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INTRODUCTION: Acquired amyloid neuropathy is an iatrogenic disease that appears years after a domino liver transplant. The objectives of our study are to analyze the efficacy and tolerability of tafamidis for the treatment of acquired amyloid neuropathy in domino liver transplant recipients. This post-authorization, prospective, longitudinal study included seven domino liver transplant recipients with acquired amyloid neuropathy who received treatment with tafamidis for 18 months. METHODS: The primary endpoints were the response rate, defined as those patients with an increase of < 2 points on the Neurological Impairment Score (NIS) from baseline, and the change in the NIS score from baseline. Secondary endpoints included the Quantitative Sensory Test, 10-m walk test, quality of life (Norfolk), and disability (Rasch-built Overall Disability Scale). As safety parameters, the evidence of graft rejection, changes in immunosuppressive trough levels and changes in antiviral and allogeneic cellular immunity before and 12 months after tafamidis treatment were also assessed. RESULTS: Six patients (85.7%) had responded at 18-months. Compared to baseline, we observed non-statistically significant improvement in mean NIS score at 6 months (- 2.54 points, CI - 5.92 to 0.84), 12 months (- 3.25 points; CI - 6.63 to 0.13), and 18 months (- 2.35 points; CI - 5.74 to 1.02). Changes in the Quantitative Sensory Test, 10-m walk tests and the quality of life and disability questionnaires were not statistically significant. The use of tafamidis did not induce relevant side effects or drug interactions. Also, no acute rejections events nor changes in functional adaptive immunity were observed. CONCLUSION: Our study supports the safety and tolerability of tafamidis for the treatment of acquired amyloid neuropathy in domino liver transplant recipients. Tafamidis shows promise as a useful treatment in the clinical management of these patients. Future randomized placebo-controlled clinical trials with longer follow-up durations are needed.
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BACKGROUND: Appropriate risk stratification for the difficulty of liver transplantation (LT) is essential to guide the selection and acceptance of grafts and avoid morbidity and mortality. METHODS: Based on 987 LTs collected from 5 centers, perioperative outcomes were analyzed across the 3 difficulty levels. Each LT was retrospectively scored from 0 to 10. Scores of 0-2, 3-5 and 6-10 were then translated into respective difficulty levels: low, moderate and high. Complications were reported according to the comprehensive complication index (CCI). RESULTS: The difficulty level of LT in 524 (53%), 323 (32%), and 140 (14%) patients was classified as low, moderate and high, respectively. The values of major intraoperative outcomes, such as cold ischemia time (p = 0.04) and operative time (p < 0.0001) increased gradually with statistically significant values among difficulty levels. There was a corresponding increase in CCI (p = 0.04), severe complication rates (p = 0.05) and length of ICU (p = 0.01) and hospital (p = 0.004) stays across the different difficulty levels. CONCLUSION: The LT difficulty classification has been validated.
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Transplante de Fígado , Complicações Pós-Operatórias , Humanos , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos , Feminino , Pessoa de Meia-Idade , Masculino , Medição de Risco , Complicações Pós-Operatórias/classificação , Complicações Pós-Operatórias/etiologia , Fatores de Risco , Resultado do Tratamento , Adulto , Reprodutibilidade dos Testes , Idoso , Fatores de Tempo , Tempo de Internação , Europa (Continente) , Duração da Cirurgia , Isquemia Fria , Seleção de Pacientes , Valor Preditivo dos TestesRESUMO
BACKGROUND: Iatrogenic bile duct injury (BDI) during cholecystectomy is associated with a complex and heterogeneous management owing to the burden of morbidity until their definitive treatment. This study aimed to define the textbook outcomes (TOs) after BDI with the purpose to indicate the ideal treatment and to improve it management. METHODS: We collected data from patients with an BDI between 1990 and 2022 from 27 hospitals. TO was defined as a successful conservative treatment of the iatrogenic BDI or only minor complications after BDI or patients in whom the first repair resolves the iatrogenic BDI without complications or with minor complications. RESULTS: We included 808 patients and a total of 394 patients (46.9%) achieved TO. Overall complications in TO and non-TO groups were 11.9% and 86%, respectively (P < .001). Major complications and mortality in the non-TO group were 57.4% and 9.2%, respectively. The use of end-to-end bile duct anastomosis repair was higher in the non-TO group (23.1 vs 7.8, P < .001). Factors associated with achieving a TO were injury in a specialized center (adjusted odds ratio [aOR], 4.01; 95% CI, 2.68-5.99; P < .001), transfer for a first repair (aOR, 5.72; 95% CI, 3.51-9.34; P < .001), conservative management (aOR, 5.00; 95% CI, 1.63-15.36; P = .005), or surgical management (aOR, 2.45; 95% CI, 1.50-4.00; P < .001). CONCLUSION: TO largely depends on where the BDI is managed and the type of injury. It allows hepatobiliary centers to identify domains of improvement of perioperative management of patients with BDI.
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Ductos Biliares , Doença Iatrogênica , Complicações Intraoperatórias , Humanos , Masculino , Feminino , Ductos Biliares/lesões , Ductos Biliares/cirurgia , Pessoa de Meia-Idade , Complicações Intraoperatórias/etiologia , Idoso , Estudos Retrospectivos , Colecistectomia/efeitos adversos , Adulto , Anastomose Cirúrgica , Colecistectomia Laparoscópica/efeitos adversos , Resultado do Tratamento , Complicações Pós-Operatórias/etiologia , Complicações Pós-Operatórias/epidemiologia , Tratamento ConservadorRESUMO
Introducción: El trasplante hepático (TH) split es un procedimiento extendido por toda Europa. En 2018 en Cataluña, se redefinió la distribución de donantes, siendo candidatos potenciales para split todos aquellos menores de 35 años y se flexibilizó la selección del adulto para el injerto derecho. El objetivo del estudio es evaluar el efecto de estas modificaciones en la utilización de donantes para split, en las listas de espera (LE) y en los resultados de los adultos que recibieron un injerto split. Métodos: Estudio observacional y retrospectivo; dos periodos de recogida de datos «pre» (2013-2017) y «post» (2018-2021). Los resultados de los receptores adultos se analizaron mediante un propensity score matching. Resultados: En el primer periodo fueron registrados tres donantes y se trasplantaron tres pacientes pediátricos y dos adultos; en el periodo post se obtuvieron 24 donantes, realizándose el trasplante en 19 adultos y 24 receptores infantiles. Al comparar las LE se evidenció una disminución significativa tanto en la de adultos (p = 0,0001) como en la infantil (p = 0,0004) y hasta en tres ocasiones no hubo receptores en la LE infantil. No se observaron diferencias significativas en cuanto a morbilidad o mortalidad, ni en la supervivencia global en el grupo de receptores adultos de injertos split. Conclusiones: La flexibilidad en la selección del receptor adulto y la nueva distribución de donantes ayuda a aumentar la tasa de bipartición, permitiendo reducir la LE pediátrica sin afectar los resultados en los trasplantados adultos ni su estancia en LE.(AU)
Introduction: SPLIT liver transplantation is a procedure performed throughout Europe. In 2018 in Catalonia, the distribution of donors was redefined, being potential candidates for SPLIT all those under 35 years and it was made flexible the adult selection for the right graft. The study aim is to evaluate the effect of this modification on the use of SPLIT donors on the adult/pediatric waiting lists, as well as to evaluate the post-transplant results of adults who received a SPLIT donor. Methods: Observational and retrospective study; 2 data collection periods «PRE» (20132017) and «POST» (20182021). The adults recipients results were analyzed by a propensity score matching. Results: In the first period, three donors were registered and three pediatric patients and two adults received a transplant. In the POST period, 24 donations with liver bipartition were made, performing the transplant in 19 adults and 24 children. When comparing the adults waiting lists, a significant decrease was evidenced, both for adults (p = 0.0001) and on the children's waiting list (p = 0.0004), and up to three times there were no recipients on the pediatric waiting list. No significant differences between hospital morbidity or mortality or overall survival were observed in the group of adult recipients of SPLIT grafts. Conclusions: The flexibility in the selection of the adult recipient and the new distribution of donors makes possible to increase the bipartition rate, reducing the pediatric waiting list without worsening the adults results transplant recipients or their permanence on the waiting list.(AU)
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Humanos , Masculino , Feminino , Cirurgia Geral , Transplante de Fígado/métodos , Doadores de Tecidos/estatística & dados numéricos , Estudos Retrospectivos , EspanhaRESUMO
La Sociedad Española de Trasplante Hepático tiene como objetivo la promoción y elaboración de documentos de consenso sobre temas de actualidad en trasplante hepático de abordaje multidisciplinario. Para ello, en noviembre de 2022 se celebró la 10.ª Reunión de Consenso, con la participación de representantes de los 26 programas de trasplante hepático españoles autorizados. En esta edición se abordó la recuperación intensificada tras el trasplante hepático y se dividieron las acciones en 3periodos, preoperatorio, intraoperatorio y postoperatorio. Se exponen a continuación las evidencias evaluadas y las consecuentes recomendaciones consensuadas.(AU)
The goal of the Spanish Society for Liver Transplantation (Sociedad Española de Trasplante Hepático) is to promote and create consensus documents about current topics in liver transplantation with a multidisciplinary approach. To this end, in November 2022, the 10th Consensus Document Meeting was held, with the participation of experts from the 26 authorized Spanish liver transplantation programs. This edition discusses enhanced recovery after liver transplantation, dividing needed actions into 3periods: preoperative, intraoperative and postoperative. The evaluated evidence and the consensus conclusions for each of these topics are described.(AU)
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Humanos , Masculino , Feminino , Transplante de Fígado , Alta do Paciente , Reabilitação , Consenso , EspanhaRESUMO
Resumen La Sociedad Española de Trasplante Hepático tiene como objetivo la promoción y elaboración de documentos de consenso sobre temas de actualidad en trasplante hepático de abordaje multidisciplinario. Para ello, en noviembre de 2022 se celebró la 10.ª Reunión de Consenso, con la participación de representantes de los 26 programas de trasplante hepático españoles autorizados. En esta edición se abordó la recuperación intensificada tras el trasplante hepático y se dividieron las acciones en 3 periodos, preoperatorio, intraoperatorio y postoperatorio. Se exponen a continuación las evidencias evaluadas y las consecuentes recomendaciones consensuadas. Abstract The goal of the Spanish Society for Liver Transplantation (Sociedad Española de Trasplante Hepático) is to promote and create consensus documents about current topics in liver transplantation with a multidisciplinary approach. To this end, in November 2022, the 10th Consensus Document Meeting was held, with the participation of experts from the 26 authorized Spanish liver transplantation programs. This edition discusses enhanced recovery after liver transplantation, dividing needed actions into 3 periods: preoperative, intraoperative and postoperative. The evaluated evidence and the consensus conclusions for each of these topics are described (AU)
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Humanos , Sociedades Médicas , Transplante de Fígado , Listas de Espera , Consenso , EspanhaRESUMO
BACKGROUND AND AIMS: Management of Budd-Chiari syndrome (BCS) has improved over the last decades. The main aim was to evaluate the contemporary post-liver transplant (post-LT) outcomes in Europe. APPROACH AND RESULTS: Data from all patients who underwent transplantation from 1976 to 2020 was obtained from the European Liver Transplant Registry (ELTR). Patients < 16 years, with secondary BCS or HCC were excluded. Patient survival (PS) and graft survival (GS) before and after 2000 were compared. Multivariate Cox regression analysis identified predictors of PS and GS after 2000. Supplemental data was requested from all ELTR-affiliated centers and received from 44. In all, 808 patients underwent transplantation between 2000 and 2020. One-, 5- and 10-year PS was 84%, 77%, and 68%, and GS was 79%, 70%, and 62%, respectively. Both significantly improved compared to outcomes before 2000 ( p < 0.001). Median follow-up was 50 months and retransplantation rate was 12%. Recipient age (aHR:1.04,95%CI:1.02-1.06) and MELD score (aHR:1.04,95%CI:1.01-1.06), especially above 30, were associated with worse PS, while male sex had better outcomes (aHR:0.63,95%CI:0.41-0.96). Donor age was associated with worse PS (aHR:1.01,95%CI:1.00-1.03) and GS (aHR:1.02,95%CI:1.01-1.03). In 353 patients (44%) with supplemental data, 33% had myeloproliferative neoplasm, 20% underwent TIPS pre-LT, and 85% used anticoagulation post-LT. Post-LT anticoagulation was associated with improved PS (aHR:0.29,95%CI:0.16-0.54) and GS (aHR:0.48,95%CI:0.29-0.81). Hepatic artery thrombosis and portal vein thrombosis (PVT) occurred in 9% and 7%, while recurrent BCS was rare (3%). CONCLUSIONS: LT for BCS results in excellent patient- and graft-survival. Older recipient or donor age and higher MELD are associated with poorer outcomes, while long-term anticoagulation improves both patient and graft outcomes.
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Síndrome de Budd-Chiari , Sobrevivência de Enxerto , Transplante de Fígado , Sistema de Registros , Humanos , Síndrome de Budd-Chiari/cirurgia , Transplante de Fígado/estatística & dados numéricos , Masculino , Sistema de Registros/estatística & dados numéricos , Feminino , Europa (Continente)/epidemiologia , Adulto , Pessoa de Meia-Idade , Resultado do Tratamento , Adulto Jovem , Adolescente , Estudos RetrospectivosRESUMO
The goal of the Spanish Society for Liver Transplantation (La Sociedad Española de Trasplante Hepático) is to promote and create consensus documents about current topics in liver transplantation with a multidisciplinary approach. To this end, in November 2022, the 10th Consensus Document Meeting was held, with the participation of experts from the 26 authorized Spanish liver transplantation programs. This edition discusses Enhanced Recovery After Liver Transplantation, dividing needed actions into three periods: preoperative, intraoperative and postoperative. The evaluated evidence and the consensus conclusions for each of these topics are described.
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Neoplasias Hepáticas , Transplante de Fígado , Humanos , Consenso , Neoplasias Hepáticas/cirurgiaRESUMO
The goal of the Spanish Society for Liver Transplantation (Sociedad Española de Trasplante Hepático) is to promote and create consensus documents about current topics in liver transplantation with a multidisciplinary approach. To this end, in November 2022, the 10th Consensus Document Meeting was held, with the participation of experts from the 26 authorized Spanish liver transplantation programs. This edition discusses enhanced recovery after liver transplantation, dividing needed actions into 3periods: preoperative, intraoperative and postoperative. The evaluated evidence and the consensus conclusions for each of these topics are described.
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Neoplasias Hepáticas , Transplante de Fígado , Humanos , Consenso , Neoplasias Hepáticas/cirurgiaRESUMO
INTRODUCTION: Surgery is the only potentially curative treatment for colorectal cancer liver metastases (CRLM) and its indication and results have varied in the last 30 years. METHODS: All patients operated on for CRLM in our centre from 1990 to 2021 were prospectively collected, establishing 3 subgroups based on the year of the first surgery: group A 1990-1999, group B 2000-2010, group C 2011-2021. Clinical characteristics and the results of survival, recurrence and prognostic factors were compared. RESULTS: 1736 hepatectomies were included (Group A n = 208; Group B n = 770; Group C n = 758). Patients in group C had better survival at 5 and 10 years (A 40.5%/28.2%; B 45.9%/32.2%; C 51.6%/33.1%, p = 0.013), although there were no differences between groups in overall recurrence at 5 and 10 years (A 73%/75.7%; B 67.6%/69.2%, and C 63.9%/66%, p = 0.524), nor in liver recurrence (A 46.4%/48.2%; B 45.8%/48.2%; and C 44.4%/48.4%, p = 0.899). An improvement was observed in median survival after recurrence, being 19 months, 23 months, and 31 months (groups A, B and C respectively). Prognostic factors of long-term survival changed over the 3 study periods. The only ones that remained relevant in the last decade were the presence of >4 liver metastasis, extrahepatic disease at the time of hepatectomy, and intraoperative blood transfusion. CONCLUSIONS: Survival after surgery for CRLM has improved significantly, although this cannot be explained by a reduction in overall and hepatic recurrence, but rather by an improvement in post-recurrence survival. Involvement of the resection margin has lost prognostic value in the last decade.
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Neoplasias Colorretais , Hepatectomia , Neoplasias Hepáticas , Humanos , Neoplasias Hepáticas/secundário , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/mortalidade , Hepatectomia/métodos , Neoplasias Colorretais/patologia , Neoplasias Colorretais/cirurgia , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Resultado do Tratamento , Recidiva Local de Neoplasia/epidemiologia , Recidiva Local de Neoplasia/cirurgia , Fatores de Tempo , Estudos Prospectivos , Taxa de Sobrevida , Prognóstico , Adulto , Idoso de 80 Anos ou maisRESUMO
Introducción: El tratamiento de los quistes hepáticos requiere del diagnóstico diferencial de quiste simple hepático (QSH) de la neoplasia mucinosa quística (NMQ) hepática. Las características radiológicas no son patognomónicas. Algunos estudios han sugerido la utilidad de los marcadores tumorales (MKT) intraquísticos. Métodos: Análisis retrospectivo de base de datos prospectiva incluyendo pacientes diagnosticados de QSH sintomático desde el 2003 hasta el 2021. El objetivo del estudio es evaluar los resultados del tratamiento de los QSH sintomáticos y analizar la utilidad de la determinación de «carcinoembryonic antigen» (CEA) y «carbohydrate antigen» CA 19.9 intraquísticos. Resultados: Se incluyeron 50 pacientes tratados por quiste sintomático. En 15 pacientes el primer tratamiento fue el drenaje percutáneo. En 35 pacientes se realizó fenestración laparoscópica. Cuatro pacientes se diagnosticaron de lesiones premalignas/malignas (NMQ, NPIB, linfoma B); tres de ellos requirieron una segunda cirugía tras la fenestración y el diagnóstico anatomopatológico. La mediana de los valores de CEA y CA- 19.9 fue de 196μg/L y 227.321U/mL respectivamente. Los pacientes con lesiones premalignas no tuvieron valores elevados de MKT. El valor predictivo positivo fue del 0% en ambos MKT, y el valor predictivo negativo fue de 89% y 91% respectivamente. Conclusiones: Los valores de CEA y CA 19.9 intraquísticos no permiten distinguir los QSH de las NMH. El tratamiento más resolutivo de los QSH sintomáticos es la fenestración quirúrgica. El análisis anatomopatológico de la pared del quiste posibilita su correcto diagnóstico, permitiendo indicar una reintervención quirúrgica en los casos de NMQ.(AU)
Introduction: To decide treatment of hepatic cysts diagnosis between simple hepatic cyst (SHC) and cystic mucinous neoplasm (CMN). Radiological features are not pathognomonic. Some studies have suggested the utility of intracystic tumor markers. Methods: Retrospective analysis of our prospective database including patients treated due to symptomatic SHC from 2003 to 2021. The aim of the study was to evaluate the results of treatment of symptomatic SHC and the usefulness of the determination of intracystic carcinoembryonic antigen (CEA) and carbohydrate antigen CA 19.9. Results: Fifty patients diagnosed and treated for symptomatic SHC were included. In 15 patients the first treatment was percutaneous drainage. In 35 patients the first treatment was laparoscopic fenestration. Four patients were diagnosed of premalignant or malignant liver cystic lesions (MCN, IPMN, and lymphoma B); three of them required surgery after initial fenestration and pathological diagnosis. Median CEA and CA 19.9 were 196μg/L and 227.321U/mL, respectively. Patients with malignant or premalignant pathology did not have higher levels of intracystic tumor markers. Positive predictive value was 0% for both markers, and negative predictive value was 89% and 91%, respectively. Conclusion: Values of intracystic tumor markers CEA and CA 19.9 do not allow distinguishing simple cysts from cystic liver neoplasms. The most effective treatment for symptomatic simple liver cysts is surgical fenestration. The pathological analysis of the wall of the cysts enables the correct diagnosis, allowing to indicate a surgical reintervention in cases of hepatic cyst neoplasia.(AU)
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Humanos , Masculino , Feminino , Diagnóstico Diferencial , Cistos/cirurgia , Fígado/lesões , Terapêutica , Neoplasias Hepáticas , Biomarcadores TumoraisRESUMO
Machine perfusion of solid human organs is an old technique, and the basic principles were presented as early as 1855 by Claude Barnard. More than 50 years ago, the first perfusion system was used in clinical kidney transplantation. Despite the well-known benefits of dynamic organ preservation and significant medical and technical development in the last decades, perfusion devices are still not in routine use. This article describes the various challenges to implement this technology in practice, critically analyzing the role of all involved stakeholders, including clinicians, hospitals, regulatory, and industry, on the background of regional differences worldwide. The clinical need for this technology is discussed first, followed by the current status of research and the impact of costs and regulations. Considering the need for strong collaborations between clinical users, regulatory bodies, and industry, integrated road maps and pathways required to achieve a wider implementation are presented. The role of research development, clear regulatory pathways, and the need for more flexible reimbursement schemes is discussed together with potential solutions to address the most relevant hurdles. This article paints an overall picture of the current liver perfusion landscape and highlights the role of clinical, regulatory, and financial stakeholders worldwide.
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Transplante de Rim , Transplante de Fígado , Humanos , Preservação de Órgãos/métodos , Perfusão/métodosRESUMO
INTRODUCTION: To decide treatment of hepatic cysts diagnosis between simple hepatic cyst (SHC) and cystic mucinous neoplasm (CMN). Radiological features are not patognomonic. Some studies have suggested the utility of intracystic tumor markers. METHODS: Retrospective analysis of our prospective database including patients treated due to symptomatic SHC from 2003 to 2021. The aim of the study was to evaluate the results of treatment of symptomatic SHC and the usefulness of the determination of intracystic "carcinoembryonic antigen" (CEA) and "carbohydrate antigen" CA 19.9. RESULTS: 50 patients diagnosed and treated for symptomatic SHC were included. In 15 patients the first treatment was percutaneous drainage. In 35 patients the first treatment was laparoscopic fenestration. Four patients were diagnosed of premalignant or malignant liver cystic lesions (MCN, IPMN, lymphoma B); three of them required surgery after initial fenestration and pathological diagnosis. Median CEA and CA 19-9 were 196 µg/L and 227.321 U/mL respectively. Patients with malignant or premalignant pathology did not have higher levels of intracystic tumor markers. Positive predictive value was 0% for both markers, and negative predictive value was 89% and 91% respectively. CONCLUSION: Values of intracystic tumor markers CEA and CA 19-9 do not allow distinguishing simple cysts from cystic liver neoplasms. The most effective treatment for symptomatic simple liver cysts is surgical fenestration. The pathological analysis of the wall of the cysts enables the correct diagnosis, allowing to indicate a surgical reintervention in cases of hepatic cyst neoplasia.
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Cistos , Hepatopatias , Neoplasias Hepáticas , Humanos , Antígeno Carcinoembrionário/análise , Biomarcadores Tumorais , Estudos Retrospectivos , Cistos/diagnóstico , Cistos/cirurgia , Antígeno CA-19-9/análise , Neoplasias Hepáticas/diagnóstico , Neoplasias Hepáticas/cirurgiaRESUMO
INTRODUCTION: Split liver transplantation is a procedure performed throughout Europe. In 2018 in Catalonia, the distribution of donors was redefined, being potential candidates for SPLIT all those under 35-years and it was made flexible the adult selection for the right graft. The study aim is to evaluate the effect of this modifications on the use of Split donors on the adult/pediatric waiting lists, as well as to evaluate the post-transplant results of adults who received a Split donor. METHODS: Observational and retrospective study; 2 data collection periods "PRE" (2013-2017) and "POST" (2018-2021). The adults recipients results were analyzed by a propensity score matching. RESULTS: In the first period 3 donors were registered and 3 pediatric patients and 2 adults recieved a transplant. In the POST period, 24 donations with liver bipartition were made, performing the transplant in 19 adults and 24 childrens. When comparing the adults waiting lists, a significant decrease was evidenced, both for adults (p = 0,0001) and on the children's waiting list (p = 0,0004), and up to 3 times there were no recipients on the pediatric waiting list. No significant differences between hospital morbidity or mortality or overall survival were observed in the group of adult recipients of Split grafts. CONCLUSIONS: The flexibility in the selection of the adult recipient and the new distribution of donors makes possible to increase the bipartition rate, reducing the pediatric waiting list without worsening the adults results transplant recipients or their permanence on the waiting list.
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Transplante de Fígado , Adulto , Criança , Humanos , Transplante de Fígado/métodos , Estudos Retrospectivos , Fígado , Doadores de Tecidos , Europa (Continente)RESUMO
A 74-year-old female was admitted for painless jaundice. Laboratory tests showed hyperbilirubinemia, cholestasis, normal coagulation, and Ca19-9:163U/L. The CT-scan reported dilation of the intrahepatic and extrahepatic bile ducts secondary to a 24mm tumor in the intrapancreatic common bile duct. The magnetic cholangioresonance showed multiple endoluminal polypoid lesions, suggestive of intraductal papillary neoplasm of the bile duct (IPNB). The endoscopic bile duct brushing was non-conclusive.(AU)
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Humanos , Feminino , Idoso , Ducto Colédoco/cirurgia , Síndromes Paraneoplásicas , Colangiocarcinoma/cirurgia , Hemofilia A , Neoplasias dos Ductos Biliares/diagnóstico por imagem , Tumor de Klatskin , Pacientes Internados , Exame Físico , Doenças do Sistema Digestório , Gastroenteropatias , Neoplasias dos Ductos Biliares/complicações , Neoplasias dos Ductos Biliares/patologiaRESUMO
Orthotopic liver transplantation (OLT) was the first treatment able to modify the natural course of hereditary transthyretin (ATTRv) amyloidosis, which is a rare and fatal disorder caused by the accumulation of misfolded transthyretin (TTR) variants in different organs and tissues and which leads to a progressive and multisystem dysfunction. Because the liver is the main source of TTR, OLT dramatically reduces the production of the pathogenic TTR variant, which should prevent amyloid formation and halt disease progression. However, amyloidosis progression may occur after OLT due to wild-type TTR deposition, especially in the nerves and heart. In this review, we discuss the disease features influencing OLT outcomes and the clinical manifestations of ATTRv amyloidosis progression post-OLT to improve our understanding of disease worsening after OLT and optimize the follow-up and clinical management of these patients. By conducting a literature review on the PubMed database, we identified patient characteristics that have been associated with worse post-OLT outcomes, including late-onset V50M and non-V50M variants, age >40 years, long disease duration, advanced neuropathy and autonomic dysfunction, and malnutrition. Regarding post-OLT mortality, deaths occurring within the first year after OLT were mainly associated with fatal graft complications and infectious diseases, whereas cardiovascular-related deaths usually occurred later. Considering the diverse clinical manifestations of ATTRv amyloidosis progression post-OLT, including worsening neuropathy and/or cardiomyopathy, autonomic dysfunction, and oculoleptomeningeal involvement, we present advice on the most relevant tests for assessing disease progression post-OLT. Finally, we discuss the use of new therapies based on TTR stabilizers and TTR mRNA silencers for the treatment of ATTRv amyloidosis patients post-OLT.
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Data comparing long-term effectiveness and safety of once-daily tacrolimus formulations in de novo liver transplantation are scarce. We compared the effectiveness, pharmacokinetic profile, and safety of LCPT (Envarsus) and PR-Tac (Advagraf) for up to 12 months post-transplant. Adult de novo liver transplant recipients who started IR-Tac (Prograf) and were converted to LCPT or PR-Tac 3-5 days post-transplant were included. Data from 163 patients were analyzed, 87 treated with LCPT and 76 with PR-Tac. The incidence of treatment failure was 30.5% in the LCPT group versus 23.0% in the PR-Tac group (p = .291). Biopsy-proven acute rejection (BPAR) was reported in 26.8% of patients in the LCPT group and 17.6% in the PR-Tac group (p = .166). Graft loss was experienced in one patient (1.2%) in the LCPT group and three patients (4.1%) in the PR-Tac group (p = .346). Death was registered in three patients (3.7%) in the LCPT group and three patients (4.1%) in the PR-Tac group (p > .999). Patients in the LCPT group showed 45.7% higher relative bioavailability (Cmin /total daily dose [TDD]; p < .01) with similar Cmin and 33.3% lower TDD versus PR-Tac (p < .01). The evolution of renal function, safety profile, and the incidence of post-transplant renal failure, dyslipidemia, obesity, hypertension, and diabetes mellitus were similar in patients treated with LCPT and PR-Tac. In de novo liver transplant patients, LCPT and PR-Tac showed comparable effectiveness with higher relative bioavailability, similar Cmin and lower TDD in the LCPT group. Renal function, safety, and post-transplant complications were comparable in LCPT and PR-Tac groups.
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Transplante de Rim , Transplante de Fígado , Adulto , Humanos , Tacrolimo/uso terapêutico , Tacrolimo/farmacocinética , Imunossupressores/uso terapêutico , Imunossupressores/farmacocinética , Transplante de Rim/efeitos adversos , Esquema de Medicação , Estudos Prospectivos , Rejeição de Enxerto/tratamento farmacológico , Rejeição de Enxerto/etiologia , TransplantadosRESUMO
COVID-19-associated pulmonary aspergillosis (CAPA) has emerged as a frequent complication in the intensive care unit (ICU). However, little is known about this life-threatening fungal superinfection in solid organ transplant recipients (SOTRs), including whether targeted anti-mold prophylaxis might be justified in this immunosuppressed population. We performed a multicentric observational retrospective study of all consecutive ICU-admitted COVID-19 SOTRs between August 1, 2020 and December 31, 2021. SOTRs receiving antifungal prophylaxis with nebulized amphotericin-B were compared with those without prophylaxis. CAPA was defined according the ECMM/ISHAM criteria. Sixty-four SOTRs were admitted to ICU for COVID-19 during the study period. One patient received antifungal prophylaxis with isavuconazole and was excluded from the analysis. Of the remaining 63 SOTRs, nineteen (30.2%) received anti-mold prophylaxis with nebulized amphotericin-B. Ten SOTRs who did not receive prophylaxis developed pulmonary mold infections (nine CAPA and one mucormycosis) compared with one who received nebulized amphotericin-B (22.7% vs 5.3%; risk ratio 0.23; 95%CI 0.032-1.68), but with no differences in survival. No severe adverse events related to nebulized amphotericin-B were recorded. SOTRs admitted to ICU with COVID-19 are at high risk for CAPA. However, nebulized amphotericin-B is safe and might reduce the incidence of CAPA in this high-risk population. A randomized clinical trial to confirm these findings is warranted.