Anjali Rai, Lauren Sproule, Tatianna Larman, Kiyoko Oshima, Daniel Rhee, Kenneth Ng, Elizabeth King, Douglas Mogul, Kathryn Lemberg
{"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
{"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}
{"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}
{"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
{"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
{"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
{"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}