Pediatric Transplantation最新文献

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Management of Trichodysplasia Spinulosa in a Pediatric Kidney Transplant Patient With Topical Cidofovir and Oral Leflunomide. 用局部西多福韦和口服来氟米特治疗小儿肾移植患者的棘层毛囊炎
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70080
Annika M Hansen, Sonia Mehra, Anneli R Bowen, Joseph Sherbotie, Sarah D Cipriano
{"title":"Management of Trichodysplasia Spinulosa in a Pediatric Kidney Transplant Patient With Topical Cidofovir and Oral Leflunomide.","authors":"Annika M Hansen, Sonia Mehra, Anneli R Bowen, Joseph Sherbotie, Sarah D Cipriano","doi":"10.1111/petr.70080","DOIUrl":"10.1111/petr.70080","url":null,"abstract":"<p><strong>Background: </strong>This case report describes a 10-year-old male with a history of kidney transplantation who developed trichodysplasia spinulosa (TS), a rare skin disorder associated with immunosuppression.</p><p><strong>Methods: </strong>The patient's condition, characterized by follicular papules and keratinous spines primarily on the face, was managed by a multidisciplinary team.</p><p><strong>Results: </strong>Treatment involved reducing immunosuppression and administering topical cidofovir alongside oral leflunomide, leading to significant clinical improvement over several months.</p><p><strong>Conclusions: </strong>This case adds to the literature of TS management and highlights the importance of multidisciplinary care as well as the challenges of treatment accessibility due to insurance limitations.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70080"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11979675/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143812036","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}
引用次数: 0
The Potential of ChatGPT as a Source of Information for Kidney Transplant Recipients and Their Caregivers. ChatGPT作为肾移植受者及其护理者信息来源的潜力。
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70068
Kaan Can Demirbaş, Seha Saygılı, Esra Karabağ Yılmaz, Rüveyda Gülmez, Ayşe Ağbaş, Mehmet Taşdemir, Nur Canpolat
{"title":"The Potential of ChatGPT as a Source of Information for Kidney Transplant Recipients and Their Caregivers.","authors":"Kaan Can Demirbaş, Seha Saygılı, Esra Karabağ Yılmaz, Rüveyda Gülmez, Ayşe Ağbaş, Mehmet Taşdemir, Nur Canpolat","doi":"10.1111/petr.70068","DOIUrl":"10.1111/petr.70068","url":null,"abstract":"<p><strong>Background: </strong>Education and enhancing the knowledge of adolescents who will undergo kidney transplantation are among the primary objectives of their care. While there are specific interventions in place to achieve this, they require extensive resources. The rise of large language models like ChatGPT-3.5 offers potential assistance for providing information to patients. This study aimed to evaluate the accuracy, relevance, and safety of ChatGPT-3.5's responses to patient-centered questions about pediatric kidney transplantation. The objective was to assess whether ChatGPT-3.5 could be a supplementary educational tool for adolescents and their caregivers in a complex medical context.</p><p><strong>Methods: </strong>A total of 37 questions about kidney transplantation were presented to ChatGPT-3.5, which was prompted to respond as a health professional would to a layperson. Five pediatric nephrologists independently evaluated the outputs for accuracy, relevance, comprehensiveness, understandability, readability, and safety.</p><p><strong>Results: </strong>The mean accuracy, relevancy, and comprehensiveness scores for all outputs were 4.51, 4.56, and 4.55, respectively. Out of 37 outputs, four were rated as completely accurate, and seven were completely relevant and comprehensive. Only one output had an accuracy, relevancy, and comprehensiveness score below 4. Twelve outputs were considered potentially risky, but only three had a risk grade of moderate or higher. Outputs that were considered risky had an accuracy and relevancy below the average.</p><p><strong>Conclusion: </strong>Our findings suggest that ChatGPT could be a useful tool for adolescents or caregivers of individuals waiting for kidney transplantation. However, the presence of potentially risky outputs underscores the necessity for human oversight and validation.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70068"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143616777","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}
引用次数: 0
Frailty in Pediatric Liver Disease May Be Associated With an Increased Incidence of Readmissions After Pediatric Liver Transplantation. 儿童肝脏疾病虚弱可能与儿童肝移植后再入院发生率增加有关。
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70077
Saleh A Alqahtani, Michael Shpoliansky, Shannon M Vandriel, Fatema Johara, Claudia Quammie, Eberhard Lurz, Estella M Alonso, James F Daniel, Veena L Venkat, Daniel H Leung, Julie Economides, Evelyn K Hsu, Kathleen M Loomes, Nitika A Gupta, Dana Mannino, Jerome Menendez, Vicky L Ng, Binita M Kamath
{"title":"Frailty in Pediatric Liver Disease May Be Associated With an Increased Incidence of Readmissions After Pediatric Liver Transplantation.","authors":"Saleh A Alqahtani, Michael Shpoliansky, Shannon M Vandriel, Fatema Johara, Claudia Quammie, Eberhard Lurz, Estella M Alonso, James F Daniel, Veena L Venkat, Daniel H Leung, Julie Economides, Evelyn K Hsu, Kathleen M Loomes, Nitika A Gupta, Dana Mannino, Jerome Menendez, Vicky L Ng, Binita M Kamath","doi":"10.1111/petr.70077","DOIUrl":"https://doi.org/10.1111/petr.70077","url":null,"abstract":"<p><strong>Background: </strong>Frailty is a phenotype of cumulative decline leading to decreased physiologic reserve and vulnerability to stressors. Frailty is associated with adverse outcomes after liver transplantation (LT) in adults, but similar data are not available in children. A prospective multicenter study previously determined that frailty is present in 46% of children with end-stage liver disease (ESLD). We utilized this cohort to evaluate the impact of pre-transplant frailty on post-LT outcomes.</p><p><strong>Methods: </strong>The study included pediatric participants from the original frailty study across 10 North American transplant centers who had subsequently undergone LT. Clinical outcomes were collected up to 1 year post LT. Participants were stratified by their pre-transplant frailty score (defined by a pre-LT frailty score of ≥ 6.0) and long-term outcomes were compared between groups.</p><p><strong>Results: </strong>28 (60.7% female, 46.4% biliary atresia) pediatric LT recipients were included, and 54% of children met criteria for frailty (n = 15). Baseline characteristics were comparable between groups; however, those with frailty were significantly more likely to have pre-transplant failure to thrive (33.3% vs. 0%, p = 0.044). Thirty-four hospital readmissions (22 in frail and 12 in non-frail children) occurred in 20 patients. Higher pre-transplant frailty scores were also significantly associated with an increased number of readmissions after transplantation (p = 0.034).</p><p><strong>Conclusions: </strong>Pediatric frailty may be associated with the adverse outcome of increased frequency of hospitalization in the first year after pediatric liver transplantation. These data support the concept that frail children should be identified and targeted for prehabilitation prior to LT.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70077"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144018710","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}
引用次数: 0
Ten-Year Pediatric Heart Transplantation: Insights and Outcomes From a Leading Center in Iran. 十年儿童心脏移植:来自伊朗领先中心的见解和结果。
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70081
Mohammad Mahdavi, Maziar Gholampour Dehaki, Alireza Yaghoubi, Hossein Shahzadi, Amirhosein Jalali, Hassan Tatari, Yaser Tolouei Tabar, Ali Sadeghpour Tabaei, Zahra Ansari Aval, Mohammad Zia Totonchi, Hossein Mehrali, Alireza Pousti, Golnar Mortaz Hejri
{"title":"Ten-Year Pediatric Heart Transplantation: Insights and Outcomes From a Leading Center in Iran.","authors":"Mohammad Mahdavi, Maziar Gholampour Dehaki, Alireza Yaghoubi, Hossein Shahzadi, Amirhosein Jalali, Hassan Tatari, Yaser Tolouei Tabar, Ali Sadeghpour Tabaei, Zahra Ansari Aval, Mohammad Zia Totonchi, Hossein Mehrali, Alireza Pousti, Golnar Mortaz Hejri","doi":"10.1111/petr.70081","DOIUrl":"https://doi.org/10.1111/petr.70081","url":null,"abstract":"<p><strong>Introduction: </strong>Heart transplantation is the definitive treatment for infants and children with nine advanced stages of congenital heart failure. This study presents the experiences of a single medical center regarding pediatric heart transplants conducted over a decade.</p><p><strong>Methods: </strong>Baseline characteristics of recipients and donors of pediatric heart transplant patients from 2012 to 2021 were recorded. Post-transplant complications and survival rates were assessed during a median follow-up period of 2 years.</p><p><strong>Results: </strong>The study, which spanned a decade and included 225 transplants, revealed several key findings. The mean age of recipients was 10.8 years, with a majority being boys. The most common diagnoses were dilated cardiomyopathy (83.6%) and restrictive cardiomyopathy (8.0%). Donors, with a mean age of 21.4 years, were predominantly male. The primary causes of donor death were head trauma/motor vehicle accidents (56.9%). Notably, post-transplant complications included Renal failure requiring dialysis (15.7%), Central Nervous System (16.9%), Cytomegalovirus infections (24.9%), and Epstein-Barr virus infections (21.8%). During the follow-up, 48 deaths were recorded, yielding a crude mortality rate of 7.4 per 100 person-years. The 1-year, 3-year, and 5-year survival rates, which were 85.7%, 79.7%, and 73.9%, respectively, demonstrate the significant progress in pediatric heart transplant outcomes over the past decade.</p><p><strong>Conclusion: </strong>Pediatric heart transplant outcomes have improved over the past decade, although challenges remain. Significant risk factors for mortality include donor age, male donor gender, and 28 Rituximab treatment. Strategies to mitigate these risks could enhance survival rates post-transplant.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70081"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144036588","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}
引用次数: 0
Delayed-Onset Renal Allograft Compartment Syndrome in a Pediatric Kidney Transplant Recipient: The Role of Surgical Re-Evaluation. 儿童肾移植受者的迟发型异体肾隔室综合征:手术重新评估的作用。
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70070
Buğra Otludil, Gülşah Kaya Aksoy, Mustafa Koyun, Elif Çomak, Ali Avanaz, Bahar Akkaya, Bülent Aydınlı, Sema Akman
{"title":"Delayed-Onset Renal Allograft Compartment Syndrome in a Pediatric Kidney Transplant Recipient: The Role of Surgical Re-Evaluation.","authors":"Buğra Otludil, Gülşah Kaya Aksoy, Mustafa Koyun, Elif Çomak, Ali Avanaz, Bahar Akkaya, Bülent Aydınlı, Sema Akman","doi":"10.1111/petr.70070","DOIUrl":"10.1111/petr.70070","url":null,"abstract":"<p><strong>Background: </strong>Renal allograft compartment syndrome (RACS) represents a rare and potentially underdiagnosed cause of allograft dysfunction, typically occurring in the immediate post-transplant period. However, delayed-onset RACS remains underrecognized.</p><p><strong>Methods: </strong>We present a case of a 14.5-year-old girl with nephronophthisis, who received a kidney transplant from her father and who was diagnosed with late-onset renal allograft compartment syndrome on post-transplant day 20.</p><p><strong>Results: </strong>In the post-transplant follow-up of this patient, the serum creatinine level increased from the 2nd day. On day 5, surgical re-evaluation was performed as she did not respond to acute rejection therapy with prednisolone, anti-human T-lymphocyte immunoglobulin, and plasmapheresis. Although macroscopic examination of the allograft did not reveal any abnormalities, serum creatinine levels rapidly returned to normal after surgery. However, on the 20th day, serum creatinine started to rise again. Calcineurin toxicity and infectious causes have been ruled out. She received treatment with immunoadsorption and intravenous immunoglobulin as the second biopsy showed glomerulitis and tubulitis. Despite these treatments, the serum creatinine level increased to 6 mg/dL, and she was reassessed surgically. Although the allograft appeared normal, there was edema in the surrounding tissue. Serum creatinine levels returned to normal (0.9 mg/dL) spontaneously after surgery. So, she was diagnosed with late-onset renal allograft compartment syndrome due to the edema surrounding the allograft and improvement observed in serum creatinine levels after fascia opening.</p><p><strong>Conclusions: </strong>RACS should be considered beyond the immediate post-transplant period, particularly when standard interventions fail to improve graft function. Surgical exploration remains a critical diagnostic and therapeutic tool in such cases.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70070"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143710715","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}
引用次数: 0
Into Adulthood: Assessing Parental Perceptions and Concerns for Pediatric Heart Transplant Recipients Around the Transition Into Adult Care. 进入成年期:评估父母对儿童心脏移植受者过渡到成人护理的看法和关注。
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70062
Katya Kaplow, Max C Downey, Conor Donnelly, Joseph P Hillenburg, Melissa McQueen, Kathleen Anderson, Melissa K Cousino, Manu Varma, Rakesh K Singh, Carolyn N Sidoti, Allan B Massie, Dorry L Segev, Macey L Levan
{"title":"Into Adulthood: Assessing Parental Perceptions and Concerns for Pediatric Heart Transplant Recipients Around the Transition Into Adult Care.","authors":"Katya Kaplow, Max C Downey, Conor Donnelly, Joseph P Hillenburg, Melissa McQueen, Kathleen Anderson, Melissa K Cousino, Manu Varma, Rakesh K Singh, Carolyn N Sidoti, Allan B Massie, Dorry L Segev, Macey L Levan","doi":"10.1111/petr.70062","DOIUrl":"10.1111/petr.70062","url":null,"abstract":"<p><strong>Background: </strong>Parents of pediatric heart transplant (HTx) recipients have a unique perspective on the challenges associated with the transition into adult care networks. We sought to assess parental perceptions of the challenges pediatric HTx recipients face daily and parental concerns around the transition from pediatric care networks.</p><p><strong>Methods: </strong>A 15-item online survey was developed in partnership with parent-stakeholders and administered to parents of pediatric HTx recipients in September 2023. Closed and open-ended questions assessed (1) the patients' diagnosis, age at diagnosis, and age at transplant, (2) parents' daily concerns about their child's well-being, (3) parents' overall concerns about their child's well-being as they transition into adulthood, (4) parents' perceptions of their child's quality-of-life (QoL) and health, and (5) parents' demographic characteristics.</p><p><strong>Results: </strong>Eighty-six parents completed the survey. On a scale of 1 (worst) to 10 (best), 75% of parents rated their child's overall QoL at 8 or higher and 76% rated their child's health-related QoL at 8 or higher. Parents' daily concerns about their child's well-being included infectious diseases, health behaviors and care management, transplant-related concerns, socialization and education, mental health, and care coordination. Concerns related to the transition into adulthood included health behaviors and self-management, life satisfaction, finances, family, transplant-related concerns, and care coordination.</p><p><strong>Conclusions: </strong>Although parents of pediatric HTx recipients reported mostly positive QoL outcomes, they have concerns related to care management, life satisfaction, and healthcare access as their children transition into adulthood. Comprehensive transition-specific interventions and guidelines are needed to support families during this high-risk period.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70062"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143634465","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}
引用次数: 0
Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single-Center, Retrospective Study in Japan. 儿童肝移植后原发性硬化性胆管炎复发:日本单中心回顾性研究
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70078
Athaya Vorasittha, Seisuke Sakamoto, Yusuke Yanagi, Kazuki Degawa, Hirotaka Kato, Tasuku Kodama, Ryuji Komine, Masaki Yamada, Hajime Uchida, Akinari Fukuda, Chiduko Haga, Takako Yoshioka, Mureo Kasahara
{"title":"Recurrence of Primary Sclerosing Cholangitis After Pediatric Liver Transplantation: A Single-Center, Retrospective Study in Japan.","authors":"Athaya Vorasittha, Seisuke Sakamoto, Yusuke Yanagi, Kazuki Degawa, Hirotaka Kato, Tasuku Kodama, Ryuji Komine, Masaki Yamada, Hajime Uchida, Akinari Fukuda, Chiduko Haga, Takako Yoshioka, Mureo Kasahara","doi":"10.1111/petr.70078","DOIUrl":"https://doi.org/10.1111/petr.70078","url":null,"abstract":"<p><strong>Aim: </strong>Liver transplantation (LT) is a well-accepted treatment for primary sclerosing cholangitis (PSC) with generally good outcomes, although recurrent PSC (rPSC) poses significant challenges. This study aimed to describe patient characteristics and identify potential risk factors of rPSC in pediatric LT recipients.</p><p><strong>Methods: </strong>This retrospective study analyzed 13 pediatric patients who underwent LT for PSC at a single center. Patient characteristics, risk factors, and outcomes were compared between those with and without rPSC.</p><p><strong>Results: </strong>The median age at PSC diagnosis was 5.2 years and at LT, 15.4 years. Inflammatory bowel disease (IBD) was present in 12 patients (92.3%), and four (30.7%) had overlapping autoimmune hepatitis (AIH) before LT. Two patients received grafts from living-related donors, and 11 from deceased donors. During a median follow-up of 53 months, 4 of the 13 patients (30.7%) developed rPSC at a median of 48.9 months post-LT. Patients with rPSC tend to be younger at PSC diagnosis. All rPSC cases were associated with IBD, and half had AIH overlap, though the frequency difference was not significant. Acute cellular rejection (ACR) was universal in rPSC patients (100%) compared to nonrecurrent cases (33.3%, p = 0.07). One case of rPSC developed pulmonary hypertension following rPSC and succumbed to PH crisis, resulting in a 5-year patient survival rate of 82%.</p><p><strong>Conclusions: </strong>The recurrence rate was high in pediatric patients with PSC. The observed association with immune-activating conditions raises the possibility of utilizing immunologic interventions to prevent rPSC, although further prospective studies are warranted to clarify the underlying mechanisms.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70078"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144023292","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}
引用次数: 0
Short and Long-Term Outcomes of Liver Transplantation in Pediatric Patients With Inborn Errors of Metabolism: A Single-Center Study. 肝移植治疗先天性代谢异常患儿的短期和长期预后:一项单中心研究
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70067
Hazel Delal Dara Kar, Halil İbrahim Aydın, Figen Özçay, Oya Balcı Sezer, Esra Baskın, Feride Şahin, Yunus Kasım Terzi, Emre Karakaya, Mehmet Haberal
{"title":"Short and Long-Term Outcomes of Liver Transplantation in Pediatric Patients With Inborn Errors of Metabolism: A Single-Center Study.","authors":"Hazel Delal Dara Kar, Halil İbrahim Aydın, Figen Özçay, Oya Balcı Sezer, Esra Baskın, Feride Şahin, Yunus Kasım Terzi, Emre Karakaya, Mehmet Haberal","doi":"10.1111/petr.70067","DOIUrl":"10.1111/petr.70067","url":null,"abstract":"<p><strong>Background: </strong>Inborn errors of metabolism (IEMs) are inherited diseases causing significant morbidity and mortality, particularly in childhood. Liver transplantation (LT) can be curative or partially effective for these diseases. LT for IEMs has increased, making IEMs the second most common reason for pediatric LT after biliary atresia.</p><p><strong>Patients and methods: </strong>Between 2001 and 2023, 50 pediatric patients with IEMs underwent LT at Başkent University, Ankara Hospital. Data collected retrospectively included diagnosis, gender, age of diagnosis, age of LT, LT indication, donor data, graft type, rejection episodes, post-transplant complications, and clinical findings of the IEMs before and after LT. Treatment methods, follow-up duration, and survival time were also recorded.</p><p><strong>Results: </strong>Of the 332 pediatric LT patients, 50 (15.1%) had IEMs, with three requiring re-transplantations. Diagnoses included glycogen storage diseases (n = 11), tyrosinemia type 1 (n = 10), primary hyperoxaluria (n = 6), urea cycle disorders (n = 6), homozygous familial hypercholesterolemia (n = 4), propionic acidemia (n = 4), deoxyguanosine kinase deficiency (n = 3), maple syrup urine disease (n = 2), methylmalonic acidemia (n = 1), Niemann-Pick disease type B (n = 1), alkaptonuria with unknown neonatal cholestasis (n = 1), and bile acid synthesis disorder (n = 1). The parental consanguinity rate was 74%. Living-related donors provided organs for 48 (90.5%) patients. The mean age at LT was 75.3 ± 8.2 months (range: 5-218), with a follow-up period of 82.1 ± 10.2 months (range:1 day-229 months). Survival rates at 1, 5, 10, and 15 years were 83.7%, 81%, 81%, and 70.9%, respectively.</p><p><strong>Conclusion: </strong>LT is an effective solution for children with IEM causing chronic organ failure and difficult to manage with medical treatment, showing a good long-term prognosis.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70067"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143616766","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}
引用次数: 0
Native BK Viral Nephropathy After Pediatric Heart Transplantation Treated With Bilateral Native Nephrectomy and Living Donor Kidney Transplantation: A Case Report. 双侧自体肾切除和活体肾移植治疗小儿心脏移植后原发性BK病毒性肾病1例报告。
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.14863
Kann Rachel, Meshkin Dana, Crane Andrew, Salgado Claudia, Xu Qingyong, Ganoza Armando
{"title":"Native BK Viral Nephropathy After Pediatric Heart Transplantation Treated With Bilateral Native Nephrectomy and Living Donor Kidney Transplantation: A Case Report.","authors":"Kann Rachel, Meshkin Dana, Crane Andrew, Salgado Claudia, Xu Qingyong, Ganoza Armando","doi":"10.1111/petr.14863","DOIUrl":"10.1111/petr.14863","url":null,"abstract":"<p><strong>Background: </strong>BK Polyoma virus (BKV) can lead to significant renal complications in immunocompromised individuals. While commonly observed in kidney transplant recipients, its occurrence in non-renal solid organ transplant (NRSOT) recipients remains rare. The mainstay of treatment for BKV nephropathy in these patients involves careful reduction of immunosuppression.</p><p><strong>Summary: </strong>In this report, we present a unique case of end-stage renal disease due to refractory BKV nephropathy in a pediatric heart transplant patient. The patient was treated with bilateral native nephrectomy to eliminate the viral reservoir with clearance of her BK viremia. This led to a six-month period of viral clearance, allowing for subsequent living donor kidney transplantation (LDKT).</p><p><strong>Conclusion: </strong>BKV nephropathy is a rare entity in NRSOT patients. This case highlights the successful management of refractory BKV nephropathy in a pediatric heart transplant recipient through bilateral native nephrectomy, leading to an extended period of viral clearance and subsequent LDKT. Further studies are needed to explore the broader applicability of this approach in NRSOT recipients.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e14863"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11926412/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143670600","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}
引用次数: 0
Precision Dosing, Therapeutic Drug Monitoring, and Clinical Pharmacokinetics in Pediatric Kidney Transplant Patients: Principles and Practice With Emphasis on Low- and Middle-Income Countries. 儿科肾移植患者的精确给药、治疗药物监测和临床药代动力学:以中低收入国家为重点的原则和实践。
IF 1.2 4区 医学
Pediatric Transplantation Pub Date : 2025-05-01 DOI: 10.1111/petr.70074
Paulo Caceres Guido, Guillermo Federico Taboada, Marta Lidia Monteverde
{"title":"Precision Dosing, Therapeutic Drug Monitoring, and Clinical Pharmacokinetics in Pediatric Kidney Transplant Patients: Principles and Practice With Emphasis on Low- and Middle-Income Countries.","authors":"Paulo Caceres Guido, Guillermo Federico Taboada, Marta Lidia Monteverde","doi":"10.1111/petr.70074","DOIUrl":"10.1111/petr.70074","url":null,"abstract":"<p><strong>Background: </strong>Pediatric kidney transplantation requires complex multidisciplinary coordination. The contributions of pharmacotherapeutic aspects to this practice have been of fundamental importance, even in low- and middle-income countries (LMIC).</p><p><strong>Methods: </strong>We conducted a quasi-systematic review of the PubMed and Google Scholar databases from inception to July 2024 using Medical Subject Headings and keywords relevant to Therapeutic Drug Monitoring (TDM) and Model-Based Precision Dosing (MIPD). The quality of the articles and data collected were appraised using the appropriate critical appraisal tools and was synthesized qualitatively.</p><p><strong>Results: </strong>TDM and the analyses and interpretations associated with pharmacometric aspects, specifically clinical pharmacokinetics, have led to the use of modern strategies such as MIPD. These strategies allow for individually adjusted drug dosages to be optimized, making them more effective and safer for many immunosuppressants, antibiotics, antivirals, antifungals, antiepileptics, antineoplastics, and antiarrhythmics, among others. Several points of interest associated with improving the implementation and practice of TDM-MIPD, particularly challenging in LMICs, include the availability and adequate management of economic resources (such as software and laboratory supplies), the development of collaborative work with other institutions (including foreign ones), the possibility of consolidating independent management not depending on other clinical services, the need to train and maintain highly skilled professional staff for clinical and research purposes, and the establishment and maintenance of specialized educational programs.</p><p><strong>Conclusion: </strong>Throughout the world, but especially in LMICs, there is a need to intensify strategies that allow for the more widespread application of TDM-MIPD to improve pharmacotherapeutic care for this highly vulnerable patient population.</p>","PeriodicalId":20038,"journal":{"name":"Pediatric Transplantation","volume":"29 3","pages":"e70074"},"PeriodicalIF":1.2,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143772861","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}
引用次数: 0
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