Abstract:
:LT is an effective therapeutic option for a variety of IEM. This approach can significantly improve the quality of life of patients who suffer from severe disease manifestations and/or life-threatening metabolic decompensations despite medical/dietary management. Due to the significant risks for systemic complications from surgical stressors, careful perioperative management is vital. Even after LT, some disorders require long-term dietary restriction, medical management, and monitoring of metabolites. Successful liver transplant for these complex disorders can be achieved with disease- and patient-specific strategies using a multidisciplinary approach. In this article, we review indications, complications, perioperative management, and long-term follow-up recommendations for IEM that are treatable with LT.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Oishi K,Arnon R,Wasserstein MP,Diaz GAdoi
10.1111/petr.12741subject
Has Abstractpub_date
2016-09-01 00:00:00pages
756-69issue
6eissn
1397-3142issn
1399-3046journal_volume
20pub_type
杂志文章,评审abstract::Retransplantation is often a necessity for children with end-stage renal disease (ESRD), as kidney graft survival is still not infinite. If a suitable live donor is present, the current policy is to use the live donor first, in order to obtain excellent long-term outcome and to prevent human leucocyte antigen (HLA) se...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2001.00049.x
更新日期:2001-06-01 00:00:00
abstract::The majority of transplant centers around the world face an ethical debate whether to retransplant a young non-adherent patient. Non-adherence to lifelong immunosuppressants presents a significant risk for graft loss, yet rates remain consistently high. Despite a number of these patients presenting for retransplantati...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2011.01633.x
更新日期:2012-02-01 00:00:00
abstract::We report a case of EBV encephalitis in a seven-yr-old child with Ph+ ALL. Two months after an allogeneic HSCT from his HLA mismatched mother, the patient showed an altered sensorium, generalized seizures, and a left hemiparesis. Brain MRI demonstrated multiple lesions highly suggestive for viral encephalitis. Blood a...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12386
更新日期:2015-02-01 00:00:00
abstract::Children receiving HCT loose protective immunity to vaccines received pre-HCT. Therefore, revaccination post-HCT is of major importance. In Denmark, a vaccination schedule with fewer doses post-HCT has been used, including two doses for diphtheria, tetanus, polio, measles, mumps, and rubella, and one dose only for Hae...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13599
更新日期:2020-02-01 00:00:00
abstract::Biliary atresia is defined as partial or total obliteration of the extra-hepatic bile ducts. In advanced cases, liver transplantation (LTx) is considered the most appropriate treatment. This report describes a female patient whose biliary atresia and subsequent cirrhosis required LTx at 1 yr of age. Macroscopic inspec...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2001.t01-1-00020.x
更新日期:2001-02-01 00:00:00
abstract::Although TEG directs effective resuscitation in adult surgical patients, pediatric data are lacking. We performed a retrospective comparative review of the effect of TEG on blood product utilization and outcomes following pediatric liver transplantation in 38 patients between 2008 and 2014. Diagnoses, laboratory value...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13176
更新日期:2018-06-01 00:00:00
abstract::Combined enzyme replacement therapy (ERT) and stem cell transplant (SCT) were done for a two year old boy with severe Hurler syndrome(HS) with the aim to decrease transplant related complications. He tolerated both the procedures well without any major complications. Urine glycosaminoglycans (GAGs) decreased post-tran...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00720.x
更新日期:2007-08-01 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::Intestinal transplant recipients are at risk of micronutrient deficiency due to the slow process of post-transplant adaptation. Another contributing factor is calcineurin inhibitor-induced renal tubular dysfunction. Patients are typically supplemented with micronutrients during PN; however, the risk of deficiency may ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12132
更新日期:2013-11-01 00:00:00
abstract::Non-adherence to immunosuppressant medications is an important risk factor for graft dysfunction. To evaluate the effectiveness of adherence-enhancing interventions, we reviewed adherence intervention studies in solid organ transplant recipients (all ages). Using the following databases: PsycINFO, PubMed, Scopus, and ...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.13086
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:In adults, the time of day for LT does not affect post-transplant outcomes. Whether this is true or not in children is unknown. In this study, we aimed to evaluate if weekend and weeknight liver transplants are associated with worse patient and graft survival in children. METHODS:We interrogated the UNOS da...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13826
更新日期:2020-12-01 00:00:00
abstract::There are few studies on invasive pneumococcal disease in pediatric transplant recipients. Given this fact plus the advent of pneumococcal conjugate vaccines, we conducted a retrospective study at a major pediatric transplant center. The objectives were to determine the incidence and outcomes of invasive pneumococcal ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00275.x
更新日期:2005-04-01 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::Prematurity and very low birthweight have often been considered relative contraindications to neonatal organ donation. Organ procurement from neonatal donors is further complicated by unclear guidelines regarding neonatal brain death. We report a successful case of multivisceral transplantation using a graft from a 10...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2012.01739.x
更新日期:2012-12-01 00:00:00
abstract::Epstein-Barr virus (EBV)-associated post-transplant lymphoproliferative disease (PTLD) is a serious complication of organ and bone marrow transplantation. The importance of EBV matching between recipient and donor remains unclear. Between October 1987 and December 1997, 64 pediatric cardio-pulmonary transplants were p...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.1999.00019.x
更新日期:1999-05-01 00:00:00
abstract::We report on survival, rejection, lymphoma and renal function following cardiac transplant using a steroid-free maintenance immunosuppressive regimen. We have performed 73 cardiac transplants in 71 children under 16 yr of age in the last 12 yr. There were eight perioperative and four late deaths giving actuarial survi...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2003.00014.x
更新日期:2003-02-01 00:00:00
abstract::RVIs are a significant cause of morbidity and mortality in immunocompromised children. We analyzed the characteristics and outcomes of infection by four respiratory viruses (RSV, adenovirus, influenza, and parainfluenza) treated at a pediatric tertiary care hospital in a retrospective cohort of patients who had receiv...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12022
更新日期:2013-03-01 00:00:00
abstract::DD kidney allocation protocols may influence timing of transplantation and graft quality for pediatric recipients. This study aimed to evaluate the effects of these protocols, including pediatric priority, on waiting time on dialysis, transplant type, donor age, and HLA matching according to state of transplant in Aus...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12544
更新日期:2015-09-01 00:00:00
abstract::Despite advances in therapy for acute leukemia, relapse continues to be the major cause of treatment failure. Hematopoietic stem cell transplant can rescue some patients after relapse, but the ability to escalate the intensity of preparative regimens is limited by toxicity to normal organs. Radiolabeled monoclonal ant...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1034/j.1399-3046.7.s3.14.x
更新日期:2003-01-01 00:00:00
abstract::eHAT is one of the most dreaded post-LT complication. Treatment approaches include retransplantation, revascularization, or observation. Systemic thrombolytic therapy is used in pediatric patients with thromboembolic events. However, there is no previous study reporting on the use of systemic r-tPA to treat eHAT. The ...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13902
更新日期:2020-10-27 00:00:00
abstract::There are few reports on the clinical significance of MRD before HSCT in pediatric ALL. We retrospectively analyzed the clinical significance of FCM-based detection of MRD (FCM-MRD) before allogeneic HSCT in pediatric ALL. Of 38 pediatric patients who underwent allogeneic HSCT for the first time between 1998 and 2014,...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12732
更新日期:2016-08-01 00:00:00
abstract::We studied restoration of the coagulation and fibrinolysis system in pediatric patients following liver transplantation and biomarkers of blood coagulation and fibrinolysis for suspecting the occurrence of acute cellular rejection. Coagulation activity recovered rapidly within two days following transplantation, but i...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01248.x
更新日期:2010-05-01 00:00:00
abstract::Cryptosporidium is an intracellular protozoa that can cause gastroenteritis in humans. In immunocompromised hosts, infection can be severe, leading to life-threatening persistent diarrhea. There is limited experience in treating this infection in solid organ transplants. Although newer drugs active against Cryptospori...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2006.00593.x
更新日期:2007-02-01 00:00:00
abstract::Psychomotor development in pediatric liver transplant (LT) recipients depends on several factors. Our aim was to evaluate the importance of parental involvement and family dynamics on psychomotor development by assessing (i) children and parents individually, (ii) the parent-child relationship, and (iii) the correlati...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12080
更新日期:2013-06-01 00:00:00
abstract::PTLD is a major complication after transplantation. Treatment options for PTLD are not standardized, usually sequential, starting with reduction in immunosuppression. Recently, we have used a dual combination of rituximab and reduced dose chemotherapy (R/C) directly after failed RI. We retrospectively identified 30 pe...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2010.01370.x
更新日期:2010-11-01 00:00:00
abstract::Historically inability to achieve portal inflow to the liver allograft operatively was felt to be a contraindication to orthotopic liver transplantation (OLTx). Cavoportal hemi transposition has been utilized more recently in adult OLTx recipients but rarely in pediatric recipients. Here we report the case of a 10-mon...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00196.x
更新日期:2004-08-01 00:00:00
abstract::CNL is a rare myeloproliferative disorder frequently seen in older adults. A significant proportion of patients show progression to AML. Here, we report the case of a patient with FA who was monitored for leukopenia but who developed leukocytosis during the follow-up and was diagnosed with CNL probably after an acquir...
journal_title:Pediatric transplantation
pub_type:
doi:10.1111/petr.13199
更新日期:2018-08-01 00:00:00
abstract::PPCA has historically been considered detrimental to donor quality in LT, but transplantation of grafts from this group of donors is now routine. Our study aims to evaluate the outcomes associated with use of donors with a history of PPCA in the pediatric population. This study is a single-center retrospective analysi...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13701
更新日期:2020-08-01 00:00:00
abstract::Neoplasms in children after organ transplantation are related to the type and intensity of immunosuppression and the donor-recipient serostatus, especially in relation to the Epstein-Barr virus. The patient was a two-yr-old female child with biliary atresia who underwent a liver transplantation from a female cadaver d...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/j.1399-3046.2012.01711.x
更新日期:2012-12-01 00:00:00
abstract::We retrospectively analyzed the effects of recombinant human growth hormone (rhGH) in a Belgian population of 36 short children with renal allografts. Seven children were dropped from the growth study: 1 had skeletal dysplasia and in 6 cases rhGH was given for less than 1 yr (1 died, 1 developed genu valgum, 2 were no...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:
更新日期:1997-11-01 00:00:00