:00:00
abstract::MCs are important effector cells in a broad range of immune responses. Their role in liver allograft rejection is not clear. Twenty-one liver transplant recipients (mean age +/- s.d.; 10.2 +/- 4.1 yr) who experienced a rejection episode are included in this study. Biopsy specimens from normal livers (allograft biopsy ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00819.x
更新日期:2008-05-01 00:00:00
abstract::The adjusted relative risk (aRR) for development of post-transplant lymphoproliferative disorder (PTLD) is higher in kidney transplant recipients receiving monoclonal antibody induction therapy, but the aRR between the different available polyclonal agents has not been investigated in detail. We analyzed data from the...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00361.x
更新日期:2005-10-01 00:00:00
abstract::Despite improvements in the prevention and treatment of acute renal allograft rejection, the long-term survival of renal transplants has not increased. Immunologic and non-immunologic factors contribute to the gradual deterioration of graft function and to the histologic lesion characterized by vascular and interstiti...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1034/j.1399-3046.1999.00044.x
更新日期:1999-11-01 00:00:00
abstract:BACKGROUND:Little data exist on immunosuppressive drug absorption in children with short bowel syndrome and intestinal failure associated liver disease (SBS-IFALD). AIM:To evaluate the absorption of immunosuppressive medications in children with SBS-IFALD undergoing isolated liver transplantation (iLTx). METHODS:A re...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2006.00504.x
更新日期:2006-09-01 00:00:00
abstract::The clinical significance of parvovirus B19 infection in pediatric solid-organ and bone marrow transplanted patients is unclear. The overall prevalence of parvovirus B19 infection in these patients is about 1-2% during the first year after transplantation. The most common symptom is anemia, but leukopenia and thromboc...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1034/j.1399-3046.2001.00035.x
更新日期:2001-10-01 00:00:00
abstract::Ingestion of rare-earth magnet beads in children has been a public health concern. The potential risk of swallowing multiple magnets is related to magnet attraction to each other, resulting in serious gastrointestinal complications, such as entero-enteric fistula formation, peritonitis, bowel ischemia or necrosis, bow...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13915
更新日期:2020-11-20 00:00:00
abstract::There is a shortage of pediatric donor hearts for waitlisted children, and yet nearly 50% of organs offered are not transplanted. Donor quality is often cited as a reason for declining organs offered from donors infected with influenza, presumably due to concern about disease transmission at transplant leading to seve...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13585
更新日期:2019-12-01 00:00:00
abstract::Vascular complications post-renal transplantation are not very common and can be associated with increased risk of graft loss. We report an unusual case of a young, female teenager with asymmetrical leg swelling. After one month of transplantation, the patient developed a rise in serum creatinine with right leg swelli...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.00941.x
更新日期:2008-09-01 00:00:00
abstract::IMI are emerging as an important cause of mortality and morbidity among the growing number of immunocompromised children. A retrospective chart review was performed in all patients with a proven diagnosis of IMI over an eight-yr period (1997-2004) at The Hospital for Sick Children, Toronto, Canada to document the inci...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.01056.x
更新日期:2009-08-01 00:00:00
abstract::In many countries, adolescents can choose to register a deceased organ donation wish when they apply for a driver's license. They often receive education about deceased organ donation in order to make an informed choice. The objective of this review was to describe the effectiveness of school-based educational program...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.12045
更新日期:2013-03-01 00:00:00