Anjali Rai, Lauren Sproule, Tatianna Larman, Kiyoko Oshima, Daniel Rhee, Kenneth Ng, Elizabeth King, Douglas Mogul, Kathryn Lemberg
Background: Neuroendocrine tumors (NETs) are rare epithelial neoplasms that arise most commonly from the gastrointestinal tract. In pediatrics, the most common site of origin is in the appendix, with the liver being the most common site of metastasis. Neuroendocrine tumors arising from the biliary tract are extremely rare.
Methods: We describe a case of a nine-year-old girl who presented with obstructive cholestasis and was found to have multiple liver masses identified on biopsy as well-differentiated neuroendocrine tumor with an unknown primary tumor site.
Result: The patient underwent extensive investigation to identify a primary tumor site, including endoscopy, endoscopic ultrasound, and capsule endoscopy. The patient ultimately underwent definitive management with liver transplant, and on explant was discovered to have multiple well-differentiated neuroendocrine tumors, WHO Grade 1, with extensive infiltration into the submucosa of bile duct, consistent with primary biliary tract neuroendocrine tumor.
Conclusion: Identifying the site of the primary tumor in NETs found within the liver can be challenging. To determine if an extrahepatic primary tumor exists, workup should include endoscopy, EUS, and capsule endoscopy. Children with well-differentiated hepatic NETs, with no identifiable primary tumor, and an unresectable tumor, are considered favorable candidates for liver transplantation.
{"title":"Liver transplant for primary biliary tract neuroendocrine tumor in a nine-year-old girl.","authors":"Anjali Rai, Lauren Sproule, Tatianna Larman, Kiyoko Oshima, Daniel Rhee, Kenneth Ng, Elizabeth King, Douglas Mogul, Kathryn Lemberg","doi":"10.1111/petr.14732","DOIUrl":"10.1111/petr.14732","url":null,"abstract":"<p><strong>Background: </strong>Neuroendocrine tumors (NETs) are rare epithelial neoplasms that arise most commonly from the gastrointestinal tract. In pediatrics, the most common site of origin is in the appendix, with the liver being the most common site of metastasis. Neuroendocrine tumors arising from the biliary tract are extremely rare.</p><p><strong>Methods: </strong>We describe a case of a nine-year-old girl who presented with obstructive cholestasis and was found to have multiple liver masses identified on biopsy as well-differentiated neuroendocrine tumor with an unknown primary tumor site.</p><p><strong>Result: </strong>The patient underwent extensive investigation to identify a primary tumor site, including endoscopy, endoscopic ultrasound, and capsule endoscopy. The patient ultimately underwent definitive management with liver transplant, and on explant was discovered to have multiple well-differentiated neuroendocrine tumors, WHO Grade 1, with extensive infiltration into the submucosa of bile duct, consistent with primary biliary tract neuroendocrine tumor.</p><p><strong>Conclusion: </strong>Identifying the site of the primary tumor in NETs found within the liver can be challenging. To determine if an extrahepatic primary tumor exists, workup should include endoscopy, EUS, and capsule endoscopy. Children with well-differentiated hepatic NETs, with no identifiable primary tumor, and an unresectable tumor, are considered favorable candidates for liver transplantation.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022400","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Allison Armstrong, Jane W Liang, Danton Char, Seth A Hollander, Kimberly A Pyke-Grimm
Background: Disparities in pediatric heart transplant outcomes based on socioeconomic status (SES) have been previously observed. However, there is a need to reevaluate these associations in contemporary settings with advancements in transplant therapies and increased awareness of health disparities. This retrospective study aims to investigate the relationship between SES and outcomes for pediatric heart transplant patients.
Methods: Data were collected through a chart review of 176 pediatric patients who underwent first orthotopic heart transplantation (OHT) at a single center from 2013 to 2021. The Area Deprivation Index (ADI), a composite score based on U.S. census data, was used to quantify SES. Cox proportional hazards models and generalized linear models were employed to analyze the association between SES and graft failure, rejection rates, and hospitalization rates.
Results: The analysis revealed no statistically significant differences in graft failure rates, rejection rates, or hospitalization rates between low-SES and high-SES pediatric heart transplant patients for our single-center study.
Conclusion: There may be patient education, policies, and social resources that can help mitigate SES-based healthcare disparities. Additional multi-center research is needed to identify post-transplant care that promotes patient equity.
背景:以前曾观察到小儿心脏移植结果因社会经济地位(SES)而存在差异。然而,随着移植疗法的进步和人们对健康差异认识的提高,有必要在现代环境中重新评估这些关联。这项回顾性研究旨在调查 SES 与小儿心脏移植患者预后之间的关系:方法:研究人员通过病历回顾收集了 2013 年至 2021 年期间在一个中心接受首次正位心脏移植手术(OHT)的 176 名儿科患者的数据。地区贫困指数(ADI)是基于美国人口普查数据的综合评分,用于量化社会经济地位。采用 Cox 比例危险模型和广义线性模型分析 SES 与移植物失败、排斥率和住院率之间的关系:分析结果显示,在我们的单中心研究中,低社会经济地位和高社会经济地位的小儿心脏移植患者在移植失败率、排斥率和住院率方面没有明显的统计学差异:结论:患者教育、政策和社会资源可能有助于减轻基于社会经济地位的医疗差异。需要开展更多的多中心研究,以确定能促进患者公平的移植后护理。
{"title":"The effect of socioeconomic status on pediatric heart transplant outcomes at a single institution between 2013 and 2022.","authors":"Allison Armstrong, Jane W Liang, Danton Char, Seth A Hollander, Kimberly A Pyke-Grimm","doi":"10.1111/petr.14695","DOIUrl":"10.1111/petr.14695","url":null,"abstract":"<p><strong>Background: </strong>Disparities in pediatric heart transplant outcomes based on socioeconomic status (SES) have been previously observed. However, there is a need to reevaluate these associations in contemporary settings with advancements in transplant therapies and increased awareness of health disparities. This retrospective study aims to investigate the relationship between SES and outcomes for pediatric heart transplant patients.</p><p><strong>Methods: </strong>Data were collected through a chart review of 176 pediatric patients who underwent first orthotopic heart transplantation (OHT) at a single center from 2013 to 2021. The Area Deprivation Index (ADI), a composite score based on U.S. census data, was used to quantify SES. Cox proportional hazards models and generalized linear models were employed to analyze the association between SES and graft failure, rejection rates, and hospitalization rates.</p><p><strong>Results: </strong>The analysis revealed no statistically significant differences in graft failure rates, rejection rates, or hospitalization rates between low-SES and high-SES pediatric heart transplant patients for our single-center study.</p><p><strong>Conclusion: </strong>There may be patient education, policies, and social resources that can help mitigate SES-based healthcare disparities. Additional multi-center research is needed to identify post-transplant care that promotes patient equity.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022408","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lung transplantation is considered as the ultimate therapy for children with advanced pulmonary disease. International data show a median conditional 1-year post-transplantation survival of 9.1 years. Recently, antibody-mediated rejection (AMR) has increasingly been recognized as an important cause of allograft dysfunction although pediatric reports are still scarce. Donor-specific anti-human leukocyte antigen (HLA) antibodies (DSA) are known to play a role in AMR development post-transplant but AMR pathogenesis is still poorly understood. Central to the concept of pulmonary AMR is immune activation with the production of allo-specific B-cells and plasma cells directed against donor lung antigens. The frequency of pulmonary AMR in children is currently unknown. Due to the lack of AMR data in children, the diagnostic approach for pediatric pulmonary AMR is solely based on adult literature. This personal viewpoint article evaluates the rational for the creation of age-based thresholds for different diagnostic categories of pulmonary AMR and data on the management of pulmonary AMR in children. To the authors' knowledge, there have been no randomized controlled trials comparing different management regimes in pulmonary AMR, and thus, management and treatment algorithms for pulmonary AMR in children are only extrapolated from adults. To advance the knowledge of AMR in children, the authors propose that children be included in collaborative, multi-center trials. It is vital that future decisions on internationally agreed upon guidelines for pulmonary AMR take its impact on children into consideration. Research is needed to fill the current knowledge gaps in the field of pulmonary AMR in children focused on optimizing outcomes.
肺移植被认为是晚期肺病患儿的终极疗法。国际数据显示,有条件的移植后 1 年生存期中位数为 9.1 年。最近,抗体介导的排斥反应(AMR)越来越被认为是导致异体移植功能障碍的一个重要原因,但有关儿科的报道仍然很少。已知供体特异性抗人类白细胞抗原(HLA)抗体(DSA)在移植后AMR的发生中起作用,但对AMR的发病机制仍知之甚少。肺AMR概念的核心是免疫激活,产生针对供体肺抗原的异体特异性B细胞和浆细胞。目前,儿童肺AMR的发病率尚不清楚。由于缺乏儿童 AMR 的数据,小儿肺 AMR 的诊断方法完全基于成人文献。这篇个人观点文章评估了为不同诊断类别的肺部 AMR 设定基于年龄的阈值的合理性,以及儿童肺部 AMR 的管理数据。据作者所知,目前还没有随机对照试验比较不同的肺AMR治疗方案,因此,儿童肺AMR的管理和治疗算法只能从成人中推断。为了增进对儿童 AMR 的了解,作者建议将儿童纳入多中心合作试验中。至关重要的是,未来国际公认的肺部 AMR 指南决策应考虑到其对儿童的影响。需要开展研究,填补目前在儿童肺部 AMR 领域的知识空白,重点是优化治疗效果。
{"title":"Antibody-mediated rejection (AMR) in pediatric lung transplantation-Current state and future directions.","authors":"Christian Benden, Kathryn A Wikenheiser-Brokamp","doi":"10.1111/petr.14739","DOIUrl":"10.1111/petr.14739","url":null,"abstract":"<p><p>Lung transplantation is considered as the ultimate therapy for children with advanced pulmonary disease. International data show a median conditional 1-year post-transplantation survival of 9.1 years. Recently, antibody-mediated rejection (AMR) has increasingly been recognized as an important cause of allograft dysfunction although pediatric reports are still scarce. Donor-specific anti-human leukocyte antigen (HLA) antibodies (DSA) are known to play a role in AMR development post-transplant but AMR pathogenesis is still poorly understood. Central to the concept of pulmonary AMR is immune activation with the production of allo-specific B-cells and plasma cells directed against donor lung antigens. The frequency of pulmonary AMR in children is currently unknown. Due to the lack of AMR data in children, the diagnostic approach for pediatric pulmonary AMR is solely based on adult literature. This personal viewpoint article evaluates the rational for the creation of age-based thresholds for different diagnostic categories of pulmonary AMR and data on the management of pulmonary AMR in children. To the authors' knowledge, there have been no randomized controlled trials comparing different management regimes in pulmonary AMR, and thus, management and treatment algorithms for pulmonary AMR in children are only extrapolated from adults. To advance the knowledge of AMR in children, the authors propose that children be included in collaborative, multi-center trials. It is vital that future decisions on internationally agreed upon guidelines for pulmonary AMR take its impact on children into consideration. Research is needed to fill the current knowledge gaps in the field of pulmonary AMR in children focused on optimizing outcomes.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022394","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Methylmalonic acidemia (MMA) is an autosomal recessive disorder caused by defects in propionyl-CoA (P-CoA) catabolism; of note, liver neoplasms rarely occur as a long-term complication of the disorder. Herein, we report the case of a patient with MMA and hepatocellular carcinoma (HCC) who was successfully treated with a living-donor liver transplant (LDLT) following prior kidney transplantation.
Case report: A 25-year-old male patient with MMA underwent LDLT with a left lobe graft because of metabolic instability and liver neoplasms. He had presented with chronic symptoms of MMA, which had been diagnosed by genetic testing. Additionally, he had undergone living-donor kidney transplantation with his father as the donor due to end-stage kidney disease 6 years before the LDLT. He had an episode of metabolic decompensation triggered by coronavirus disease in 2019. Imaging studies revealed an intrahepatic neoplasm in the right hepatic lobe. Due to concerns about metabolic decompensation after hepatectomy, LDLT was performed using a left lobe graft obtained from the patient's mother. Pathological findings were consistent with the characteristics of well-to-moderately differentiated HCC. The postoperative course was uneventful, and the patient was discharged 48 days after the LDLT without any complications. At the 9-month follow-up, the patient's condition was satisfactory, with sufficient liver graft function and without metabolic decompensation.
Conclusion: This case indicates that although HCC is a rare complication in patients with MMA, clinicians should be aware of hepatic malignancies during long-term follow-up.
{"title":"Living-donor liver transplantation for methylmalonic acidemia patient with hepatocellular carcinoma: A case report and literature review.","authors":"Harunori Deguchi, Seisuke Sakamoto, Seiichi Shimizu, Akinari Fukuda, Hajime Uchida, Yusuke Yanagi, Toshimasa Nakao, Tasuku Kodama, Ryuji Komine, Kentaro Nishi, Koichi Kamei, Chizuko Haga, Takako Yoshioka, Kimikazu Matsumoto, Reiko Horikawa, Mureo Kasahara","doi":"10.1111/petr.14719","DOIUrl":"10.1111/petr.14719","url":null,"abstract":"<p><strong>Background: </strong>Methylmalonic acidemia (MMA) is an autosomal recessive disorder caused by defects in propionyl-CoA (P-CoA) catabolism; of note, liver neoplasms rarely occur as a long-term complication of the disorder. Herein, we report the case of a patient with MMA and hepatocellular carcinoma (HCC) who was successfully treated with a living-donor liver transplant (LDLT) following prior kidney transplantation.</p><p><strong>Case report: </strong>A 25-year-old male patient with MMA underwent LDLT with a left lobe graft because of metabolic instability and liver neoplasms. He had presented with chronic symptoms of MMA, which had been diagnosed by genetic testing. Additionally, he had undergone living-donor kidney transplantation with his father as the donor due to end-stage kidney disease 6 years before the LDLT. He had an episode of metabolic decompensation triggered by coronavirus disease in 2019. Imaging studies revealed an intrahepatic neoplasm in the right hepatic lobe. Due to concerns about metabolic decompensation after hepatectomy, LDLT was performed using a left lobe graft obtained from the patient's mother. Pathological findings were consistent with the characteristics of well-to-moderately differentiated HCC. The postoperative course was uneventful, and the patient was discharged 48 days after the LDLT without any complications. At the 9-month follow-up, the patient's condition was satisfactory, with sufficient liver graft function and without metabolic decompensation.</p><p><strong>Conclusion: </strong>This case indicates that although HCC is a rare complication in patients with MMA, clinicians should be aware of hepatic malignancies during long-term follow-up.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Aleena Malik, Vicky L Ng, Blayne A Sayed, Asad Siddiqui, Dimitri A Parra
Background: Biliary complications are common in pediatric liver transplant. Strictures resistant to interventional radiology procedures can be extremely challenging to manage and may result in the need of surgery or retransplantation.
Methods: This case report illustrates the use of biodegradable stents post left lateral segment live donor liver transplant in a pediatric patient with a recalcitrant chronic stricture of the biliary-enteric anastomosis. The patient developed a high stricture requiring multiple interventions and eventual access of both the segment II and segment III ducts of the graft.
Results: To ensure adequate biliary drainage, two biodegradable stents were deployed using a "kissing-stent" technique. The stents were successfully deployed and allowed the patient to remain free from an internal-external biliary drain for 11 months, with eventual redeployment of an additional biodegradable stent.
Conclusion: In patients with recalcitrant stenosis of the biliary anastomosis, biodegradable stents may provide durable drainage, optimizing graft function and delaying retransplantation in addition to keeping patients without external devices, thus improving quality of life.
背景:胆道并发症在小儿肝移植中很常见。对介入放射学手术有抵抗力的胆道狭窄极难处理,可能导致需要手术或再次移植:本病例报告说明了在左侧段活体肝移植术后使用生物可降解支架治疗胆肠吻合口顽固性慢性狭窄的儿科患者的情况。该患者的胆肠吻合口出现高度狭窄,需要多次介入治疗,并最终进入移植物的 II 段和 III 段管道:为确保充分的胆道引流,采用 "吻合支架 "技术放置了两个生物可降解支架。结果:为确保胆道引流通畅,采用 "吻合支架 "技术放置了两个可降解支架。支架放置成功后,患者在11个月的时间里一直没有使用胆道内外引流管,最终又重新放置了一个可降解支架:结论:对于胆道吻合口狭窄顽固的患者,生物可降解支架可提供持久的引流,优化移植物的功能,推迟再次移植的时间,此外还能让患者无需外部装置,从而提高生活质量。
{"title":"Biodegradable biliary stents placement using a \"kissing-stent\" technique for management of a recalcitrant stricture post-live donor liver transplant.","authors":"Aleena Malik, Vicky L Ng, Blayne A Sayed, Asad Siddiqui, Dimitri A Parra","doi":"10.1111/petr.14725","DOIUrl":"10.1111/petr.14725","url":null,"abstract":"<p><strong>Background: </strong>Biliary complications are common in pediatric liver transplant. Strictures resistant to interventional radiology procedures can be extremely challenging to manage and may result in the need of surgery or retransplantation.</p><p><strong>Methods: </strong>This case report illustrates the use of biodegradable stents post left lateral segment live donor liver transplant in a pediatric patient with a recalcitrant chronic stricture of the biliary-enteric anastomosis. The patient developed a high stricture requiring multiple interventions and eventual access of both the segment II and segment III ducts of the graft.</p><p><strong>Results: </strong>To ensure adequate biliary drainage, two biodegradable stents were deployed using a \"kissing-stent\" technique. The stents were successfully deployed and allowed the patient to remain free from an internal-external biliary drain for 11 months, with eventual redeployment of an additional biodegradable stent.</p><p><strong>Conclusion: </strong>In patients with recalcitrant stenosis of the biliary anastomosis, biodegradable stents may provide durable drainage, optimizing graft function and delaying retransplantation in addition to keeping patients without external devices, thus improving quality of life.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022396","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Zibya Barday, Bianca Davidson, Paul Harden, Zakira Mukuddem-Sablay, Nicola Wearne, Erika S W Jones, Daley Cross, Mignon McCulloch
Adolescents and Young Adults (AYAs) with chronic kidney disease (CKD) have challenges unique to this developmental period, with increased rates of high-risk behavior and non-adherence to therapy which may impact the progression of kidney disease and their requirement for kidney replacement therapy (KRT). Successful transition of AYA patients are particularly important in low- and middle-income countries (LMICs) where KRT is limited, rationed or not available. Kidney AYA transition clinics have the potential to improve clinical outcomes but there is a paucity of data on the clinical translational impact of these clinics in Africa. This review is a reflection of the 20-year growth and development of the first South African kidney AYA transition clinic. We describe a model of care for patients with CKD, irrespective of etiology, aged 10-25 years, transitioning from pediatric to adult nephrology services. This unique service was established in 2002 and re-designed in 2015. This multidisciplinary integrated transition model has improved patient outcomes, created peer support groups and formed a training platform for future pediatric and adult nephrologists. In addition, an Adolescent Centre of Excellence has been created to compliment the kidney AYA transition model of care. The development of this transition pathway challenges and solutions are explored in this article. This is the first kidney AYA transition clinic in Africa. The scope of this service has expanded over the last two decades. With limited resources in LMICs, such as KRT, the structured transition of AYAs with kidney disease is not only possible but essential. It is imperative to preserve residual kidney function, maximize the kidney allograft lifespan and improve adherence, to enable young individuals an opportunity to lead productive lives.
{"title":"Kidney adolescent and young adult clinic: A transition model in Africa.","authors":"Zibya Barday, Bianca Davidson, Paul Harden, Zakira Mukuddem-Sablay, Nicola Wearne, Erika S W Jones, Daley Cross, Mignon McCulloch","doi":"10.1111/petr.14690","DOIUrl":"10.1111/petr.14690","url":null,"abstract":"<p><p>Adolescents and Young Adults (AYAs) with chronic kidney disease (CKD) have challenges unique to this developmental period, with increased rates of high-risk behavior and non-adherence to therapy which may impact the progression of kidney disease and their requirement for kidney replacement therapy (KRT). Successful transition of AYA patients are particularly important in low- and middle-income countries (LMICs) where KRT is limited, rationed or not available. Kidney AYA transition clinics have the potential to improve clinical outcomes but there is a paucity of data on the clinical translational impact of these clinics in Africa. This review is a reflection of the 20-year growth and development of the first South African kidney AYA transition clinic. We describe a model of care for patients with CKD, irrespective of etiology, aged 10-25 years, transitioning from pediatric to adult nephrology services. This unique service was established in 2002 and re-designed in 2015. This multidisciplinary integrated transition model has improved patient outcomes, created peer support groups and formed a training platform for future pediatric and adult nephrologists. In addition, an Adolescent Centre of Excellence has been created to compliment the kidney AYA transition model of care. The development of this transition pathway challenges and solutions are explored in this article. This is the first kidney AYA transition clinic in Africa. The scope of this service has expanded over the last two decades. With limited resources in LMICs, such as KRT, the structured transition of AYAs with kidney disease is not only possible but essential. It is imperative to preserve residual kidney function, maximize the kidney allograft lifespan and improve adherence, to enable young individuals an opportunity to lead productive lives.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022399","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Matthew Letts, Zainab Arslan, Lucy Plumb, Pippa Bailey, Siân Griffin, Jelena Stojanovic
{"title":"Management of pediatric patients with a failing kidney transplant: A survey of UK-based renal units.","authors":"Matthew Letts, Zainab Arslan, Lucy Plumb, Pippa Bailey, Siân Griffin, Jelena Stojanovic","doi":"10.1111/petr.14730","DOIUrl":"10.1111/petr.14730","url":null,"abstract":"","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022402","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"How to choose a best kidney for pediatric kidney transplant recipients.","authors":"Asha Moudgil, Amy Bobrowski","doi":"10.1111/petr.14717","DOIUrl":"10.1111/petr.14717","url":null,"abstract":"","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139990852","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Should urinary CXCL10/creatinine be measured for kidney transplantation?","authors":"Guido Filler, Ajay P Sharma","doi":"10.1111/petr.14729","DOIUrl":"10.1111/petr.14729","url":null,"abstract":"","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022405","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
A Marion Ybarra, Alicia M Kamsheh, Matthew J O'Connor, Seth A Hollander, Maria Bano, Michelle Ploutz, Gabrielle Vaughn, Andrea Lambert, Michael Wallendorf, James Kirklin, Charles E Canter
Background: There are conflicting data regarding the relationship between center volume and outcomes in pediatric heart transplantation. Previous studies have not fully accounted for differences in case mix, particularly in high-risk congenital heart disease (CHD) groups. We aimed to evaluate the relationship between center volume and outcomes using the Pediatric Heart Transplant Society (PHTS) Registry and explore how case mix may affect outcomes.
Methods: A retrospective cohort study of all pediatric patients in the PHTS Registry who received a heart transplant from 2009 to 2018 was performed. Centers were divided into 5 groups based on average yearly transplant volume. The primary outcome was time to death or graft loss and outcomes were compared using Kaplan-Meier analysis.
Results: There were 4583 cases among 55 centers included. There was no difference in time to death or graft loss by center volume in the entire cohort (p = .75), in patients with CHD (p = .79) or in patients with cardiomyopathy (p = .23). There was also no difference in time to death or graft loss by center size in patients undergoing transplant after Norwood, Glenn or Fontan (log rank p = .17, p = .31, and p = .10 respectively). There was a statistically significant difference in outcomes by center size in the positive crossmatch group (p < .0001), though no discernible pattern related to high or low center volume.
Conclusions: Outcomes are similar among transplant centers of all sizes, including for high-risk patient groups with CHD. Future work is needed to understand how patient-specific risk factors may vary among centers of various sizes and whether this influences patient outcomes.
背景:关于中心规模与小儿心脏移植手术结果之间的关系,存在相互矛盾的数据。以往的研究没有充分考虑病例组合的差异,尤其是高风险先天性心脏病(CHD)组。我们的目的是利用小儿心脏移植协会(PHTS)登记册评估中心规模与疗效之间的关系,并探讨病例组合如何影响疗效:我们对PHTS注册中心2009年至2018年接受心脏移植的所有儿科患者进行了一项回顾性队列研究。根据年平均移植量将中心分为 5 组。主要结果是死亡或移植物丢失的时间,并使用卡普兰-梅耶尔分析法对结果进行比较:结果:55个中心共纳入4583个病例。在整个队列中,死亡或移植物丢失的时间在中心数量(P = .75)、心脏病患者(P = .79)或心肌病患者(P = .23)中没有差异。诺伍德、格伦或丰坦移植患者的死亡时间或移植物损失也没有因中心大小而异(对数秩分别为 p = .17、p = .31 和 p = .10)。在交叉配型阳性组中,不同中心规模的移植结果差异有统计学意义(p 结论:不同中心规模的移植结果相似:各种规模的移植中心,包括患有心脏病的高风险患者群体,其结果都是相似的。今后需要开展工作,以了解患者的特定风险因素在不同规模的移植中心之间有何差异,以及这是否会影响患者的预后。
{"title":"Survival does not differ by annual center transplant volume-A Pediatric Heart Transplant Society Registry study.","authors":"A Marion Ybarra, Alicia M Kamsheh, Matthew J O'Connor, Seth A Hollander, Maria Bano, Michelle Ploutz, Gabrielle Vaughn, Andrea Lambert, Michael Wallendorf, James Kirklin, Charles E Canter","doi":"10.1111/petr.14720","DOIUrl":"10.1111/petr.14720","url":null,"abstract":"<p><strong>Background: </strong>There are conflicting data regarding the relationship between center volume and outcomes in pediatric heart transplantation. Previous studies have not fully accounted for differences in case mix, particularly in high-risk congenital heart disease (CHD) groups. We aimed to evaluate the relationship between center volume and outcomes using the Pediatric Heart Transplant Society (PHTS) Registry and explore how case mix may affect outcomes.</p><p><strong>Methods: </strong>A retrospective cohort study of all pediatric patients in the PHTS Registry who received a heart transplant from 2009 to 2018 was performed. Centers were divided into 5 groups based on average yearly transplant volume. The primary outcome was time to death or graft loss and outcomes were compared using Kaplan-Meier analysis.</p><p><strong>Results: </strong>There were 4583 cases among 55 centers included. There was no difference in time to death or graft loss by center volume in the entire cohort (p = .75), in patients with CHD (p = .79) or in patients with cardiomyopathy (p = .23). There was also no difference in time to death or graft loss by center size in patients undergoing transplant after Norwood, Glenn or Fontan (log rank p = .17, p = .31, and p = .10 respectively). There was a statistically significant difference in outcomes by center size in the positive crossmatch group (p < .0001), though no discernible pattern related to high or low center volume.</p><p><strong>Conclusions: </strong>Outcomes are similar among transplant centers of all sizes, including for high-risk patient groups with CHD. Future work is needed to understand how patient-specific risk factors may vary among centers of various sizes and whether this influences patient outcomes.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":null,"pages":null},"PeriodicalIF":1.3,"publicationDate":"2024-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140022406","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}