Abstract:
:Liver transplant is a treatment option for patients with MMA-emia. While this therapy does not bring about a complete cure, it is expected to prolong survival and improve the QOL of patients. The aim of this study was to evaluate the significance of LDLT for patients with MMA-emia in Japan. Clinical information on 13 patients with MMA-emia who underwent LDLT was acquired using a self-developed questionnaire sent to the doctors who provided medical care to patients with MMA-emia after LDLT. Almost all of the patients continued on a protein-restricted diet, and the number of acidosis attacks had significantly decreased. Physical growth had recovered to within the normal range by 2.5 years after LDLT, especially in patients who underwent LDLT before the age of 1 year. The average propionyl carnitine (C3) level had significantly decreased after LDLT, and the DQs had not worsened. Liver transplant should be performed for MMA-emia in early life. This can be expected to maintain neurological development and improve the growth and QOL of patients. However, LDLT is not a curative treatment for MMA-emia. A protein-restricted diet should be continued, and renal function should be monitored closely, with consideration of a renal transplant.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Sakamoto R,Nakamura K,Kido J,Matsumoto S,Mitsubuchi H,Inomata Y,Endo Fdoi
10.1111/petr.12804subject
Has Abstractpub_date
2016-12-01 00:00:00pages
1081-1086issue
8eissn
1397-3142issn
1399-3046journal_volume
20pub_type
杂志文章abstract::Paediatric HR T-cell ALL demonstrates dismal prognosis with chemotherapy, and poor outcomes could be improved with allo-SCT. HID-SCT is an almost immediately available choice; however, few studies have focused on the outcomes of HID-SCT for paediatric HR T-ALL. Forty-eight consecutive HR T-ALL children who underwent H...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12704
更新日期:2016-06-01 00:00:00
abstract::Administration of hematopoietic growth factors, with or without chemotherapy, can augment progenitor cell numbers available for collection. The dose of granulocyte colony stimulating factor (G-CSF) used for mobilization of peripheral blood progenitor cells (PBPC) is controversial, and doses between 5 and 32 microg/kg/...
journal_title:Pediatric transplantation
pub_type: 临床试验,杂志文章,随机对照试验
doi:
更新日期:1998-05-01 00:00:00
abstract:BACKGROUND:Scientific advancements are occurring in cardiac xenotransplantation (XTx). However, there have been religious and social concerns surrounding this allotransplantation alternative. The purpose of this study was to explore the acceptance of XTx among stakeholders of the congenital heart disease (CHD) communit...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13851
更新日期:2020-10-06 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::Transplantation is the accepted mode of treatment for patients with end-stage organ disease affecting the heart, lungs, kidney, pancreas, liver and intestine. Long-term outcomes have significantly improved and the aim of management is no longer only long-term survival, but also focuses on quality of life especially in...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.12333
更新日期:2014-11-01 00:00:00
abstract::UCBT recipients with TM are at high risk of EF related to low number of stem cells and prior alloimmunization after multiple blood transfusions. Here, we evaluated the safety and efficacy of double-unit UCBT using TT-containing conditioning regimens in TM. Retrospective analysis of children who underwent double-unit U...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13901
更新日期:2020-11-02 00:00:00
abstract::Portal vein aneurysms are very rare and represent <3% of all venous aneurysms. They can be congenital or acquired. Most patients do not have liver disease at diagnosis. Although uncommon, portal vein aneurysm has been described after liver transplant. We report the case of a six-yr-old girl who presented with an aneur...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2012.01782.x
更新日期:2013-02-01 00:00:00
abstract::Recurrent and de novo disease is an increasing problem and is known to negatively impact transplant graft survival. Immunosuppressive medications have not had an impact on the prevalence of recurrent and de novo disease. Renal Allograft Disease Registry (RADR) was established to study the prevalence, impact and risk f...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00180.x
更新日期:2004-08-01 00:00:00
abstract::During the past 50 yr, intensive studies into the use of hematopoietic cell transplantation (HCT) for therapy of cancer and non-malignant hematologic diseases have changed this treatment modality from one that was thought to be plagued by insurmountable complications to one that is now standard therapy for some diseas...
journal_title:Pediatric transplantation
pub_type: 历史文章,杂志文章
doi:10.1111/j.1398-2265.2004.00203.x
更新日期:2004-06-01 00:00:00
abstract::Success after solid organ transplantation is dependent on the proper balance of immunosuppression to prevent rejection of the allograft while limiting the risk of developing infections and malignancy. We present a 9-year-old girl, remote from transplant, who presented with airway plaque after a change in immunosuppres...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13046
更新日期:2017-11-01 00:00:00
abstract::Since December 1995, pediatric renal transplant recipients in our unit have received a DMSA scan as soon as possible post-transplant in order to provide a baseline for comparison in the event of subsequent complications. We retrospectively reviewed the case notes and DMSA scans of the 45 patients who underwent a scan ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1046/j.1399-3046.2003.00073.x
更新日期:2003-12-01 00:00:00
abstract:BACKGROUND:ILNEB constitute an autosomal recessive disorder caused by homozygous or compound heterozygous mutation of the gene for the ITGA3. To date, 8 ILNEB patients have been reported, but all 6 neonatal-onset ILNEB patients suffered early death within 2 years. The most common cause of death among previously reporte...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13971
更新日期:2021-01-20 00:00:00
abstract::Effective antibody removal using PE, DFPP and IA has led to increased access to live donor organs through ABOi RT for patients with chronic kidney disease. However, there have been no head-to-head comparator studies between these modalities, and the choice of technique is usually influenced by cost and institutional p...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12227
更新日期:2014-05-01 00:00:00
abstract::Mycoplasma pneumoniae has rarely been reported in renal transplant recipients. We present the case of a 10-yr-old boy with a six-month history of chronic cough, recurrent pyrexia, and weight loss three yr after RTx. The patient's post-transplant course was complicated by recurrence of NS that resolved with plasmaphere...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01130.x
更新日期:2010-05-01 00:00:00
abstract:BACKGROUND:ADH is a rare and potentially fatal complication following LT. In this study, a systematic review was completed to identify risk factors which may contribute to ADH. METHODS:Transplant databases at three LT programs were reviewed. Four pediatric and zero adult cases were identified. Next, a systematic revie...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13296
更新日期:2018-12-01 00:00:00
abstract::A major risk faced by bone-marrow and solid organ transplant patients is the development of post-transplant lymphoproliferative disease or post-transplant lymphoma (PTLD). In pediatric transplantation, PTLD onset is often associated with a rapid rise in Epstein-Barr virus (EBV) load in peripheral blood mononuclear cel...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2003.00090.x
更新日期:2003-08-01 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::We present the unique case of a pediatric patient who received chemotherapy for a diagnosis of CD, while mechanically supported with a Berlin EXCOR LVAD secondary to restrictive cardiomyopathy. A four-yr-old previously healthy male with restrictive cardiomyopathy required MCS after cardiac arrest but was diagnosed wit...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12398
更新日期:2015-02-01 00:00:00
abstract::The clinical utility of tacrolimus monitoring in adults has been well documented. The present study compared tacrolimus monitoring in a pediatric population of 34 liver transplant patients in four US centers with an adult population of 111 patients in six US centers. Subjects (adult and pediatric) were evaluated, at d...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1046/j.1397-3142.2000.00000.x
更新日期:2001-04-01 00:00:00
abstract::Many pediatric centers utilize a variety of protocols including preemptive plasmapheresis to prevent the recurrence of FSGS post-transplant. But the effectiveness of this expensive, time-consuming process of plasmapheresis in the prevention of FSGS recurrence is still unclear. We retrospectively reviewed all pediatric...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13154
更新日期:2018-05-01 00:00:00
abstract::Depression is common in pediatric chronic kidney disease (CKD) patients. Depression is associated with inferior long-term outcomes. There is a paucity of studies that evaluate depression and possible associated factors in children and adolescents requiring renal replacement therapy (RRT). Cross-sectional study using C...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13591
更新日期:2019-12-01 00:00:00
abstract::Although de novo DSA are associated with inferior graft survival, there are no effective strategies to prevent their formation. Underexposure to MPA (prodrug: MMF) also contributes to rejection rates early after transplantation, but the effect of this phenomenon on the formation of DSA long-term post-transplantation i...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12637
更新日期:2016-02-01 00:00:00
abstract::The purpose of this study was to assess the inter-rater reliability of the P-TRI, a 17-item instrument developed to identify risk factors associated with poor treatment adherence in pediatric solid organ transplant candidates. Because factors influencing treatment adherence may vary with age, the 89 subject samples we...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01428.x
更新日期:2011-03-01 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::PTLD is a serious complication of both solid organ and BMT. This study assessed whether (18) F-FDG PET, when added to CT scan, had additional value in the initial evaluation of PTLD in pediatric patients and whether PET/CT at baseline can reliably guide biopsy. This retrospective study evaluated 34 consecutive pediatr...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12611
更新日期:2015-12-01 00:00:00
abstract:: FSGS has a high recurrence rate after renal transplantation. To examine the effects of the use of preemptive and post-transplant PP on recurrence and graft outcome, we conducted a retrospective study on 34 pediatric patients (mean age 13±5 yr) with biopsy-proven pretransplant FSGS and who underwent a renal transplan...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2011.01478.x
更新日期:2011-08-01 00:00:00
abstract::This report describes a successful renal Tx in a patient with chronic renal failure, caused by dysplastic kidneys, who received a cadaveric kidney with post-infectious glomerulonephritis. Sequential renal biopsies were performed at 12 h before Tx, and at 1 h and on days 8 and 58 post-Tx. Post-operative hematuria disap...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2000.00076.x
更新日期:2000-02-01 00:00:00
abstract::Omenn's syndrome is a rare inherited variant of SCID. It is inevitably fatal, unless treated by bone marrow or stem cell transplantation. However, treatment-related complications and graft rejection are major obstacles to the success of transplantation. In this report, we describe an eight-month-old baby with Omenn's ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01413.x
更新日期:2012-03-01 00:00:00
abstract::This prospective observational study analyzed the pharmacokinetics of busulfan in Japanese children and evaluated the predicting accuracy of previous pediatric PPK models of busulfan. This study enrolled five patients (aged 2-12 years, BW 14-48 kg) receiving a busulfan-based conditioning regimen for hematopoietic stem...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13696
更新日期:2020-06-01 00:00:00
abstract::Although the majority of pediatric solid organ transplant centers in the United States employ psychosocial criteria to assess the suitability of potential transplant candidates, there are no standardized pretransplant psychosocial assessment measures. Assessment scales that have been developed were designed for adult ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00785.x
更新日期:2008-02-01 00:00:00