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1.
Am J Transplant ; 23(10): 1612-1621, 2023 10.
Artigo em Inglês | MEDLINE | ID: mdl-37419452

RESUMO

We report initial results of a liver paired exchange (LPE) program established at the Liver Transplant Institute at Inonu University through collaboration with design economists. Since June 2022, the program has been using a matching procedure that maximizes the number of living donor liver transplants (LDLTs) to the patients in the pool subject to the ethical framework and the logistical constraints of the program. In 1 4-way and 4 2-way exchanges, 12 LDLTs have been performed via LPE in 2022. The 4-way exchange, generated in the same match run with a 2-way exchange, is a first worldwide. This match run generated LDLTs for 6 patients, revealing the value of the capacity to carry out larger than 2-way exchanges. With only 2-way exchanges, only 4 of these patients would receive a LDLT. The number of LDLTs from LPE can be increased by developing the capacity to perform larger than 2-way exchanges in either high-volume centers or multicenter programs.


Assuntos
Transplante de Rim , Transplante de Fígado , Humanos , Transplante de Fígado/métodos , Doadores Vivos , Transplante de Rim/métodos , Fígado , Pessoal de Saúde
2.
Hepatology ; 75(6): 1579-1589, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-34859474

RESUMO

Interest in anonymous nondirected living organ donation is increasing in the United States and a small number of transplantation centers are accumulating an experience regarding nondirected donation in living donor liver transplantation. Herein, we review current transplant policy, discuss emerging data, draw parallels from nondirected kidney donation, and examine relevant considerations in nondirected living liver donation. We aim to provide a consensus guidance to ensure safe evaluation and selection of nondirected living liver donors and a schema for just allocation of nondirected grafts.


Assuntos
Transplante de Rim , Transplante de Fígado , Obtenção de Tecidos e Órgãos , Humanos , Rim , Doadores Vivos , Estados Unidos
3.
Clin Transplant ; 37(7): e14924, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36733213

RESUMO

In the United States, living donor liver transplantation (LDLT) is limited to transplant centers with specific experience. However, the impact of recipient characteristics on procedure selection (LDLT vs. deceased donor liver transplant [DDLT]) within these centers has not been described. Transplant registry data for centers that performed ≥1 LDLT in 2002-2019 were analyzed using hierarchal regression modeling to quantify the impact of patient and center factors on the adjusted odds ratio (aOR) of LDLT (vs DDLT). Among 73,681 adult recipients, only 4% underwent LDLT, varying from <1% to >60% of total liver transplants. After risk adjustment, the likelihood of receiving an LDLT rose by 73% in recent years (aOR 1.73 for 2014-2019 vs. 2002-2007) but remained lower for older adults, men, racial and ethnic minorities, and obese patients. LDLT was less commonly used in patients with hepatocellular carcinoma or alcoholic cirrhosis, and more frequently in those with hepatitis C and with lower severity of illness (Model for End-Stage Liver Disease (MELD) score < 15). Patients with public insurance, lower educational achievement, and residence in the Northwest and Southeast had decreased access. While some differences in access to LDLT reflect clinical factors, further exploration into disparities in LDLT utilization based on center practice and socioeconomic determinants of health is needed.


Assuntos
Doença Hepática Terminal , Neoplasias Hepáticas , Transplante de Fígado , Masculino , Humanos , Estados Unidos , Idoso , Doadores Vivos , Transplante de Fígado/métodos , Doença Hepática Terminal/cirurgia , Estudos Retrospectivos , Índice de Gravidade de Doença , Neoplasias Hepáticas/patologia , Resultado do Tratamento
4.
Clin Transplant ; 37(7): e14953, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36890717

RESUMO

INTRODUCTION: Living donor liver transplantation (LDLT) reduces liver transplant waitlist mortality and provides excellent long-term outcomes for persons with end stage liver disease. Yet, utilization of LDLT has been limited in the United States (US). METHODS: In October 2021, the American Society of Transplantation held a consensus conference to identify important barriers to broader expansion of LDLT in the US, including data gaps, and make recommendations for impactful and feasible mitigation strategies to overcome these barriers. Domains addressed encompassed the entirety of the LDLT process. Representation from international centers and living donor kidney transplantation were included for their perspective/experience in addition to members across disciplines within the US liver transplantation community. A modified Delphi approach was employed as the consensus methodology. RESULTS: The predominant theme permeating discussion and polling results centered on culture; the beliefs and behaviors of a group of people perpetuated over time. CONCLUSIONS: Creating a culture of support for LDLT in the US is key for expansion and includes engagement and education of stakeholders across the spectrum of the process of LDLT. A shift from awareness of LDLT to acknowledgement of benefit of LDLT is the primary goal. Propagation of the maxim "LDLT is the best option" is pivotal.


Assuntos
Doença Hepática Terminal , Transplante de Fígado , Humanos , Estados Unidos , Doadores Vivos , Resultado do Tratamento
5.
Clin Transplant ; 37(7): e14967, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-36938716

RESUMO

INTRODUCTION: A successful living donor liver transplant (LDLT) is the culmination of a multifaceted process coordinated among key stakeholders. METHODS: We conducted an electronic survey of US liver transplant (LT) centers (August 26, 2021-October 10, 2021) regarding attitudes, barriers, and facilitators of LDLT to learn how to expand LDLT safely and effectively in preparation for the American Society of Transplantation Living Donor Liver Transplant Consensus Conference. RESULTS: Responses were received from staff at 58 programs (40.1% of US LT centers). There is interest in broadening LDLT (100% of LDLT centers, 66.7% of non-LDLT centers) with high level of agreement that LDLT mitigates donor shortage (93.3% of respondents) and that it should be offered to all suitable candidates (87.5% of respondents), though LDLT was less often endorsed as the best first option (29.5% of respondents). Key barriers at non-LDLT centers were institutional factors and surgical expertise, whereas those at LDLT centers focused on waitlist candidate and donor factors. Heterogeneity in candidate selection for LDLT, candidate reluctance to pursue LDLT, high donor exclusion rate, and disparities in access were important barriers. CONCLUSION: Findings from this study may help guide current and future expansion of LDLT more efficiently in the US. These efforts require clear and cohesive messaging regarding LDLT benefits, engagement of the public community, and dedicated resources to equitably increase LDLT access.


Assuntos
Transplante de Fígado , Humanos , Estados Unidos , Doadores Vivos , Seleção do Doador , Inquéritos e Questionários , Atitude , Resultado do Tratamento
6.
Clin Transplant ; 37(7): e14968, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37039541

RESUMO

The practice of LDLT currently delivers limited impact in western transplant centers. The American Society of Transplantation organized a virtual consensus conference in October 2021 to identify barriers and gaps to LDLT growth, and to provide evidence-based recommendations to foster safe expansion of LDLT in the United States. This article reports the findings and recommendations regarding innovations and advances in approaches to donor-recipient matching challenges, the technical aspects of the donor and recipient operations, and surgical training. Among these themes, the barriers deemed most influential/detrimental to LDLT expansion in the United States included: (1) prohibitive issues related to donor age, graft size, insufficient donor remnant, and ABO incompatibility; (2) lack of acknowledgment and awareness of the excellent outcomes and benefits of LDLT; (3) ambiguous messaging regarding LDLT to patients and hospital leadership; and (4) a limited number of proficient LDLT surgeons across the United States. Donor-recipient mismatching may be circumvented by way of liver paired exchange. The creation of a national registry to generate granular data on donor-recipient matching will guide the practice of liver paired exchange. The surgical challenges to LDLT are addressed herein and focuses on the development of robust training pathways resulting in proficiency in donor and recipient surgery. Utilizing strong mentorship/collaboration programs with novel training practices under the auspices of established training and certification bodies will add to the breadth and depth of training.


Assuntos
Transplante de Fígado , Humanos , Incompatibilidade de Grupos Sanguíneos , Transplante de Fígado/métodos , Doadores Vivos
7.
Transpl Infect Dis ; 24(6): e13941, 2022 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-35989545

RESUMO

INTRODUCTION: Surgical site infections (SSI) are a significant cause of morbidity in liver transplant recipients, and the current data in the pediatric population are limited. The goal of this study was to identify the incidence, classification, risk factors, and outcomes of SSIs among children undergoing liver transplantation (LT). METHODS: A single-center, retrospective descriptive analysis was performed of patients age ≤18 years undergoing LT between September 2007 and April 2017. SSI identified within the first 30 days were analyzed. Primary endpoints included incidence, classification, risk factors, and outcomes associated with SSIs. RESULTS: We included 86 patients, eight patients (9.3%) developed SSIs. Among segmental grafts (SG) recipients, 7/61 (11.4%) developed SSI. Among whole grafts recipients, 1/25 (4%) developed SSI. SSIs were associated with the presence of biliary complications (35% vs. 3%, p < .01; odds ratios 24, 95% CI: 3.41-487.37, p<.01). There were no differences in long term graft or patient survival associated with SSI. Patients who developed SSI were more likely to undergo reoperation (50% vs. 16.7%, p = .045) and had an increased total number of hospital days in the first 60 days post-transplant (30.5 vs. 12.5 days, p = .001). CONCLUSIONS: SSIs after pediatric LT was less frequent than what has been previously reported in literature. SSIs were associated with the presence of biliary complications without an increase in mortality. SG had an increased rate of biliary complications without an association to SSIs but, considering its positive impact on organ shortage barriers, should not be a deterrent to the utilization of SGs.


Assuntos
Sistema Biliar , Transplante de Fígado , Humanos , Criança , Adolescente , Infecção da Ferida Cirúrgica/epidemiologia , Infecção da Ferida Cirúrgica/etiologia , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos , Incidência , Fatores de Risco , Transplantados
8.
HPB (Oxford) ; 24(6): 817-824, 2022 06.
Artigo em Inglês | MEDLINE | ID: mdl-34742650

RESUMO

BACKGROUND: Outcomes of left lateral segment (LLS) grafts in pediatric recipients were compared between living (LD-LLS) and deceased donor (DD-LLS) grafts. METHODS: 195 LLS grafts (99DD-LLS-96LD-LLS) were analyzed with a median follow-up of 9.1years. The primary endpoints were overall patient/graft survival. RESULTS: LD-LLS grafts were younger (0.9vs.1.4years, p = 0.039), more likely to have a fulminant liver failure (17.9%vs.5.3%,p = 0.002), less likely to have a metabolic disorder (6.3%vs.25.5%,p = 0.002), and less likely to be undergoing retransplantation (5.3% vs.16.2%,p = 0.015). There was a trend toward decreased hepatic artery thrombosis in LD-LLS grafts (6.6% vs. 15.5%,p = 0.054). No differences in the overall biliary complications occurred. The LD-LLS group had prolonged survival compared to the DD-LLS group with 10-year survival rates of 81%, and 74% (p = 0.005), respectively. LD-LLS grafts had longer graft survival compared to DD-LLS grafts (10-year graft survival 85%vs.67%,p = 0.005). Recipient age >1year (HR 2.39,p = 0.026), aortic reconstruction (HR 2.12,p = 0.046) and vascular complication (HR 3.12,p < 0.001) were independent predictors of poor patient survival. Non-biliary liver disease (HR 2.17,p = 0.015), DD-LLS (HR 2.06,p = 0.034) and vascular complication (HR 4.61,p < 0.001) were independent predictors of poor graft survival. CONCLUSION: The use of SLT remains a viable option with excellent long-term outcomes. We show improved graft and patient survival with living donor grafts.


Assuntos
Hepatopatias , Transplante de Fígado , Criança , Sobrevivência de Enxerto , Humanos , Transplante de Fígado/efeitos adversos , Doadores Vivos , Estudos Retrospectivos , Resultado do Tratamento
9.
Am J Transplant ; 19(11): 3114-3123, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31152483

RESUMO

Organ shortage is a barrier to liver transplantation (LT). Split LT (SLT) increases organ utilization, saving 2 recipients. A simulation of Organ Procurement and Transplantation Network/United Network for Organ Sharing data (2007-2017) was performed to identify whole-organ LT grafts (WLT) that met the criteria for being splittable to 2 recipients. Waitlist consequences presented. Deceased donor (DD) livers transplanted as whole organs were evaluated for suitability to split. Of these DD organs, we identified the adolescent and adult recipients of WLT who were suitable for SLT. Pediatric candidates suitable to share the SLT were ascertained from DD match-run lists, and 1342 splittable DD organs were identified; 438 WLT recipients met the criteria for accepting a SLT. Review of the 438 DD match-run lists identified 420 children next on the list suitable for SLT. Three hundred thirty-three children (79%) underwent LT, but had longer wait-times compared to 591 actual pediatric SLT recipients (median 147 days vs 44 days, P  < 0.001). Thirty-three of 420 children died on waitlist after a mean 206 days (standard deviation 317). Sharing organs suitable for splitting increases the number of LT, saving more lives. With careful patient selection, SLT will not be a disadvantage to the adult recipients. With a children-first allocation scheme, SLT will naturally increase the number of allografts because adult organs are too large for small children.


Assuntos
Doença Hepática Terminal/mortalidade , Rejeição de Enxerto/mortalidade , Transplante de Fígado/mortalidade , Transplante de Fígado/estatística & dados numéricos , Seleção de Pacientes , Alocação de Recursos/normas , Doadores de Tecidos/provisão & distribuição , Listas de Espera/mortalidade , Adulto , Criança , Doença Hepática Terminal/cirurgia , Feminino , Seguimentos , Rejeição de Enxerto/etiologia , Sobrevivência de Enxerto , Humanos , Transplante de Fígado/efeitos adversos , Masculino , Prognóstico , Alocação de Recursos/estatística & dados numéricos , Fatores de Risco , Fatores de Tempo , Obtenção de Tecidos e Órgãos/estatística & dados numéricos
10.
Liver Transpl ; 25(5): 712-723, 2019 05.
Artigo em Inglês | MEDLINE | ID: mdl-30746848

RESUMO

Body image (BI) concerns have been reported to play a significant role in the psychological adaptation after organ transplantation. There is a paucity of data about BI beliefs in liver transplant recipients. We report the results of a cross-sectional study of 177 liver transplant recipients for whom we assessed BI, anxiety, depression, and quality of life (QOL) using validated instruments. Our results indicate that higher BI concerns correlated with higher levels of anxiety and depression. BI concerns were more elevated in females, younger patients, and patients with a lower income. Patients with chronic liver disease had more BI concerns than patients who received liver transplantation for acute liver failure. Specific BI concerns also correlated independently with QOL scores. We conclude that BI concerns are significant in liver transplant recipients and should be evaluated by clinicians involved in the mental health care of this population.


Assuntos
Transtornos Dismórficos Corporais/epidemiologia , Imagem Corporal/psicologia , Transplante de Fígado/psicologia , Qualidade de Vida , Transplantados/psicologia , Adaptação Psicológica , Adulto , Idoso , Ansiedade/diagnóstico , Ansiedade/epidemiologia , Ansiedade/psicologia , Transtornos Dismórficos Corporais/diagnóstico , Transtornos Dismórficos Corporais/psicologia , Estudos Transversais , Depressão/diagnóstico , Depressão/epidemiologia , Depressão/psicologia , Feminino , Humanos , Renda/estatística & dados numéricos , Transplante de Fígado/efeitos adversos , Masculino , Pessoa de Meia-Idade , Progesterona/análogos & derivados , Autorrelato/estatística & dados numéricos , Fatores Sexuais , Transplantados/estatística & dados numéricos , Adulto Jovem
11.
Palliat Support Care ; 17(1): 35-41, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-29860964

RESUMO

OBJECTIVE: Structured, empirically supported psychological interventions are lacking for patients who require organ transplantation. This stage IA psychotherapy development project developed and tested the feasibility, acceptability, tolerability, and preliminary efficacy of an 8-week group cognitive behavioral stress management intervention adapted for patients with end-stage liver disease awaiting liver transplantation. METHOD: Twenty-nine English-speaking United Network for Organ Sharing-registered patients with end-stage liver disease from a single transplantation center enrolled in 8-week, group cognitive-behavioral liver stress management and relaxation training intervention adapted for patients with end-stage liver disease. Patients completed pre- and postintervention surveys that included the Beck Depression Inventory II and the Beck Anxiety Inventory. Feasibility, acceptability, tolerability, and preliminary efficacy were assessed.ResultAttendance rate was 69.40%. The intervention was rated as "good" to "excellent" by 100% of participants who completed the postintervention survey in teaching them new skills to relax and to cope with stress, and by 94.12% of participants in helping them feel supported while waiting for a liver transplant. No adverse events were recorded over the course of treatment. Attrition was 13.79%. Anxious and depressive symptoms were not statistically different after the intervention.Significance of resultsThe liver stress management and relaxation training intervention is feasible, acceptable, and tolerable to end-stage liver disease patients within a transplant clinic setting. Anxious and depressive symptoms remained stable postintervention. Randomized controlled trials are needed to study the intervention's effectiveness in this population.


Assuntos
Terapia Cognitivo-Comportamental/normas , Doença Hepática Terminal/terapia , Transplante de Fígado/psicologia , Estresse Psicológico/psicologia , Distribuição de Qui-Quadrado , Terapia Cognitivo-Comportamental/métodos , Doença Hepática Terminal/psicologia , Estudos de Viabilidade , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Projetos Piloto , Psicometria/instrumentação , Psicometria/métodos , Estresse Psicológico/etiologia , Estresse Psicológico/terapia , Inquéritos e Questionários
12.
Pediatr Transplant ; 22(4): e13184, 2018 06.
Artigo em Inglês | MEDLINE | ID: mdl-29654655

RESUMO

Development of macrovesicular steatosis post-LT in patients with PFIC-1 is increasingly being observed, with the etiology not fully understood. We highlight successful and effective EBD for reversal of allograft steatosis in 2 patients with PFIC-1 disease and discuss our experience with internal biliary diversion in this patient population.


Assuntos
Colestase Intra-Hepática/cirurgia , Drenagem/métodos , Fígado Gorduroso/terapia , Transplante de Fígado , Complicações Pós-Operatórias/terapia , Aloenxertos , Fígado Gorduroso/etiologia , Feminino , Humanos , Lactente , Masculino , Transplante Homólogo
13.
Ann Hepatol ; 16(4): 621-629, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28611270

RESUMO

Neuroendocrine tumors comprise approximately 1-2% of all gastrointestinal tumors, and while the liver is the most common site for metastasis of these tumors, primary hepatic neuroendocrine tumors are very rare entities. Since first being reported in 1958, there have been less than 150 cases reported in the literature. Because of the infrequent occurrence of these tumors, the pool of data available for analysis regarding these tumors is small. As such, the medical community must rely on the publication of case report data to further enlarge this data pool, with the hopes of eventually having enough data to draw meaningful, statistically significant conclusions with regard to diagnosis and management of these rare tumors. We have encountered two patients at our institution within the last year with primary hepatic neuroendocrine tumors. We present their cases in this manuscript in an effort to contribute to the available data on the disease. We also provide a concise review of the literature available to date regarding primary hepatic neuroendocrine tumors.


Assuntos
Carcinoma Neuroendócrino , Neoplasias Hepáticas , Adulto , Biomarcadores Tumorais/análise , Biópsia , Carcinoma Neuroendócrino/química , Carcinoma Neuroendócrino/patologia , Carcinoma Neuroendócrino/cirurgia , Hepatectomia , Humanos , Imuno-Histoquímica , Neoplasias Hepáticas/química , Neoplasias Hepáticas/patologia , Neoplasias Hepáticas/cirurgia , Imageamento por Ressonância Magnética , Masculino , Tomografia Computadorizada por Raios X , Resultado do Tratamento
14.
Mol Genet Metab ; 117(1): 19-26, 2016 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-26597322

RESUMO

BACKGROUND: The hepatic urea cycle is the main metabolic pathway for detoxification of ammonia. Inborn errors of urea cycle function present with severe hyperammonemia and a high case fatality rate. Long-term prognosis depends on the residual activity of the defective enzyme. A reliable method to estimate urea cycle activity in-vivo does not exist yet. The aim of this study was to evaluate a practical method to quantify (13)C-urea production as a marker for urea cycle function in healthy subjects, patients with confirmed urea cycle defect (UCD) and asymptomatic carriers of UCD mutations. METHODS: (13)C-labeled sodium acetate was applied orally in a single dose to 47 subjects (10 healthy subjects, 28 symptomatic patients, 9 asymptomatic carriers). RESULTS: The oral (13)C-ureagenesis assay is a safe method. While healthy subjects and asymptomatic carriers did not differ with regards to kinetic variables for urea cycle flux, symptomatic patients had lower (13)C-plasma urea levels. Although the (13)C-ureagenesis assay revealed no significant differences between individual urea cycle enzyme defects, it reflected the heterogeneity between different clinical subgroups, including male neonatal onset ornithine carbamoyltransferase deficiency. Applying the (13)C-urea area under the curve can differentiate between severe from more mildly affected neonates. Late onset patients differ significantly from neonates, carriers and healthy subjects. CONCLUSION: This study evaluated the oral (13)C-ureagenesis assay as a sensitive in-vivo measure for ureagenesis capacity. The assay has the potential to become a reliable tool to differentiate UCD patient subgroups, follow changes in ureagenesis capacity and could be helpful in monitoring novel therapies of UCD.


Assuntos
Acetato de Sódio/farmacocinética , Distúrbios Congênitos do Ciclo da Ureia/diagnóstico , Ureia/metabolismo , Administração Oral , Adolescente , Adulto , Isótopos de Carbono/metabolismo , Criança , Pré-Escolar , Feminino , Humanos , Hiperamonemia/diagnóstico , Hiperamonemia/metabolismo , Lactente , Recém-Nascido , Masculino , Pessoa de Meia-Idade , Monitorização Fisiológica , Doença da Deficiência de Ornitina Carbomoiltransferase/diagnóstico , Traçadores Radioativos , Acetato de Sódio/administração & dosagem , Adulto Jovem
15.
Clin Transplant ; 30(3): 306-11, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26780305

RESUMO

Vancomycin-resistant enterococci (VRE) infections cause significant morbidity in liver transplant recipients. The epidemiology and impact of pre-transplant colonization with VRE among patients who undergo liver transplantation are poorly understood. We conducted an observational cohort study to identify risk factors and outcomes associated with pre-transplant VRE colonization and described the molecular diversity among VRE strains colonizing patients who undergo liver transplantation. Perirectal VRE surveillance cultures were performed prior to transplantation. Repetitive sequence-based polymerase chain reaction (rep-PCR) testing was used to identify clonality among VRE isolates. Of 61 patients who underwent pre-transplant VRE surveillance and subsequent liver transplantation, 27 (44%) were colonized with VRE. In multivariate analysis, pre-transplant VRE colonization was associated with central venous catheterization (OR 9.4, 95% confidence interval [CI]= 1.3-70.2, p = 0.03) and rifaximin use (OR 15.4, 95% CI 1.5-159.7, p = 0.02). Pre-transplant VRE colonization was associated with more hospital days post-transplant (26.6 vs. 16.1 d, p = 0.04). Of VRE-colonized patients analyzed with rep-PCR, 68% were colonized with the same strain as another patient in the cohort. Active surveillance identifies VRE-colonized patients who may benefit from targeted antimicrobial prophylaxis and enhanced infection prevention measures to prevent VRE spread. The relationship between rifaximin receipt and VRE colonization warrants further study. The identification of similar VRE isolates may suggest linked transmission during pre-transplant hospitalizations, which should be further investigated in prospective studies.


Assuntos
Antibacterianos/efeitos adversos , Enterococcus/efeitos dos fármacos , Infecções por Bactérias Gram-Positivas/epidemiologia , Hepatopatias/cirurgia , Transplante de Fígado/efeitos adversos , Resistência a Vancomicina , Vancomicina/efeitos adversos , Connecticut/epidemiologia , Enterococcus/isolamento & purificação , Feminino , Seguimentos , Infecções por Bactérias Gram-Positivas/tratamento farmacológico , Infecções por Bactérias Gram-Positivas/microbiologia , Infecções por Bactérias Gram-Positivas/patologia , Humanos , Hepatopatias/microbiologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Retrospectivos , Fatores de Risco
16.
Pediatr Transplant ; 20(8): 1157-1163, 2016 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-27762491

RESUMO

NAFLD is a common condition linked to obesity, type 2 diabetes, and metabolic syndrome. Simple hepatic steatosis is a risk factor for inflammatory reactions in the liver (NASH), which may lead to cirrhosis. While the mechanism is unclear, NAFLD and NASH are associated with panhypopituitarism, which in the pediatric population often results from craniopharyngioma or pituitary adenoma and the sequelae of treatment, causing hypothyroidism, adrenal insufficiency, hypogonadotropic hypogonadism, and GH deficiency. Refractory NAFLD in panhypopituitarism may be amenable to GH replacement. Here, we report a pediatric case of NASH secondary to panhypopituitarism from craniopharyngioma, which recurred by 11 months after LDLT. Despite low-dose GH replacement, the patient remained GH deficient. Pubertal dosed GH therapy led to rapid and complete resolution of hepatic steatosis, which we tracked using serial 1 H MRS. Pediatric patients with NASH cirrhosis secondary to panhypopituitarism can be good candidates for liver transplantation, but hormone deficiencies predispose to recurrence after transplant. High-dose GH replacement should be considered in pediatric patients with GH deficiency and recurrent disease. A multidisciplinary team approach is essential for successful outcomes.


Assuntos
Hormônio do Crescimento Humano/uso terapêutico , Hipopituitarismo/tratamento farmacológico , Transplante de Fígado , Hepatopatia Gordurosa não Alcoólica/tratamento farmacológico , Hepatopatia Gordurosa não Alcoólica/cirurgia , Adolescente , Fibrose/etiologia , Humanos , Hipopituitarismo/complicações , Masculino , Hepatopatia Gordurosa não Alcoólica/complicações , Recidiva , Resultado do Tratamento
18.
Prog Transplant ; 25(4): 289-96, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26645920

RESUMO

BACKGROUND: Psychotic disorders are considered a relative or absolute contraindication to organ transplant, but information about their impact on transplant is limited. OBJECTIVE: To describe the clinical course of psychotic patients while they were on the waiting list and the outcomes of patients with psychotic disorders undergoing evaluation for organ transplant. METHODS: Thirty-eight transplant candidates with a diagnosis of psychotic disorder were analyzed in this descriptive study. The following variables were collected before transplant: demographics, type of transplant, cause of organ failure, medical comorbid conditions, and psychiatric variables (diagnosis, hospitalizations, treatment, substance abuse, family history, suicide attempts). For transplant recipients, the following posttransplant variables were recorded: rejection, toxic effects of medication, nonadherence, psychotic episodes (number, time interval after transplant), and number of hospitalizations. RESULTS: Of the 38 transplant candidates, 34 had a history of psychotic disorder before transplant. Nineteen (56%) of the 34 were listed for transplant, and 10 (29%) underwent transplant. Median follow-up time was 1.9 (IQR, 0.16-17.9) years. Among organ recipients with a history of psychotic disorders, psychiatric hospitalizations were 0.42 per patient per year (PPY), psychotic episodes 0.68 PPY, rejection 0.21 PPY, and toxic effects of immunosuppressants 0.05 PPY. None of the recipients lost their graft. CONCLUSIONS: Patients with a history of psychotic disorder can do well after organ transplant. Further studies are needed with factor analyses including severity of psychosis, medication adherence, and associated comorbid conditions.


Assuntos
Transplante de Rim/psicologia , Transplante de Fígado/psicologia , Transplante de Órgãos/psicologia , Transtornos Psicóticos/complicações , Transplantados/psicologia , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores de Risco , Estados Unidos , Listas de Espera
19.
J Hepatol ; 61(5): 1178-83, 2014 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-25016225

RESUMO

Hepatocellular carcinoma (HCC) rarely occurs in childhood. We describe a patient with new onset of pruritus at 8 months of age who at 17 months of age was found to have a 2.5 cm HCC. To delineate the possible genetic basis of this tumour, we performed whole exome sequencing (WES) of the germline DNA and identified two novel predictably deleterious missense mutations in ABCB11, encoding bile salt export pump (BSEP), confirmed in the parental DNA as bi-allelic and inherited. Although inherited ABCB11 mutations have previously been linked to HCC in a small number of cases, the molecular mechanisms of hepatocellular carcinogenesis in ABCB11 disease are unknown. WES of the HCC tissue uncovered somatic driver mutations in the beta-catenin (CTNNB1) and nuclear-factor-erythroid-2-related-factor-2 (NFE2L2) genes. Moreover, clonality analysis predicted that the CTNNB1 mutation was clonal and occurred earlier during carcinogenesis, whereas the NFE2L2 mutation was acquired later. Interestingly, background liver parenchyma showed no inflammation or fibrosis and BSEP expression was preserved. This is the first study to identify somatic CTNNB1 and NFE2L2 mutations in early childhood arisen in the setting of inherited bi-allelic ABCB11 mutations. Rapid WES analysis expedited this child's diagnosis and treatment, and likely improved her prognosis.


Assuntos
Transportadores de Cassetes de Ligação de ATP/genética , Carcinoma Hepatocelular/genética , Neoplasias Hepáticas/genética , Mutação , Fator 2 Relacionado a NF-E2/genética , beta Catenina/genética , Membro 11 da Subfamília B de Transportadores de Cassetes de Ligação de ATP , Sequência de Aminoácidos , Sequência de Bases , Carcinoma Hepatocelular/complicações , Carcinoma Hepatocelular/patologia , Colestase Intra-Hepática/complicações , Colestase Intra-Hepática/genética , DNA de Neoplasias/genética , Feminino , Mutação em Linhagem Germinativa , Humanos , Lactente , Neoplasias Hepáticas/complicações , Neoplasias Hepáticas/patologia , Dados de Sequência Molecular , Mutação de Sentido Incorreto , Homologia de Sequência de Aminoácidos
20.
J Surg Res ; 187(1): 297-301, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24252852

RESUMO

BACKGROUND: Arterialized orthotopic liver transplantation (OLT) in the mouse mimics human liver transplantation physiologically and clinically. The present method of sutured anastomosis for reconstruction of the hepatic artery is complex and is associated with high incidence of complications and failure. This makes the endpoint assessment of using this complex model difficult because of the many variables of the technical aspect. METHODS: A total of 14 pairs of donors and recipients from syngeneic male mice were used for arterialized OLT. The grafts were stored in University of Wisconsin solution at 4°C for less than 4 h, and the recipients underwent OLT using a two-cuff technique. The arterial reconstruction was facilitated by the use of a single stent connecting the donor liver artery segment to the recipient common hepatic artery. RESULTS: All 14 recipients survived with the time for arterial reconstruction ranging from 4-10 min. Patency of the artery was confirmed by transecting the artery near the graft 2 and 14 d after transplantation. At day 2, five of the six arteries transected were patent and at day 14, seven of the remaining eight were patent for an overall patency rate of 85.7%. CONCLUSIONS: The stent-facilitated arterial reconstruction can be done quickly with a high patency rate. This model expands the translational research efforts to address marginal livers such as steatotic livers.


Assuntos
Prótese Vascular , Artéria Hepática/cirurgia , Transplante de Fígado/métodos , Stents , Procedimentos Cirúrgicos Vasculares/métodos , Anastomose Cirúrgica/instrumentação , Anastomose Cirúrgica/métodos , Animais , Artéria Celíaca/cirurgia , Fígado/irrigação sanguínea , Fígado/cirurgia , Transplante de Fígado/instrumentação , Masculino , Camundongos , Camundongos Endogâmicos C57BL , Modelos Animais , Grau de Desobstrução Vascular , Procedimentos Cirúrgicos Vasculares/instrumentação
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