paediatric transplantation

儿科移植
  • 文章类型: Journal Article
    超急性GVHD(HaGVHD)是造血干细胞移植(HSCT)在植入前发生的一种罕见并发症,通常涉及皮肤和/或肠道和/或肝脏的综合征,发病率和死亡率增加。骨髓清除条件(MAC)制度和不匹配的供体移植增加了HaGVHD的风险。在发展为HaGVHD的人中,类固醇难治性和慢性GVHD的可能性更高。关于HaGVHD的文献有限,尤其是在儿科年龄组。该回顾性单中心病例系列包括五名在2013年4月1日至2015年7月31日期间在印度南部三级护理中心接受HSCT的儿科患者。符合Kim等人的HaGVHD标准。和谁的后续数据可用。我们注意到他们的风险因素,临床病程和预后。有五名小儿HaGVHD患者。其中注意到的危险因素是三个MAC方案和三个不匹配的无关供体来源。两个人患有类固醇难治性疾病,4人继续发展为慢性GVHD,3人死于GVHD或治疗相关并发症.高度怀疑指数是必要的,以识别HaGVHD,尤其是在具有已知危险因素的HSCT后出现发热伴皮疹的患者中。
    Hyperacute GVHD (HaGVHD) is a rare complication of hematopoietic stem cell transplantation (HSCT) occurring before engraftment, a syndrome commonly involving skin and/or gut and/or liver, with increased morbidity and mortality. Myeloablative conditioning (MAC) regimes and mismatched donor transplants have an increased risk for HaGVHD. There is a higher chance of steroid-refractoriness and chronic GVHD in those who develop HaGVHD. There is limited literature about HaGVHD, especially in the paediatric age group. This retrospective single-centre case series included five paediatric patients who underwent HSCT between 1st April 2013 and 31st July 2015 at a tertiary care centre in South India, who fulfilled the criteria for HaGVHD as per criteria by Kim et al. and whose follow up data was available. We noted their risk factors, clinical course and prognosis. There were five paediatric HaGVHD patients. The risk factors noted among them were MAC regimen in three and mismatched unrelated donor sources in three. Two had steroid-refractory disease, four went on to develop chronic GVHD and three died of GVHD or treatment-related complications. A high index of suspicion is necessary to recognize HaGVHD, especially in patients with known risk factors developing a fever with rash post-HSCT.
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  • 文章类型: Journal Article
    ABO不相容性影响高加索患者群体中约40%的同种异体干细胞移植。因为骨髓(BM),来自儿科兄弟姐妹捐赠者和非恶性疾病的首选移植物,红细胞(RBC)含量与血液相似,抗供体异凝集素必须从受体中耗尽或从移植物中去除红细胞。为了获得未操作的BM移植物的耐受性,我们在移植前使用供体ABO型红细胞单位的受控输注来消耗等凝集素.这项回顾性研究评估了2007年至2019年间在我们中心进行的52例ABO主要不相容BM移植的结果。供体型红细胞输血的使用耐受性良好。它们有效地降低了等凝集素水平,通常在一次(60%)或两次(29%)输血后达到目标滴度。该方法允许成功且平稳地注入未操作的BM,从而及时植入。移植结果并不低于ABO相同供体的配对对照组。
    ABO incompatibility affects approximately 40% of allogeneic stem cell transplants in Caucasian patient populations. Because bone marrow (BM), the preferred graft from paediatric sibling donors and for non-malignant diseases, has a red blood cell (RBC) content similar to blood, anti-donor isoagglutinins must either be depleted from the recipient or RBCs removed from the graft. To achieve tolerability of unmanipulated BM grafts, we used controlled infusions of donor ABO-type RBC units to deplete isoagglutinins before the transplant. This retrospective study evaluates the outcomes of 52 ABO major incompatible BM transplants performed at our centre between 2007 and 2019. The use of donor-type RBC transfusions was well tolerated. They effectively reduced isoagglutinins levels, typically achieving target titres after one (60%) or two (29%) transfusions. The approach allowed for successful and uneventful infusions of unmanipulated BM which provided timely engraftment. The transplant outcomes were not inferior to those of a matched-pair control group of patients with ABO-identical donors.
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  • 文章类型: Journal Article
    本研究的目的是确定影响受者致敏率和小儿肾移植患者预后的因素。
    为此,我们对143例小儿肾移植手术进行了回顾性分析.这包括对患者和捐赠者的人口统计数据的评估,HLA不匹配,免疫抑制治疗,拒绝事件,组反应性抗体(PRA)和移植后淋巴增生性疾病(PTLD)。
    接受移植的患者平均年龄为11.5岁,平均随访时间为9.33±5.05年。有人指出,超过59年的捐赠者的移植物存活率结果最差。HLA匹配对移植结果没有统计学意义。超过一个活检证实的排斥反应的移植物存活也显著缩短(p=0.008)。在28%的受者移植后发现PRA,并显示与较低的移植物存活率相关(p<0.001)。在本研究中,22.7%(5/22)的EBV感染患者出现PTLD。
    总而言之,良好的移植物存活,对未来移植的敏感性降低,并将PTLD的风险降至最低,可以通过捐赠者年龄之间的平衡来确保,应寻求HLA匹配和接受者的状况。此外,儿科患者最好接受10至59岁的捐献者的器官.EBV感染可能是发生PTLD的相关因素。
    UNASSIGNED: The aim of this study was to identify factors impacting recipient sensitization rates and paediatric renal transplant patient outcomes.
    UNASSIGNED: For this purpose, a retrospective analysis of 143 paediatric renal transplants was carried out. This included the evaluation of patient\'s and donor\'s demographic data, HLA mismatches, immunosuppressive therapy, rejection episodes, panel reactive antibody (PRA) and post-transplant lymphoproliferative disease (PTLD).
    UNASSIGNED: The mean patient age at the point of transplant receival was 11.5 years with a mean follow up time of 9.33±5.05 years. It was noted that graft survival rates for donors over 59 years had the worst outcome. HLA match did not show statistically significant influence on graft outcome. Graft survival for more than one biopsy-proven rejection was also significantly shorter (p=0.008). PRA were found in 28% of the recipient\'s post-transplantation and showed association with lower graft survival rates (p<0.001). In the present study, 22.7% (5/22) of the patients with EBV infections presented a PTLD.
    UNASSIGNED: In conclusion, good graft survival with reduced sensitization for future transplantations and minimize the risk of PTLD, can be ensured through a balance between donor age, HLA match and condition of the recipient should be sought. Furthermore, paediatric patients should preferably receive organs from donors between the age of 10 and 59. EBV infection could be a relevant factor for developing PTLD.
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  • 文章类型: Journal Article
    我们试图在来自发展中国家的儿科OHT患者队列中更好地定义移植后淋巴增生性疾病(PTLD)的人口统计学和特征。数据是从心脏研究所收集的,圣保罗,适用于1992年10月至2018年10月期间的所有儿科OHT接受者.PTLD和非PTLD队列之间的组差异通过Fisher精确和Mann-WhitneyU检验进行评估。Kaplan-Meier曲线分析了各组的存活率。对202名儿科OHT接受者的数据进行了审查。Overall1-,整个队列的5年和10年生存率为76.5%,68.3%和62.9%;24例患者(11.9%)在OHT后中位3.1年(IQR0.8-9.0)出现PTLD。病例在随访期间分布均匀,在3年存活的患者中,有9.8%(n=137)被诊断为PTLD,15.3%(n=78)的患者在5年时存活,29.3%(n=41)的患者在10年时存活。PTLD的最常见形式是弥漫性大B细胞淋巴瘤(n=9),大多数患者接受利妥昔单抗联合免疫抑制和化疗治疗(n=15).我们发现PTLD与PTLD之间的死亡率没有增加。多变量分析中的非PTLD队列(P=0.365)。小儿OHT后的PTLD结果可接受。然而,PTLD的危险因素尚未确定,需要进一步调查.
    We sought to better define the demographics and characteristics of post-transplant lymphoproliferative disorders (PTLD) in a cohort of paediatric OHT patients from a developing country. Data were collected from the Heart Institute, Sao Paulo, for all paediatric OHT recipients from October 1992 to October 2018. Group differences between the PTLD and non-PTLD cohorts were assessed by Fisher exact and Mann-Whitney U tests. Kaplan-Meier curves analysed the survival in each group. Data were reviewed for 202 paediatric OHT recipients. Overall 1-, 5- and 10-year survival for the entire cohort was 76.5%, 68.3% and 62.9%; 24 patients (11.9%) developed PTLD at a median 3.1 years (IQR 0.8-9.0) after OHT. Cases were evenly spread over the follow-up period, with PTLD diagnosed in 9.8% (n = 137) of patients who were alive at 3 years, 15.3% (n = 78) of patients who were alive at 5 years and 29.3% (n = 41) of patients who were alive at 10 years. The commonest form of PTLD was diffuse large B cell lymphoma (n = 9), and most patients received rituximab with immunosuppression and chemotherapy as treatment (n = 15). We identified no increased risk in mortality amongst the PTLD vs. non-PTLD cohorts in multivariate analysis (P = 0.365). PTLD after paediatric OHT had acceptable outcomes. However, risk factors for PTLD were not identified and warrant further investigation.
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  • 文章类型: Journal Article
    This review reports the outcomes of paediatric renal transplantation in the United Kingdom over the last 25 years. UK Transplant Registry data on 3236 paediatric renal transplants performed between 1 January 1992 and 31 December 2016 were analysed. Significant improvements in human leucocyte antigen (HLA) matching have been achieved; 84% of recipients received 000 or favourable (0 DR and 0 or 1 B) mismatched kidneys in 2016 compared with 27% in 1992. The median waiting time has increased from 126 days in 1999 to 351 days in 2016. Tacrolimus replaced ciclosporin in most immunosuppressive regimens after 2002. Renal transplant outcome has improved significantly, mainly because of a reduction in early graft loss. One-year donation after brain death renal allograft survival for those transplanted from 2012 to 2016 was 98%, compared with 72% for those transplanted from 1987 to 1991. Renal allograft survival for first kidney only transplants at 1, 5, 10, 20 and 25 years were 89%, 79%, 65%, 42% and 33% respectively. Superior survival with living donor was maintained throughout the study period with 25-year graft survival at 33% compared with 31% from deceased donor (P < 0.0001). Changes in immunosuppression regimens, improvements in HLA matching and a reduction of cold ischaemia time may in part explain the improvements in graft survival.
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  • 文章类型: Journal Article
    背景:为了评估小儿肾移植后的移植物和患者存活率,并检测影响因素,这会影响移植后的时间。
    方法:我们分析了小儿肾移植后的长期生存率和并发症,并搜索了移植物功能的预测参数。
    结果:在132名患者中,进行了143例肾移植。移植失败发生在25%。慢性排斥是移植物丢失的主要原因(42.9%)。1年后移植物存活率为92.2%,5年后85.5%,10年后为71.1%,15年后为62.1%。以下参数强烈影响移植物存活:移植数量(p=0.014),移植年份(1997-2005年p<0.0001),移植后的Epo治疗(p=0.001),低血压供体(p=0.027),冷缺血时间(p=0.023),吻合时间>50分钟(p=0.008),移植功能延迟(p=0.003)和死亡捐赠(p=0.039)。死亡的患者比例为5.6%。1年后患者总生存率为99.3%,5年后的95.2%,10年后94.2%,15年后90.7%。各种类型的感染(42.9%)是主要的死亡原因。
    结论:小儿肾移植术后死亡的主要原因是恶性肿瘤和感染。为了避免血管并发症,尤其是在年轻受者(<9岁)中,冷缺血时间应尽可能短。
    BACKGROUND: To evaluate graft- and patient survival after paediatric kidney transplantation and detecting influence factors, which affect the post-transplant time.
    METHODS: We analysed long-term survival rates and complications after paediatric kidney transplantation and searched for predictive parameters for graft function.
    RESULTS: In 132 patients, 143 kidney transplantations were performed. Graft failure occurred in 25%. Chronic rejections were the leading cause of graft loss (42.9%). Graft survival rates were 92.2% after 1 year, 85.5% after 5 years, 71.1% after 10 years and 62.1% after 15 years. The following parameters strongly influenced graft survival: number of transplants (p = 0.014), year of transplant (p < 0.0001 for 1997-2005), Epo-therapy post-transplant (p = 0.001), hypotension donor (p = 0.027), cold ischemia time (p = 0.023), anastomosis time >50 min (p = 0.008), delayed graft function (p = 0.003) and deceased donation (p = 0.039). The percentage of patients who died was 5.6%. Overall patient survival rates were 99.3% after 1 year, 95.2% after 5 years, 94.2% after 10 years and 90.7% after 15 years. Various types of infections (42.9%) were the main causes of death.
    CONCLUSIONS: The main causes of death after kidney transplantations in paediatric recipients are malignancy and infections. To avoid vascular complications especially in young recipients (<9 years), the cold ischemia time should be as short as possible.
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  • 文章类型: Journal Article
    肝移植(SLT)已在欧洲广泛采用,导致儿科候补死亡率显着降低。左分裂移植物(LSG)通常用于儿科接受者;但是,已故捐赠者的选择标准并不普遍。这项研究的目的是分析来自欧洲肝移植注册的LSG结果,并确定移植物失败的风险因素。回顾性分析了2006年至2014年从已故供体移植LSG的1500名儿童的数据。总的来说,移植5年后移植物损失为343(22.9%),240(70.0%)发生在前3个月内。从SLT开始,3个月时估计患者生存率为89.1%,5年时估计为82.9%。再移植率为11.5%。在多变量分析中,3个月时移植物衰竭的重要危险因素包括:紧急SLT(HR=1.73,P=0.0012),受体体重≤6kg(HR=1.91,P=0.0029),供体年龄>50岁(HR=1.87,P=0.0039),和冷缺血时间(CIT)[HR=每小时1.07,P=0.0227]。LSG具有良好的结果,SLT是当前器官短缺时代儿科接受者的绝佳选择。我们确定了LSG捐赠者和接受者选择标准的实用指南:在没有其他风险因素的情况下,捐赠者年龄可以安全地延长到50岁;因此,<6kg的儿童和紧急移植需要CIT<6h和适当的移植物/受体大小匹配以实现良好的结果。
    Split liver transplantation (SLT) has been widely adopted across Europe, resulting in remarkable reduction in the paediatric waiting-list mortality. Left split graft (LSG) is commonly used for paediatric recipients; however, deceased donor criteria selection are not universal. The aim of this study was to analyse the LSG outcome from the European Liver Transplant Registry and to identify risk factors for graft failure. Data from 1500 children transplanted in 2006-2014 with LSG from deceased donors were retrospectively analysed. Overall, graft losses were 343(22.9%) after 5 years from transplantation, 240(70.0%) occurred within the first 3 months. Estimated patient survival was 89.1% at 3 months and 82.9% at 5 years from SLT. Re-transplantation rate was 11.5%. At multivariable analysis, significant risk factors for graft failure at 3 months included the following: urgent SLT (HR = 1.73, P = 0.0012), recipient body weight ≤6 kg (HR = 1.91, P = 0.0029), donor age >50 years (HR = 1.87, P = 0.0039), and cold ischaemic time (CIT) [HR = 1.07 per hour, P = 0.0227]. LSG has good outcomes and SLT is excellent option for paediatric recipients in the current organ shortage era. We identified practical guidelines for LSG donor and recipient selection criteria: donor age may be safely extended up to 50 years in the absence of additional risk factors; thus, children <6 kg and urgent transplantation need CIT <6 h and appropriate graft/recipient size-matching to achieve good outcomes.
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  • 文章类型: Journal Article
    随着时间的推移,儿科受者肝移植(LT)后的生存率显着提高。然而,仍然缺乏有关从儿科过渡到成人医疗保健服务(AHS)后结局的数据.因此,我们的目标是前瞻性地评估LT接受者过渡后的结果,获取患者不依从性并确定不依从性的潜在危险因素。
    在2010年至2015年期间,对所有连续进入帕多瓦大学医院AHS的青少年LT接受者进行了评估。人口统计数据,肝功能检查,急性或慢性排斥反应发作的发生率和对药物处方的依从性,进行了前瞻性评估。自2015年以来实施了一项教育试点研究,以促进过渡期间的依从性。
    总之,评估了32例患者(M/F16/16,中位年龄:23岁)。LT和过渡之间的中位间隔时间为15年(范围:1-26年)。LT的主要指征是胆道闭锁(31%),而免疫抑制方案以他克莫司为基础的占75%。经过29个月的中位随访(范围:12-83),未观察到肝功能试验的显著改变.活检证实的慢性排斥反应在6/32(18%)的患者中被诊断出,他克莫司谷水平的标准差高于无他克莫司谷水平的患者(1.5vs1,P=0.03)。8/32(25%)的患者报告了非依从性,并且与饮酒显着相关(P=.003)。患者和移植物存活率分别为96%和93%,分别。
    经历过渡到AHS的青少年LT患者具有良好的长期预后。然而,应使用旨在促进依从性的多学科方法。
    Survival rates after liver transplantation (LT) in paediatric recipients have significantly improved over time. However, data regarding outcomes after transition from Paediatric to Adult Healthcare Service (AHS) are still lacking. Therefore, we aimed to prospectively evaluate the outcome of LT recipients after transition, to access patients\' non-adherence and identify potential risk factors for non-adherence.
    All consecutive adolescent LT recipients moving to the AHS at Padua University Hospital were evaluated between 2010 and 2015. Demographic data, liver function tests, incidence of acute or chronic rejection episodes and adherence to medical prescription, were prospectively evaluated. An educational pilot study was implemented since 2015 to foster adherence during transition.
    In all, 32 patients (M/F 16/16, median age: 23 years) were evaluated. Median interval time between LT and transition was 15 years (range: 1-26 years). The main indication for LT was biliary atresia (31%), whereas immunosuppression regimen was tacrolimus-based in 75%. After a median follow-up of 29 months (range: 12-83), no significant modifications of liver function tests were observed. Biopsy-proven chronic rejection was diagnosed in 6/32 (18%) of patients, who had higher standard deviation of tacrolimus trough level than patients without (1.5 vs 1, P = .03). Non-adherence was reported in 8/32 (25%) of patients and was significantly associated with alcohol consumption (P = .003). Patient and graft survival were 96% and 93%, respectively.
    Adolescent LT patients who undergo transition to the AHS have good long-term outcomes. However, a multidisciplinary approach aiming at fostering adherence should be used.
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  • 文章类型: Journal Article
    BACKGROUND: There is enough evidence concerning the short-term safety of living donors after kidney transplantation. However, long-term complications continue to be studied, with a particular interest in young donors. Previous studies have been conducted in older donors for adult renal patients. We present a study of long-term complications in kidney donors for our paediatric population.
    METHODS: We carried out a long-term donor study for the 54 living kidney-donor transplantations performed at our department from 1979 to June 2014. We monitored the glomerular filtration rate (GFR) on the basis of 24-hour urine creatinine clearance, 24-hour proteinuria and the development of arterial hypertension in the 48 donors who were followed up for more than one year. Only the 39 patients who were exclusively followed up by our department have been included in the results analysis.
    RESULTS: GFR through creatinine clearance was stable after an initial decrease. No proteinuria was observed in any of the cases. One patient developed chronic kidney disease (CKD), which resulted in a cumulative incidence of 2%. GFR below 60mL/min/1.73 m2 was not reported in any other patients. Arterial hypertension was diagnosed in 25% of donors, 90% of which were treated with antihypertensives.
    CONCLUSIONS: Risk of CKD and hypertension in living kidney donors for paediatric recipients, who are carefully monitored throughout their evolution, is similar to that of the general population. Therefore, this technique appears to be safe in both the short and long term.
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  • 文章类型: Journal Article
    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 children. Transplantation in Africa faces a number of challenges including wide socioeconomic disparity, lack of legislation around brain death and organ donation in many countries, shortage of skilled medical personnel and facilities, infectious disease burden and insecure access to and monitoring of immunosuppression. Whilst there is a need for transplantation, the establishment and sustainability of transplant programmes require careful planning with national government and institutional support. Legislation regarding brain death diagnosis and organ retrieval/donation; appropriate training of the transplant team; and transparent and equitable criteria for organ allocation are important to establish before embarking on a transplant programme. Establishing sustainable, self-sufficient transplant programmes in Africa with equal access to all citizens is an important step towards curtailing transplant tourism and organ trafficking and has a further beneficial effect in raising the level of medical and surgical care in these countries.
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