Annals of Transplantation最新文献

{"title":"Free-Circulating Nucleic Acids as Biomarkers in Patients After Solid Organ Transplantation.","authors":"Joanna Raszeja-Wyszomirska,&nbsp;Michał Macech,&nbsp;Monika Kolanowska,&nbsp;Marek Krawczyk,&nbsp;Sławomir Nazarewski,&nbsp;Anna Wójcicka,&nbsp;Jolanta Małyszko","doi":"10.12659/AOT.939750","DOIUrl":"https://doi.org/10.12659/AOT.939750","url":null,"abstract":"<p><p>A number types of extracellular DNA (eg, cell-free, cfDNA) circulate in human blood, including mitochondrial, transcriptome, and regulatory DNA, usually at low concentrations. Larger amounts of cfDNA appear in any inflammatory condition, including organ damage due to a variety of reasons. The role of cfDNA in solid organ transplantation is discussed in this review as a valuable additional tool in the standard of care of transplant patients. Post-transplant monitoring requires the use of high-quality biomarkers for early detection of graft damage or rejection to be able to apply early therapeutic intervention. CfDNA complements the traditional monitoring strategies, being a risk stratification tool and an important prognostic marker. However, improving the sensitivity and specificity of cfDNA detection is necessary to facilitate personalized patient management, warranting further research in terms of measurement, test standardization, and storage, processing, and shipping. A diagnostic test (Allosure, CareDx, Inc., Brisbane, CA) for kidney, heart and lung transplant patients is now commercially available, and validation for other organs (eg, liver) is pending. To date, donor-derived cfDNA in combination with other biomarkers appears to be a promising tool in graft rejection as it is minimally invasive, time-sensitive, and cost-effective. However, improvement of sensitivity and specificity is required to facilitate personalized patient management. Whether it could be an alternate to graft biopsy remains unclear.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e939750"},"PeriodicalIF":1.1,"publicationDate":"2023-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/6e/55/anntransplant-28-e939750.PMC10439677.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10048278","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Graft and Patient Survival in Kidney Transplant with Deceased Donor Using KDRI (Kidney Donor Risk Index), KDPI (Kidney Donor Profile Index), and EPTS (Estimated Post-Transplant Survival) in Colombia.","authors":"Anabel Vanin A,&nbsp;Luis Alfonso Valderrama Cometa,&nbsp;Carlos Fernando Acuña Roldan,&nbsp;Norman A Alhajj,&nbsp;Carlos Julián Devia Santacruz","doi":"10.12659/AOT.940522","DOIUrl":"https://doi.org/10.12659/AOT.940522","url":null,"abstract":"<p><p>BACKGROUND EPTS (Estimated Post-Transplant Survival), KDRI (Kidney Donor Risk Index), and KDPI (Kidney Donor Profile Index) were developed aiming to ameliorate donor-recipient longevity matching in kidney transplants. They are based on a prediction model made using the United States population; evidence of their use outside EEUU remains limited. The aim of this study was to describe the quality of deceased-donor kidneys and to determine recipient and graft survival, glomerular filtration rate, and incidence of delayed graft function in renal transplantation according to these indices in Cali, Colombia. MATERIAL AND METHODS In this historical cohort study, Kaplan-Meier method was used to analyze survival of recipient and graft according to the values of the indices categorized by quintiles. Glomerular filtration rate and incidence of delayed graft function were also analyzed according to KDRI and KDPI. RESULTS We included 380 patients. Medians of EPTS, KDRI, and KDPI were 24% (IQR 9-60), 0.8 (IQR 0.71-0.99), and 27% (IQR 13-49), respectively. Two-year survival was 97.8% in recipients with EPTS ≤20% and it decreased with higher values of the index. Recipient and graft survival were lower for all periods when donors had KDPI >80%. Incidence of delayed graft function was higher in patients whose donors had KDPI ≥60% (44% vs 21%). Glomerular filtration rate decreased with the highest values of KDPI for all periods. CONCLUSIONS Our study represents the initial evaluation of the usefulness of these indices in Colombia. Our results suggest that KDRI, KDPI, and EPTS may serve as valuable tools for kidney allocation in our setting. Further research with larger sample sizes is necessary to validate these indices in our population.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e940522"},"PeriodicalIF":1.1,"publicationDate":"2023-08-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/1a/8c/anntransplant-28-e940522.PMC10403991.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9948033","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Recurrent Membranoproliferative Glomerulonephritis After Kidney Transplantation: Risk Factors and Impact on Graft Survival.","authors":"Lais Ceccatto de Paula,&nbsp;Marilda Mazzali,&nbsp;Marcos Vinicius de Sousa","doi":"10.12659/AOT.940502","DOIUrl":"https://doi.org/10.12659/AOT.940502","url":null,"abstract":"<p><p>BACKGROUND Membranoproliferative glomerulonephritis (MPGN) is an uncommon cause of end-stage renal disease (ESRD). Recurrence rates after transplantation range from 11.8% to 18.9% after 5 and 15 years, respectively. This study aimed to assess the risk factors of MPGN recurrence after kidney transplantation and its impact on graft survival. MATERIAL AND METHODS This was a single-center retrospective cohort, including renal transplant recipients older than 18 years, with a diagnosis of MPGN in native kidneys. Data were obtained from medical records during the first 5-year post-transplant follow-up. Primary endpoints were graft function and survival. Secondary endpoints were MPGN recurrence risk factors and these cases' clinical, laboratory, and histological features. RESULTS Twenty-eight patients were included; the majority male (60.7%), with a mean age of 24.0±9.4 years. At MPGN native diagnosis, all patients presented proteinuria, with C3 consumption in 42.9%. Histological analysis showed 13 (42.9%) MPGN type I and 5 (17.9%) type II, with no cases of type III. MPGN recurrence occurred in 7 (25.0%) patients; 85.7% were male, 57.1% were recipients from a living donor, all presenting nephrotic syndrome and hematuria, with C3 consumption in 71.4%. The graft function was similar between the groups. Two (28.6%) patients progressed to graft failure in the recurrence group, and 1 died with a functioning graft. CONCLUSIONS The MPGN recurrence rate was 25%, most of them recipients of kidneys from living donors. Nephrotic syndrome and C3 consumption were frequent at recurrence. The graft function was similar between the groups, and the 5-year graft survival rate in the recurrence group was higher than in other studies.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e940502"},"PeriodicalIF":1.1,"publicationDate":"2023-07-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/9f/0a/anntransplant-28-e940502.PMC10362803.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9847533","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Drug Overdose and Cardiovascular Deaths Among Deceased Organ Donors: Implications for Donor Utilization and Data Reporting.","authors":"Michie A Adjei,&nbsp;Steven A Wisel,&nbsp;Irene K Kim,&nbsp;Justin A Steggerda","doi":"10.12659/AOT.940255","DOIUrl":"https://doi.org/10.12659/AOT.940255","url":null,"abstract":"<p><p>BACKGROUND The present study evaluated expanded cause of death (COD) definitions and its implications on donor utilization for solid organ transplantation. MATERIAL AND METHODS The OPTN Standard Transplant and Research file was queried for potential donors between 2005 and 2019. Donor- and organ-specific utilization were evaluated. Expanded donor COD were identified: trauma, cardiovascular (CV), cerebrovascular accident (CVA) or stroke, drug intoxication (DI), anoxia not otherwise specified (NOS), and other. Descriptive analyses and multivariable logistic regression analyses for donor utilization were performed. RESULTS Among 132 783 potential donors identified, the most common COD was CVA/Stroke (n=44 707, 33.7%), followed by trauma (n=43 356, 32.7%), CV (n=20 053, 15.1%), anoxia-NOS (n=12 261, 9.2%), DI (n=10 205, 7.7%), and other causes (n=2201, 1.7%). Significant differences between CV, DI, and anoxia-NOS groups existed for donor age, sex, ethnicity, body mass index, and comorbidities. Donors from trauma had the highest unadjusted utilization rate (97.2%) while CV donors had the lowest (90.1%). Multivariable analysis of brain-dead donors (DBD) showed that compared to trauma, donors from DI had higher likelihood of utilization (odds ratio 1.217, 95% 1.025-1.446) while CV donors were lower (OR 0.717, 95% CI 0.642-0.800, P<0.001). Among donation after circulatory death (DCD) donors, there was decreased utilization compared to trauma for both CV (OR 0.607, 95% CI 0.523-0.705) and DI (OR 0.754, 95% CI 0.603-0.914, P<0.001). CONCLUSIONS Current COD definitions should be expanded to capture significant differences in the donor population. DI donors are the fastest growing cohort and the most likely utilized DBD donors, while trauma donors remain the most likely utilized DCD donors.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e940255"},"PeriodicalIF":1.1,"publicationDate":"2023-07-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/dc/2b/anntransplant-28-e940255.PMC10329409.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9764546","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A 15-Year Retrospective Study of Supportive Extracorporeal Therapies Including Plasma Exchange and Continuous Venovenous Hemodiafiltration of 114 Adults with Acute Liver Failure Awaiting Liver Transplantation.","authors":"Ilhan Ocak","doi":"10.12659/AOT.939745","DOIUrl":"https://doi.org/10.12659/AOT.939745","url":null,"abstract":"<p><p>BACKGROUND Recently, there has been a recommendation to utilize a combination of supportive extracorporeal therapies, specifically plasma exchange and continuous venovenous hemodiafiltration, in patients with acute liver failure. This 15-year retrospective study aimed to evaluate supportive extracorporeal therapy, including plasma exchange and continuous venovenous hemodiafiltration, for 114 adults with acute liver failure awaiting liver transplant. MATERIAL AND METHODS In this retrospective study, the medical records of 1288 adult patients who underwent liver transplantation and 161 adult patients who received alternative therapy were analyzed; 114 patients who received combined supportive extracorporeal therapy for acute liver failure were included in the study. Biochemical laboratory data were compared before and after therapy. RESULTS The study included 50 male and 64 female patients. The first group (34 patients) recovered with liver transplantation, and 4 patients died in the first year after liver transplantation. In the second group (80 patients), 66 patients recovered without liver transplantation, while 14 patients died within the first 2 weeks after therapy. All patients showed significant reductions in serum hepatic function tests (alanine transaminase, aspartate transaminase, and total bilirubin), ammonia, and prothrombin time/international normalized ratio after discontinuation of combined supportive extracorporeal therapy (P<0.01). There was also a significant improvement in the hemodynamic parameter. CONCLUSIONS This combined extracorporeal therapy can be used as a supportive treatment for both recovery and bridge to liver transplantation in patients with acute liver failure. In addition, treatment can be continued until liver regeneration and until a usable donor is found.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e939745"},"PeriodicalIF":1.1,"publicationDate":"2023-06-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/52/27/anntransplant-28-e939745.PMC10314717.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9741336","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Survival analysis of transplant-associated thrombotic microangiopathy under different diagnostic criteria and the efficacy of plasma exchange.","authors":"Yifan Xu,&nbsp;Yan Wei,&nbsp;Lijun Wang,&nbsp;Ning Lu,&nbsp;Yongli Wu,&nbsp;Liping Dou,&nbsp;Daihong Liu,&nbsp;Meng Li,&nbsp;Chunji Gao","doi":"10.12659/AOT.939890","DOIUrl":"https://doi.org/10.12659/AOT.939890","url":null,"abstract":"<p><p>BACKGROUND Transplant-associated thrombotic microangiopathy (TA-TMA) is a serious complication of hematopoietic stem cell transplantation (HSCT). The efficacy and survival of plasma exchange (PE) for TA-TM have not been fully clarified. In addition, there is a lack of consensus on diagnostic criteria for TA-TMA.  MATERIAL AND METHODS We retrospectively analyzed 32 patients diagnosed with TA-TMA by different diagnostic criteria from January 2018 to February 2022 at the First Medical Center of the PLA General Hospital. RESULTS (1) The patients with TA-TMA treated with PE in this study had a remission rate of 42.8%, a 100-day OS of 47.6%, and a 6-month OS of 38.1%. The only factor affecting the response to PE treatment was the number of PE sessions (P = 0.047). (2) III-IV aGVHD prior to TA-TMA diagnosis (P = 0.002), renal or neurological dysfunction (P = 0.021), and the time to onset of TA-TMA (P = 0.002) were independent risk factors for overall survival with TA- TMA. (3) Probable TA-TMA had the highest survival rate, but the Jodele criteria are expected to diagnose earlier and provide the greatest benefit to patients. CONCLUSIONS PE is an effective treatment for TA-TMA especially in cases where complement blockers are not available. In addition, probable TA-TMA improved prognostic survival through early detection of patients with TA-TMA. There is a need for further large prospective trials to identify the population more suitable for PE treatment of TA-TMA and more valid diagnostic criteria.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e939890"},"PeriodicalIF":1.1,"publicationDate":"2023-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/bf/d2/anntransplant-28-e939890.PMC10290434.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10066271","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Errate: Modification of Venous Outflow to Avoid Thrombotic Graft Failure in Pancreas Transplantation.","authors":"Je Ho Ryu,&nbsp;Jae Ryong Shim,&nbsp;Tae Beom Lee,&nbsp;Kwangho Yang,&nbsp;Taeun Kim,&nbsp;Seo Rin Kim,&nbsp;Byunghyun Choi","doi":"10.12659/AOT.941495","DOIUrl":"https://doi.org/10.12659/AOT.941495","url":null,"abstract":"<p><p>The authors asked for an errata to correct the affiliation information. The corrected affiliations are as follows:Je Ho Ryu1,2, Jae Ryong Shim1, Tae Beom Lee1, Kwang Ho Yang1, Taeun Kim3, Seo Rin Kim4, Byung Hyun Choi1,21 Division of Hepato-Biliary-Pancreatic Surgery and Transplantation, Department of Surgery, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, South Korea2 Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Yangsan, South Korea3 Department of Radiology, Pusan National University Yangsan Hospital, Pusan National University School of Medicine, Yangsan, South Korea4 Department of Internal medicine, Pusan National University Yangsan Hospital, Pusan National University School of Medicine,  Yangsan, South KoreaThe change of affiliation does not affect the content or findings of the publication in any way. It is solely an update to the -authors' institutional affiliations.Reference:Je Ho Ryu, Jae Ryong Shim, Tae Beom Lee, Kwangho Yang, Taeun Kim, Seo Rin Kim, Byunghyun Choi. Modification of Venous Outflow to Avoid Thrombotic Graft Failure in Pancreas Transplantation. Ann Transplant. 2022; 27: e937514. DOI: 10.12659/AOT.937514.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e941495"},"PeriodicalIF":1.1,"publicationDate":"2023-06-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/22/fa/anntransplant-28-e941495.PMC10288887.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9699959","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Incidence of Thromboembolic Complications Following Kidney Transplantation with Short and Extended Aspirin Prophylaxis: A Retrospective Single-Center Study.","authors":"Angus H Pegler,&nbsp;Katharine Hegerty,&nbsp;Ryan P Gately,&nbsp;Carmel M Hawley,&nbsp;David W Johnson,&nbsp;Yeoungjee Cho,&nbsp;Dev K Jegatheesan,&nbsp;Andrew B McCann,&nbsp;Michelle E Harfield,&nbsp;Nicole M Isbel","doi":"10.12659/AOT.939143","DOIUrl":"https://doi.org/10.12659/AOT.939143","url":null,"abstract":"<p><p>BACKGROUND Aspirin prophylaxis has been associated with reduced graft-related thrombosis following kidney transplantation. Aspirin cessation, however, can increase risk of venous thromboembolic complications, including pulmonary thromboembolism and deep venous thrombosis. This single-center, retrospective, pre-post interventional study from Brisbane, Australia, aimed to compare the rate of thrombotic complications in 1208 adult kidney transplant recipients receiving postoperative aspirin for 5 days or >6 weeks. MATERIAL AND METHODS We enrolled1208 kidney transplant recipients who received 100 mg aspirin for 5 days (n=571) or >6 weeks (n=637) postoperatively. The primary outcome was venous thromboembolism (VTE) in the first 6 weeks after transplant, examined by multivariable logistic regression analysis. Secondary outcomes were renal vein/artery thrombosis, 1-month serum creatinine, rejection, myocardial infarction, stroke, blood transfusion, dialysis at day 5 and day 28, and mortality. RESULTS Sixteen (1.3%) patients experienced VTE (5-day n=8, 1.4%; >6-week n=8, 1.3%; P=0.8). Extended aspirin duration was not independently associated with a reduction in VTE (OR 0.91, 95% CI 0.32-2.57; P=0.9). Graft thrombosis was rare (n=3, 0.25%). Aspirin duration was not associated with cardiovascular events, blood transfusion, graft thrombosis, graft dysfunction, rejection, or mortality. VTE was independently associated with older age (OR 1.09, 95% CI 1.04-1.16; P=0.002), smoking (OR 3.59, 95% CI 1.20-13.2; P=0.032), younger donor age (OR 0.96, 95% CI 0.93-1.00; P=0.036), and thymoglobulin use (OR 10.5, 95% CI 3.09-32.1; P≥0.001). CONCLUSIONS Extended-duration aspirin use did not significantly reduce the incidence of VTE in the first 6 weeks following kidney transplantation. An association was identified between anti-human thymocyte immunoglobulin and VTE, which requires further assessment.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e939143"},"PeriodicalIF":1.1,"publicationDate":"2023-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/4c/f9/anntransplant-28-e939143.PMC10276531.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9654599","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Results of Liver Retransplantation After Rescue Hepatectomy: A Single-Center Study.","authors":"Erika Laine,&nbsp;Hanna Al Sabeah,&nbsp;Marie Tranäng,&nbsp;Antonio Romano,&nbsp;Greg Nowak","doi":"10.12659/AOT.939557","DOIUrl":"https://doi.org/10.12659/AOT.939557","url":null,"abstract":"<p><p>BACKGROUND Liver retransplantation (reLT) is a well-accepted treatment for liver graft failure in selected patients. A rescue hepatectomy (RH), on the contrary, is a rare and controversial procedure in which a deteriorating liver graft causing failure of other organ systems is removed to stabilize the patient's condition before a new liver graft is available. MATERIAL AND METHODS In this retrospective cohort study, we evaluated the outcomes of the 104 patients who were listed for a first single-organ reLT in our center during the period 2000-2019, to compare the results after RH to other reLTs. RESULTS In the study population, RH was performed on 8 patients, while 7 of these received a new graft (8% of all first time reLTs) and 1 died before reLT. All RHs were performed within 1 week after the first transplantation. The median anhepatic time after RH was 36 hours (range 14-99). The 1-year patient survival rate was 57% for reLTs with RH and 69% for acute reLTs without RH that were performed within 14 days after the first transplantation (P=0.66). The 5-year survival rate was 50% in the RH and 47% in the non-RH group (P=1.0). CONCLUSIONS The use of RH prior to reLT results in a similar outcome to reLTs without RH. Therefore, RH should be considered in patients with a severe clinical instability caused by a deteriorating liver graft. However, further studies are needed to establish guidelines based on objective parameters for when RH should be performed.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e939557"},"PeriodicalIF":1.1,"publicationDate":"2023-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/38/dd/anntransplant-28-e939557.PMC10257354.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9608890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Outcomes from a Single Transplant Center of 5 Pediatric Cases of Domino Liver Transplantation from Live Donors with Maple Syrup Urine Disease.","authors":"Jin-Ping Zhang,&nbsp;Zhi-Jun Zhu,&nbsp;Li-Ying Sun,&nbsp;Lin Wei,&nbsp;Wei Qu,&nbsp;Zhi-Gui Zeng,&nbsp;Hai-Ming Zhang,&nbsp;Ying Liu","doi":"10.12659/AOT.939893","DOIUrl":"https://doi.org/10.12659/AOT.939893","url":null,"abstract":"<p><p>BACKGROUND Maple syrup urine disease (MSUD) is a rare genetic deficiency of the branched-chain alpha-keto acid dehydrogenase (BCKAD) complex that breaks down amino acids, resulting in multi-organ failure. This report is of 5 pediatric cases of domino liver transplantation (DLT) from live donors with MSUD from a single transplant center in Beijing. CASE REPORT All MSUD donors were confirmed to have disease-causing mutations in BCKDHA (branched-chain keto acid dehydrogenase E1, alpha polypeptide) or BCKDHB (branched-chain keto acid dehydrogenase E1, ß polypeptide) genes by peripheral blood whole-exon sequencing. Serum leucine and valine concentrations were significantly higher than normal values. Recipients ranged in age from 0.75 to 9 years old. Three patients underwent auxiliary liver transplantation, and the other children all underwent liver or partial liver transplantation. This case report was followed up for 25 to 79 months. The prognosis, growth, and development of patients were followed up. By the end of the last follow-up, all children had survived. All patients had normal serum leucine and valine concentrations after surgery. In case 1, portal vein stenosis post-operatively. In case 2, stenosis of hepatic artery and bile duct occurred. In case 5, hepatic artery and portal vein stenosis occurred, resulting in graft loss.   CONCLUSIONS The findings from our center support the findings from other pediatric liver transplant centers that liver transplantation using MSUD donors can have successful outcomes without the development of MSUD in the recipient.</p>","PeriodicalId":7935,"journal":{"name":"Annals of Transplantation","volume":"28 ","pages":"e939893"},"PeriodicalIF":1.1,"publicationDate":"2023-05-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/e8/54/anntransplant-28-e939893.PMC10239205.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9928448","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}