Abstract:
:aHUS is caused by the over-activation and dysregulation of the alternative complement pathway. Data regarding outcomes of pediatric aHUS patients after kidney transplantation are still very scarce. Accordingly, the aim of this study was to describe the clinical findings and outcomes of pediatric aHUS patients after renal transplantation. This is a retrospective, multicenter study including 12 patients from the national registry system. Among the 12 patients, eight had received prophylactic eculizumab and none of those patients (except one) had experienced aHUS recurrence during a median follow-up period of 58.5 (min-max, 4-94) months. Although eculizumab had been started on the day before transplantation in one of them, aHUS recurrence occurred during the transplantation procedure. Eculizumab had been stopped in only one patient who had no complement gene mutation after 35 months of therapy, and recurrence had not been observed during the 19 months of follow-up. In three patients, maintenance doses had been spaced out without any recurrence. One additional patient with anti-CFH antibody received only two doses of eculizumab for transplantation and had been followed for 46 months without aHUS recurrence. The remaining three patients had not received anti-C5 therapy and none of those patients experienced aHUS recurrence during a median follow-up period of 21 (min-max, 9-42) months. Prophylactic eculizumab is a safe and effective treatment for the prevention of aHUS recurrence. Eculizumab interval prolongation, discontinuation, and transplantation without eculizumab prophylaxis can be tried in selected patients with close follow-up.
journal_name
Pediatr Transplantjournal_title
Pediatric transplantationauthors
Özçakar ZB,Ozaltin F,Gülhan B,Çomak E,Parmaksız G,Baskın E,Topaloğlu R,Kasap Demir B,Canpolat N,Yuruk Yildirim Z,Demircioğlu Kılıç B,Yüksel S,Söylemezoğlu Odoi
10.1111/petr.13914subject
Has Abstractpub_date
2020-11-20 00:00:00pages
e13914eissn
1397-3142issn
1399-3046pub_type
杂志文章abstract::SAB assays have increased the sensitivity and specificity to detect HLA alloantibodies, but there is uncertainty about the clinical relevance of SAB-positive alloantibodies when the FCXM is negative. We performed a retrospective study to evaluate the clinical significance of SAB-detected DSA in 82 pediatric recipients...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01281.x
更新日期:2010-09-01 00:00:00
abstract::Autoimmune and allergic diseases cause morbidity and diminished quality of life in pediatric organ transplant recipients. We hypothesize that younger age at transplantation and immunosuppression regimen play a role in the development of immune-mediated disease following heart transplant. A single institution retrospec...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12205
更新日期:2014-03-01 00:00:00
abstract::Acute liver failure (ALF) is a rare but often fatal disorder in childhood. Its aetiology includes infections, toxins, metabolic disorders, infiltrative diseases, autoimmune hepatitis, ischaemia, irradiation damage, but in a high proportion of cases it remains unknown. In contrast to adults, in children with ALF hepati...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2004.00292.x
更新日期:2004-12-01 00:00:00
abstract::To evaluate the role of BAL, CTB, and OLB in the management of pulmonary infiltrates in pediatric HSCT recipients, we conducted a retrospective review of clinical records of pediatric HSCT recipients. Data were analyzed using Chi-square for dichotomous and anova for continuous variables. Logistic regression was used t...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2007.00733.x
更新日期:2007-11-01 00:00:00
abstract::Approximately 800 pediatric renal transplants are performed annually in the United States. VUR or obstruction may cause graft failure requiring redo ureteroneocystostomy. We examined possible risk factors and cost using the PHIS national database. We examined the PHIS for 8.5 yr to determine the association between re...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12522
更新日期:2015-08-01 00:00:00
abstract::Surgical techniques that have been used during liver transplantation (LT) together with patient's coagulation profile and institutional practices are reported to have an effect on transfusion requirements. The aim of this study is to evaluate the transfusion requirement in both cadaveric (CDLT, n = 22) and living dono...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2005.00284.x
更新日期:2005-06-01 00:00:00
abstract::This review details the indications for heart transplantation in children. Contraindications have evolved from absolute to relative. Controversial issues remain and this paper represents a consensus of more than a dozen centers that have programs that remain active performing pediatric heart transplants. ...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1034/j.1399-3046.1999.00045.x
更新日期:1999-11-01 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::Organ transplant is life-saving and any given organ may be valuable to a multitude of potential recipients. An allocation system must be used to reconcile the difference between supply and demand, and this system must take into account the impact that accepting international patients may have on the local system. The ...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1111/petr.12769
更新日期:2016-09-01 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: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:BACKGROUND:Pediatric heart-lung transplantation (HLT) is rare, and no report has analyzed patient outcomes since time of listing. We analyzed pediatric HLTs to understand risk factors for waitlist and post-HLT mortality. METHODS:All pediatric (<18 year old) HLT candidates were identified within the UNOS database (n = ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13682
更新日期:2020-05-01 00:00:00
abstract::To evaluate the effects of gender on HRQOL and overall health status in our pediatric kidney transplants. We performed a cross-sectional study in 77 children who received living renal allo-transplants in our center between 1981 and 2003. The patients were given a questionnaire at a post-transplant visit. After complet...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2009.01174.x
更新日期:2010-03-01 00:00:00
abstract::Adjusted survival outcomes following hepatic resection and transplantation for pediatric liver tumors have not been compared. To address this question, we conducted a retrospective cohort study using the SEER registry. While SEER lacks certain specifics regarding staging, chemotherapy, comorbidities, and recurrence, i...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12144
更新日期:2013-12-01 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::Total body irradiation and high-dose chemotherapy, applied as a preparatory regimen for bone marrow transplantation (BMT) in children with acute lymphoblastic leukemia (ALL), are particularly hazardous to the gonads and, in addition, can impair hypothalamo pituitary-gonadal control. Longitudinal data on pubertal devel...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.1999.00006.x
更新日期:1999-02-01 00:00:00
abstract::Treatment of severe aplastic anemia (SAA) patients who lack human leukocyte antigen (HLA)-matched donors and failed immunosuppressive therapy (IST) is challenging. Recently, umbilical cord blood transplantation (CBT) after non-myeloablative therapy has been reported in adult but not in childhood SAA. However, most cas...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2006.00486.x
更新日期:2006-06-01 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::Severe portopulmonary hypertension (POPH) is a contraindication for liver transplantation (LT) because of the high risk of postoperative heart failure. The early detection of POPH is important for patients with biliary atresia (BA). Brain natriuretic peptide (BNP) is known to be correlated with liver fibrosis in patie...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.13203
更新日期:2018-08-01 00:00:00
abstract::EPVO is a common cause of prehepatic portal hypertension in pediatric patients and sometimes results in cavernous transformation of the PV. We herein present the cases of two patients who underwent LDLT for EPVO with post-Kasai biliary atresia. PV reconstruction was performed with a porto-left gastric vein anastomosis...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12067
更新日期:2013-05-01 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::Potential age-dependent changes of sirolimus metabolite patterns in pediatric renal transplant recipients remain elusive. Thirteen pediatric solid organ transplant recipients (10 kidney, one combined liver-kidney, two liver, mean age 8.0 +/- 5.0 yr) underwent a sirolimus pharmacokinetic profile in steady-state with 10...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.00956.x
更新日期:2009-02-01 00:00:00
abstract::Rejection remains a major threat in pediatric renal transplantation (Tx), causing graft failure and increased exposure to drugs. The new chimeric antibody, basiliximab, directed against the alpha-chain of the interleukin-2 receptor (IL-2R), has been shown to be effective in preventing rejection episodes in adult renal...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1034/j.1399-3046.2001.005004297.x
更新日期:2001-08-01 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::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::IHHE as the most common vascular tumor of the liver in infancy can present with acute postnatal liver and congestive heart failure. LTx may be a lifesaving option, but can be complicated by extrahepatic involvement and bleeding complications, especially in neonates. Here we discuss the benefit of LTx in cases of acute...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/j.1399-3046.2008.01039.x
更新日期:2009-08-01 00:00:00
abstract::Vitamin C deficiency in developed countries is typically observed in patients with unique clinical conditions such as cystic fibrosis or anorexia nervosa, or in patients on long-term tube feeds. We report here a clinical observation in six pediatric and adolescent patients (median age 17.5 yr, range 9.8-23.5 yr) with ...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12285
更新日期:2014-08-01 00:00:00
abstract::LI is a subset of the heterotaxy syndrome and a rare birth defect that involves the heart and other organs. It can be combined with extracardiac abnormalities, especially BA. CHD can be associated with LI in up to 15% of cases, although it is rare in BA. Pediatric LT for a child with ESLD due to BA combined with LI an...
journal_title:Pediatric transplantation
pub_type: 杂志文章
doi:10.1111/petr.12364
更新日期:2014-12-01 00:00:00
abstract::Investigations of HCT for refractory JIA have employed high dose immunosuppressive drugs, such as CY and ATG with or without low dose TBI. Initial disease response has been observed in approximately two-thirds of patients; however, relapse occurs in about half of the responders. The current report describes a regimen ...
journal_title:Pediatric transplantation
pub_type: 杂志文章,多中心研究
doi:10.1111/j.1399-3046.2009.01129.x
更新日期:2010-09-01 00:00:00
abstract::Adolescence is a difficult time for transplant recipients, who need to cope with the challenges of becoming independent from their parents, as well as taking responsibility for their own behavior and medication. They need to balance their developing sexuality with a body image which may be affected by the side-effects...
journal_title:Pediatric transplantation
pub_type: 杂志文章,评审
doi:10.1034/j.1399-3046.2002.01090.x
更新日期:2002-12-01 00:00:00