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1.
Artigo em Inglês | MEDLINE | ID: mdl-39160627

RESUMO

BACKGROUND: In Japan, there has never been a national analysis of pediatric deceased donor liver transplantation (pDDLT) based on donor and recipient factors. We constructed a Japanese nationwide database and assessed outcomes of pDDLT focusing on the pediatric prioritization system introduced in 2018. METHODS: We collected data on pDDLTs (<18 years) performed between 1999 and 2021 from the Japan Organ Transplant Network and Japanese Liver Transplantation Society, identified risk factors for graft survival and compared the characteristics and graft survival in pDDLTs conducted before and after the introduction of the pediatric prioritization system. RESULTS: Overall, 112 cases of pDDLT were included, with a 1-year graft survival rate of 86.6%. Four poor prognostic factors were identified: recipient intensive care unit stay, model for end-stage liver disease/pediatric end-stage liver disease score, donor cause of death, and donor total bilirubin. After the introduction of the system, allografts from pediatric donors were more reliably allocated to pediatric recipients and the annual number of pDDLTs increased. The 1-year graft survival rate improved significantly as did pDDLT conditions indicated by the risk factors. CONCLUSIONS: Under the revised allocation system, opportunities for pDDLT increased, resulting in favorable recipient and donor conditions and improved survival.

2.
BJS Open ; 8(4)2024 Jul 02.
Artigo em Inglês | MEDLINE | ID: mdl-39092977

RESUMO

BACKGROUND: Validating the expanded criteria for living donor liver transplantation for hepatocellular carcinoma using national data is highly significant. The aim of this study was to evaluate the validity of the new Japanese criteria for living donor liver transplantation for hepatocellular carcinoma patients and identify factors associated with a poor prognosis using the Japanese national data set. METHODS: The study population comprised patients who underwent living donor liver transplantation for hepatocellular carcinoma at 37 centres in Japan between 2010 and 2018. In a nationwide survey, the overall survival and recurrence-free survival rates were evaluated based on the new Japanese criteria for applying the 5-5-500 rule when extending the indication beyond the Milan criteria. Prognostic factors within the Japanese criteria were determined using the Cox proportional hazards model. RESULTS: Patients within (485 patients) and beyond (31 patients) the Japanese criteria exhibited 5-year overall survival rates of 81% and 58% and 5-year recurrence-free survival rates of 77% and 48% respectively. Patients who met the Milan criteria, but not the 5-5-500 rule, had poorer outcomes. Multivariate analysis for 474 patients identified a neutrophil-to-lymphocyte ratio greater than or equal to 5 and a history of hepatectomy as independent risk factors. CONCLUSION: This nationwide survey confirms the validity of the Japanese criteria. The poor prognostic factors within the Japanese criteria include a neutrophil-to-lymphocyte ratio greater than or equal to 5 and previous hepatectomy.


Assuntos
Carcinoma Hepatocelular , Neoplasias Hepáticas , Transplante de Fígado , Doadores Vivos , Humanos , Carcinoma Hepatocelular/cirurgia , Carcinoma Hepatocelular/mortalidade , Neoplasias Hepáticas/cirurgia , Neoplasias Hepáticas/mortalidade , Masculino , Japão/epidemiologia , Feminino , Pessoa de Meia-Idade , Adulto , Fatores de Risco , Idoso , Prognóstico , Estudos de Coortes , Taxa de Sobrevida , Hepatectomia , Modelos de Riscos Proporcionais , Intervalo Livre de Doença , População do Leste Asiático
3.
New Microbiol ; 47(1): 52-59, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38700884

RESUMO

Monitoring Epstein-Barr virus (EBV) and cytomegalovirus (CMV) infection after transplantation is recommended to enable preemptive therapy. However, the most suitable sample type remains unclear. Patients who underwent hematopoietic stem cell or liver transplantation were included in this study. Viral loads in sequential whole-blood and plasma samples were retrospectively analyzed. EBV DNA was detected more frequently in whole blood (55%) than in plasma (18%). The detection rate of CMV DNA was similar between the two sample types. The correlation of viral loads between the two sample types were 0.515 and 0.688 for EBV and CMV, respectively. Among paired samples in which EBV DNA was detected in whole blood, the plasma EBV detection rate was significantly higher in patients who underwent hematopoietic stem cell transplantation than in those who underwent liver transplantation. The viral DNA load in whole blood and plasma showed similar trends. The EBV detection rate was higher in whole blood, and a high correlation was observed between CMV DNA loads and whole blood and plasma. These results indicate that whole blood is more sensitive for monitoring both EBV and CMV, whereas plasma is a potential alternative sample for monitoring CMV.


Assuntos
Infecções por Citomegalovirus , Citomegalovirus , Infecções por Vírus Epstein-Barr , Herpesvirus Humano 4 , Carga Viral , Humanos , Citomegalovirus/genética , Citomegalovirus/isolamento & purificação , Infecções por Citomegalovirus/virologia , Infecções por Citomegalovirus/sangue , Infecções por Citomegalovirus/diagnóstico , Herpesvirus Humano 4/genética , Herpesvirus Humano 4/isolamento & purificação , Infecções por Vírus Epstein-Barr/virologia , Infecções por Vírus Epstein-Barr/sangue , Infecções por Vírus Epstein-Barr/diagnóstico , Masculino , Feminino , Pessoa de Meia-Idade , Adulto , Estudos Retrospectivos , DNA Viral/sangue , Adulto Jovem , Transplante de Células-Tronco Hematopoéticas , Idoso , Plasma/virologia , Transplante de Fígado , Adolescente
4.
Cancer Diagn Progn ; 4(3): 295-300, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38707737

RESUMO

Background/Aim: Surgical outcomes of colorectal cancer (CRC) in patients with renal failure (RF) remain to be clarified. The objective of this research was to investigate how RF impacts the surgical outcomes in patients with CRC. Patients and Methods: A retrospective analysis was performed on clinical data from 633 patients who underwent colorectal resection for CRC between January 2017 and December 2021. Outcomes of the patients with and without RF were compared. RF was defined as estimated Glomerular Filtration Rate less than 30. Results: Forty-five (7%) patients with RF were identified. RF was a significant risk factor for postoperative complications after colorectal cancer surgery (odds ratio=2.19, 95% confidence interval=1.08-4.42, p=0.0284). The patients with RF had significantly more comorbidity (p=0.016), and higher American Society of Anesthesiologists physical status (p<0.01). Hemoglobin level (p<0.01) and PNI (p<0.01) were significantly lower in those with RF. Postoperative complications were significantly higher (p=0.016), and the postoperative hospital stay was significantly longer (p<0.01) among patients with RF compared to those without RF. Patients with RF, excluding those undergoing hemodialysis, had significantly more complications compared to those without RF (p=0.004). Conclusion: Careful attention should be paid to perioperative management in RF colorectal cancer patients.

5.
J Magn Reson Imaging ; 2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38490816

RESUMO

BACKGROUND: Portal vein thrombosis (PVT) is thought to arise from stagnant blood flow, yet conclusive evidence is lacking. Relative residence time (RRT) assessed using 4D Flow MRI may offer insight into portal flow stagnation. PURPOSE: To explore the relationship between RRT values and the presence of PVT in cirrhotic participants. STUDY TYPE: Prospective. POPULATION: Forty-eight participants with liver cirrhosis (27 males, median age 67 years [IQR: 57-73]) and 20 healthy control participants (12 males, median age 45 years [IQR: 40-54]). FIELD STRENGTH/SEQUENCE: 3 T/4D Flow MRI. ASSESSMENT: Laboratory (liver and kidney function test results and platelet count) and clinical data (presence of tumors and other imaging findings), and portal hemodynamics derived from 4D Flow MRI (spatiotemporally averaged RRT [RRT-mean], flow velocity, and flow rate) were analyzed. STATISTICAL TESTS: We used multivariable logistic regression, adjusted by selected covariates through the Lasso method, to explore whether RRT-mean is an independent risk factor for PVT. The area under the ROC curve (AUC) was also calculated to assess the model's discriminative ability. P < 0.05 indicated statistical significance. RESULTS: The liver cirrhosis group consisted of 16 participants with PVT and 32 without PVT. Higher RRT-mean values (odds ratio [OR] 11.4 [95% CI: 2.19, 118]) and lower platelet count (OR 0.98 per 1000 µL [95% CI: 0.96, 0.99]) were independent risk factors for PVT. The incorporation of RRT-mean (AUC, 0.77) alongside platelet count (AUC, 0.75) resulted in an AUC of 0.84. When including healthy control participants, RRT-mean had an adjusted OR of 12.4 and the AUC of the combined model (RRT-mean and platelet count) was 0.90. DATA CONCLUSION: Prolonged RRT values and low platelet count were significantly associated with the presence of PVT in cirrhotic participants. RRT values derived from 4D Flow MRI may have potential clinical relevance in the management of PVT. EVIDENCE LEVEL: 2 TECHNICAL EFFICACY: Stage 2.

6.
Ann Transplant ; 28: e941456, 2023 Dec 15.
Artigo em Inglês | MEDLINE | ID: mdl-38098225

RESUMO

BACKGROUND The management and fate of liver transplant (LT) recipients with preformed donor-specific antibodies (pDSA) remain controversial. The aim of this study was to evaluate the clinical impact of rituximab desensitization on pDSA in LT recipients. MATERIAL AND METHODS This retrospective observational study enrolled 120 LT patients aged ≥18 years. Patients with pDSA were administered 500 mg/body rituximab 1-21 days before LT, except for those who had an active infection or had insufficient time to receive rituximab. We allocated patients to groups with or without pDSA, and then divided patients with pDSA into rituximab (+) and rituximab (-) groups for further analysis. RESULTS Twenty-three patients (19.2%) with pDSA were identified. Of these, 18 received rituximab and 5 did not receive rituximab. No patients developed adverse events related to rituximab. In both groups, the levels of pDSA class I in all patients were decreased immediately after LT, whereas those of pDSA class II decreased slowly. There were no significant differences in pathology findings and overall survival between patients with pDSA who were rituximab (+) or rituximab (-), and between patients with or without pDSA. CONCLUSIONS Rituximab desensitization for LT patients with pDSA was managed successfully without significant complications. Due to the small sample size, we could not demonstrate the benefit of rituximab desensitization for LT patients compared with the rituximab (-) group. Additionally, clinical outcomes in patients with pDSA, with or without rituximab, were similar to those without pDSA. Rituximab desensitization might be not essential for LT.


Assuntos
Transplante de Rim , Transplante de Fígado , Humanos , Adolescente , Adulto , Rituximab/uso terapêutico , Transplante de Fígado/efeitos adversos , Transplante de Rim/efeitos adversos , Anticorpos , Doadores de Tecidos , Estudos Retrospectivos , Rejeição de Enxerto , Sobrevivência de Enxerto , Antígenos HLA
7.
Stem Cell Reports ; 18(11): 2071-2083, 2023 11 14.
Artigo em Inglês | MEDLINE | ID: mdl-37832542

RESUMO

UGT1A1 (UDP glucuronosyltransferase family 1 member A1) is the primary enzyme required for bilirubin conjugation, which is essential for preventing hyperbilirubinemia. Animal models lack key human organic anion transporting polypeptides with distinct epigenetic control over bilirubin metabolism, necessitating a human model to interrogate the regulatory mechanism behind UGT1A1 function. Here, we use induced pluripotent stem cells to develop human liver organoids that can emulate conjugation failure phenotype. Bilirubin conjugation assays, chromatin immunoprecipitation, and transcriptome analysis elucidated the role of glucocorticoid antagonism in UGT1A1 activation. This antagonism prevents the binding of transcriptional repressor MECP2 at the expense of NRF2 with associated off-target effects. Therefore, we introduced functional GULO (L-gulonolactone oxidase) in human organoids to augment intracellular ascorbate for NRF2 reactivation. This engineered organoid conjugated more bilirubin and protected against hyperbilirubinemia when transplanted in immunosuppressed Crigler-Najjar syndrome rat model. Collectively, we demonstrate that our organoid system serves as a manipulatable model for interrogating hyperbilirubinemia and potential therapeutic development.


Assuntos
Síndrome de Crigler-Najjar , Células-Tronco Pluripotentes , Humanos , Animais , Ratos , Bilirrubina/farmacologia , Bilirrubina/metabolismo , Fator 2 Relacionado a NF-E2/metabolismo , Fígado/metabolismo , Síndrome de Crigler-Najjar/genética , Síndrome de Crigler-Najjar/terapia , Hiperbilirrubinemia/genética , Hiperbilirrubinemia/metabolismo , Hiperbilirrubinemia/terapia , Glucuronosiltransferase/genética , Glucuronosiltransferase/metabolismo , Células-Tronco Pluripotentes/metabolismo
8.
Medicine (Baltimore) ; 102(38): e35324, 2023 Sep 22.
Artigo em Inglês | MEDLINE | ID: mdl-37746947

RESUMO

RATIONALE: The purpose of this case report is to describe a case of successful early rehabilitation intervention for simultaneous liver and kidney transplantation (SLKT). PATIENT CONCERNS: A 51-year-old Japanese man was diagnosed with Caroli disease 27 years ago. Hemodialysis was introduced due to end-stage renal disease 17 years ago. DIAGNOSES: After successful SLKT, the patient was extubated on postoperative day (POD) 1, liberated from dialysis on POD 4, and discharged from the intensive care unit on POD 9. INTERVENTIONS: Supervised rehabilitation was started on POD 2, and the patient was able to walk 100 m on POD 9. Electrical muscle stimulation therapy was started to improve muscle weakness in both legs on POD 16, and aerobic exercise using a cycle-ergometer was started on POD 24. OUTCOMES: The 6-minute walking distance improved from 324 m on POD 14 to 501 m on POD 28. The patient could walk 4000 to 5000 steps per day at hospital discharge, and was discharged home on POD 32. There were no adverse events, including worsening hepatic or renal function, during the rehabilitation period. One month after discharge, the patient was able to perform 30 to 40 minutes of aerobic exercise every day, and returned to work 5 months after discharge. LESSONS: This case shows that early rehabilitation intervention immediately after SLKT safely and rapidly improved physical performance without adverse events. The results in the present case suggest that regular physical assessment and appropriate interventions with a variety of exercise modalities can contribute to improved physical performance in SLKT patients.


Assuntos
Transplante de Rim , Transplante de Fígado , Masculino , Humanos , Pessoa de Meia-Idade , Diálise Renal , Doadores Vivos , Fígado
10.
Surg Case Rep ; 9(1): 100, 2023 Jun 08.
Artigo em Inglês | MEDLINE | ID: mdl-37286818

RESUMO

BACKGROUND: Neuroblastoma is the most common extracranial solid tumor in childhood. Stage 4S neuroblastoma is a unique subset of neuroblastoma characterized by a favorable course and potentially low malignancy with a high rate of spontaneous tumor regression. However, recent reports have shown that there is a subgroup of patients with stage 4S neuroblastoma characterized by MYCN amplification, chromosomal aberrations, age of < 2 months at diagnosis, and significantly poorer outcomes. CASE PRESENTATION: A 1-month-old male infant with a huge abdominal tumor was transferred to our hospital and diagnosed with stage 4S neuroblastoma. The patient showed respiratory distress due to abdominal compartment syndrome secondary to massive hepatic invasion, and he required a silo operation and mechanical ventilation. After chemotherapy using carboplatin and etoposide, the infiltrative massive hepatic invasion was resolved and the abdominal compartment syndrome gradually improved; however, liver dysfunction as evidenced by hyperbilirubinemia, coagulopathy, and hyperammonemia continued. At the age of 3 months, living-donor liver transplantation was performed for treatment of sustained liver failure using a reduced lateral segment graft from the patient's father. Post-transplant liver function recovered immediately. Examination of the explanted liver demonstrated that the majority of liver tissue had been replaced by fibroblastic cells after massive hepatocyte dropout. There were only small areas of residual neuroblastoma cells in the liver specimen. The patient was discharged from the hospital 5 months after transplantation with home intermittent respiratory support. At the time of this writing (23 months after liver transplantation), he was in good condition with no signs of recurrence of neuroblastoma. CONCLUSIONS: We have herein presented a case of successful pediatric living-donor liver transplantation for sustained liver failure even after resolution of infiltrative massive hepatic invasion of stage 4S neuroblastoma. Our case clearly shows that liver transplantation can be added as an appropriate extended treatment option for liver failure after resolution of stage 4S neuroblastoma.

11.
J Gastroenterol ; 58(6): 586-597, 2023 06.
Artigo em Inglês | MEDLINE | ID: mdl-37099152

RESUMO

BACKGROUND: Pancreatic neuroendocrine neoplasms (PanNENs) are a heterogeneous group of tumors. Although the prognosis of resected PanNENs is generally considered to be good, a relatively high recurrence rate has been reported. Given the scarcity of large-scale reports about PanNEN recurrence due to their rarity, we aimed to identify the predictors for recurrence in patients with resected PanNENs to improve prognosis. METHODS: We established a multicenter database of 573 patients with PanNENs, who underwent resection between January 1987 and July 2020 at 22 Japanese centers, mainly in the Kyushu region. We evaluated the clinical characteristics of 371 patients with localized non-functioning pancreatic neuroendocrine tumors (G1/G2). We also constructed a machine learning-based prediction model to analyze the important features to determine recurrence. RESULTS: Fifty-two patients experienced recurrence (14.0%) during the follow-up period, with the median time of recurrence being 33.7 months. The random survival forest (RSF) model showed better predictive performance than the Cox proportional hazards regression model in terms of the Harrell's C-index (0.841 vs. 0.820). The Ki-67 index, residual tumor, WHO grade, tumor size, and lymph node metastasis were the top five predictors in the RSF model; tumor size above 20 mm was the watershed with increased recurrence probability, whereas the 5-year disease-free survival rate decreased linearly as the Ki-67 index increased. CONCLUSIONS: Our study revealed the characteristics of resected PanNENs in real-world clinical practice. Machine learning techniques can be powerful analytical tools that provide new insights into the relationship between the Ki-67 index or tumor size and recurrence.


Assuntos
Tumores Neuroendócrinos , Neoplasias Pancreáticas , Humanos , Tumores Neuroendócrinos/diagnóstico , Tumores Neuroendócrinos/cirurgia , Antígeno Ki-67 , Estudos Retrospectivos , Prognóstico , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/cirurgia
12.
BMC Gastroenterol ; 23(1): 106, 2023 Apr 05.
Artigo em Inglês | MEDLINE | ID: mdl-37020184

RESUMO

OBJECTIVE: Comorbid psychiatric disorders negatively affect the survival rate of patients with some physical disorders. In liver transplant recipients, various psychiatric disorders have been identified as worsening prognosis. However, little is known about how the presence of any comorbid (overall) disorders affect the survival rate of transplant recipients. In this study, we examined the effect of overall comorbid psychiatric disorders on survival rate in liver transplant recipients. METHODS: A total of 1006 recipients who underwent liver transplantation between September 1997 and July 2017 across eight transplant facilities with a psychiatric consultation-liaison team were identified consecutively. Recipients were categorized into those with comorbid psychiatric disorders and those without comorbid psychiatric disorders. In the comorbid psychiatric disorder group, psychiatric disorder diagnosis and time of diagnosis were investigated retrospectively. RESULTS: Of the 1006 recipients, 294 (29.2%) had comorbid psychiatric disorders. Comorbid psychiatric disorders in the 1006 recipients were insomnia (N = 107, 10.6%), delirium (N = 103, 10.2%), major depressive disorder (N = 41, 4.1%), adjustment disorder (N = 19, 1.9%), anxiety disorder (N = 17, 1.7%), intellectual disability (N = 11, 1.1%), autism spectrum disorder (N = 7, 0.7%), somatic symptom disorder (N = 4, 0.4%) schizophrenia (N = 4, 0.4%), substance use disorder (N = 24, 2.4%) and personality disorder (N = 2, 0.2%). The most common time of psychiatric disorder diagnosis was within the first 3 months after liver transplantation (51.6%). The final mortality in patients with comorbid psychiatric disorder diagnosis during the five periods (pretransplant, transplant to 3 months, months to 1 year, 1 to 3 years, and over 3 years posttransplant) was 16.2%, 18.8%, 39.1%, 28.6%, and 16.2% respectively, and there were no significant differences between the five periods (χ2 = 8.05, df = 4, p = 0.09). Overall comorbid psychiatric disorders were significantly associated with shorter survival time (log-rank test: p = 0.01, hazard ratio: 1.59 [95% confidence interval: 1.14-2.21], survival rate at the endpoint [%]: 62.0 vs. 83.3). However, after adjusting for confounding variables using Cox proportional hazards regression, there was no significant effect of overall comorbid psychiatric disorders on prognosis. CONCLUSION: Comorbid psychiatric disorders did not affect the survival rate of liver transplant recipients in this study.


Assuntos
Transtorno do Espectro Autista , Transtorno Depressivo Maior , Transplante de Fígado , Transtornos Mentais , Humanos , Estudos Retrospectivos , Encaminhamento e Consulta
13.
Eur Heart J Open ; 3(2): oead028, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37026023

RESUMO

Aims: Coronary microvascular dysfunction (CMD) is related to the pathophysiology, mortality, and morbidity of heart failure with preserved ejection fraction (HFpEF). A novel single-photon emission computed tomography (SPECT) camera with cadmium zinc telluride (CZT) detectors allows for the quantification of absolute myocardial blood flow and myocardial flow reserve (MFR) in patients with coronary artery disease. However, the potential of CZT-SPECT assessing for CMD has never been evaluated in patients with HFpEF. Methods and results: The clinical records of 127 consecutive patients who underwent dynamic CZT-SPECT were retrospectively reviewed. Rest and stress scanning were started simultaneously with 3 and 9 MBq/kg of 99mTc-sestamibi administration, respectively. Dynamic CZT-SPECT imaging data were analysed using a net-retention model with commercially available software. Transthoracic echocardiography was performed in all patients. The MFR value was significantly lower in the HFpEF group (mean ± SEM = 2.00 ± 0.097) than that in the non-HFpEF group (mean ± SEM = 2.74 ± 0.14, P = 0.0004). A receiver operating characteristic analysis indicated that if a cut-off value of 2.525 was applied, MFR could efficiently distinguish HFpEF from non-HFpEF. Heart failure with preserved ejection fraction had a consistently low MFR, regardless of the diastolic dysfunction score. Heart failure with preserved ejection fraction patients with MFR values lower than 2.075 had a significantly higher incidence of heart failure exacerbation. Conclusion: Myocardial flow reserve assessed by CZT-SPECT was significantly reduced in patients with HFpEF. A lower MFR was associated with a higher hospitalization rate in these patients. Myocardial flow reserve assessed by CZT-SPECT has the potential to predict future adverse events and stratify the severity of disease in patients with HFpEF.

14.
Case Rep Transplant ; 2023: 9075184, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-36968338

RESUMO

Recently, magnetic resonance imaging (MRI) has been developed as a widely available and noninvasive method for detecting and evaluating hepatic iron overload. This case report presents a successful living donor liver transplantation (LDLT) in which the donor was suspected to have hepatic iron deposition by MRI evaluation. A preoperative donor liver biopsy and genetic examination were performed to exclude hereditary hemochromatosis and other chronic liver diseases. A liver biopsy showed an almost normal liver specimen with a slight deposition of iron in 2-3% of hepatocytes, and a genetic examination of hereditary hemochromatosis revealed no typical mutations in HFE, TFR2, HJV, HAMP, or SLC40A1. Despite the traumatic hemothorax complication caused by the liver biopsy, the liver transplant eligibility was confirmed. Two months after the hemothorax complication, an LDLT donor operation was performed. The donor was discharged from the hospital on postoperative day (POD) #17 with favorable liver function. The recipient's posttransplant clinical course was generally favorable except for acute cellular rejection and biliary complications, and the recipient was discharged from the hospital on POD #87 with excellent graft function. A one-year follow-up liver biopsy of the recipient demonstrated almost normal liver with iron deposition in less than 1% of the hepatocytes, and no iron deposition was identified in the liver graft by MRI examination. Liver biopsy and genetic examination are effective methods to evaluate the eligibility of liver transplant donors with suspected hepatic iron deposition. The living donor with slight hepatic iron deposition, if hereditary hemochromatosis was ruled out, can donate partial liver safely.

15.
Exp Clin Transplant ; 20(10): 954-958, 2022 10.
Artigo em Inglês | MEDLINE | ID: mdl-36409054

RESUMO

Erythropoietic protoporphyria is a rare inherited metabolic disorder involving the heme biosynthesis pathway and leads to the accumulation of protoporphyrin in the erythrocytes or liver. Although peripheral neuropathy is known to develop occasionally in other types of porphyria, it rarely occurs in patients with erythropoietic protoporphyria. A 16-year-old boy was transferred to our hospital due to end-stage liver disease secondary to erythropoietic protoporphyria. Severe systemic peripheral neuropathy, similar to that presented in Guillain-Barré syndrome, developed; it was promptly managed with mechanical ventilation. Electrophysiological assessment of the presented neuropathy showed no responsiveness, indicating severe axonopathy. Six weeks after the transfer, liver transplant was performed.Postoperatively, hepatorenal syndromes improved immediately, and his erythrocyte protoporphyrin level decreased from 6291 to 174 µg/dL red blood cells.The patient started to move his limbs gradually and was weaned from mechanical ventilation 2 months after liver transplant. Eventually, he was discharged from hospital and was able to ambulate with assistance 10 months after liver transplant. To our knowledge, this is the first report detailing the clinical course in a patient with erythropoietic protoporphyria who recovered from severe systemic peripheral neuropathy after liver transplant.


Assuntos
Transplante de Fígado , Doenças do Sistema Nervoso Periférico , Protoporfiria Eritropoética , Humanos , Masculino , Adolescente , Protoporfiria Eritropoética/complicações , Protoporfiria Eritropoética/diagnóstico , Protoporfiria Eritropoética/cirurgia , Transplante de Fígado/efeitos adversos , Protoporfirinas/metabolismo , Resultado do Tratamento , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/etiologia , Doenças do Sistema Nervoso Periférico/cirurgia
16.
Prev Med Rep ; 28: 101844, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-35637895

RESUMO

Several studies have been reported that sleep duration and circadian rhythms are associated with metabolic syndrome (MetS). However, there are few studies of a relationship between sleep and MetS based on subjective evaluation of sleep regularity. The aim of this study is to examine the relationship between subjective sleep irregularity and metabolic syndrome. This cross-sectional study included 3,880 participants (1,383 males, 2,497 females) from 2013 to 2017, and we use a self-administered questionnaire to acquire information about sleep (sleep regularity, duration and bedtime). Odds ratios (ORs) and 95% confidence intervals (CIs) were calculated using logistic regression analyses to evaluate the associations between sleep regularity and the prevalence of MetS. The irregularity of sleep was significantly associated with MetS (OR 1.231, 95% CI 1.101-1.375) adjusted for age, sex, METs, sleep duration, bedtime, drinking and smoking statuses, and a history of using sleeping pills. We examined the interaction of MetS with sleep regularity and sleep duration/bedtime, stratified by multiplying the two groups of sleep regularity/irregularity and the three groups of sleep duration/bedtime. Each group of sleep duration/bedtime showed no relationship in the sleep regularity group with MetS, but a significant relationship in the sleep irregularity group. Leptin was significantly elevated in the irregular sleep group regardless of sleep duration and bedtime. Although many studies have shown a link between sleep and MetS especially in terms of sleep duration, this study showed that irregular sleep is more strongly associated with MetS than sleep duration or bedtime.

17.
Transplant Proc ; 54(3): 749-754, 2022 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-35256203

RESUMO

BACKGROUND: Early mobilization after liver transplant (LT) plays an important role in postoperative recovery and complication prevention; however, patients undergoing LT cannot achieve early mobilization because of mechanical ventilation and poor preoperative physical performance. We investigated the effect of neuromuscular electrical stimulation (NMES) on lower limb muscle strength after living donor liver transplant (LDLT). METHODS: Adult patients who underwent LDLT between December 2016 and January 2019 at a university hospital were recruited. A consecutive series of patients who underwent LDLT without NMES therapy before the clinical trial (April 2014-May 2016) were enrolled as the non-NMES (control) group. Patients in the NMES group received NMES on the quadriceps muscles starting 1 day post LDLT for 4 weeks. The study was conducted in accordance with the Declaration of Helsinki, and all patients provided informed consent. RESULTS: Twenty-four patients in the NMES group and 16 patients in the non-NMES group were analyzed. There was no significant difference between groups regarding changes in any outcome. CONCLUSIONS: The application of NMES in patients with LDLT did not yield greater improvement of muscle strength, functional capacity, activities of daily living, or length of hospital stay 4 weeks postoperatively compared with the control group. However, developing a novel NMES device and confirming whether additional NMES is effective for other body areas may yield different results.


Assuntos
Transplante de Fígado , Atividades Cotidianas , Adulto , Estudos de Casos e Controles , Estimulação Elétrica , Humanos , Transplante de Fígado/efeitos adversos , Doadores Vivos , Extremidade Inferior , Força Muscular/fisiologia
18.
BMC Gastroenterol ; 21(1): 449, 2021 Nov 29.
Artigo em Inglês | MEDLINE | ID: mdl-34844561

RESUMO

BACKGROUND: There are long-standing controversies about the transplant indications for alcoholic liver disease (ALD), because of the recognition that ALD is fundamentally self-inflicted. However, it is unclear whether psychosocial characteristics of ALD are different from that of non-alcoholic liver disease (NALD) in the selection of liver transplantation (LT) recipients. We aimed to clarify the psychosocial characteristics of ALD recipients (ALD-R)/ALD recipient candidates (ALD-RC) and NALD recipients (NALD-R)/ NALD recipient candidates (NALD-RC). METHODS: From 2011 to 2019, 75 patients were enrolled in this prospective observational study (ALD-RC, n = 19; NALD-RC, n = 56), LT were carried out as follow; ALD-R, n = 6; NALD-R, n = 52. We evaluated psychosocial characteristics in the preoperative period and 3, 12 months after LT (ALD-R, n = 3/3; NALD-R, n = 28/25). The following scales were used to evaluate psychosocial characteristics: Visual Analogue Scale, Alcohol Use Disorders Identification Test, Hospital Anxiety and Depression Scale, Beck Depression Inventory, Brief Evaluation of Medication Influences and Beliefs, Social Support Questionnaire (SSQ), Temperament and Character Inventory, Parental Bonding Instrument (PBI), the Short Form Health Survey (SF-36). RESULTS: When evaluating on the basis of abstinence rule, a comparison of ALD-RC and NALD-RC in the preoperative period identified similar patterns of psychosocial characteristics, except that the NALD-RC scored higher on the PBI item "overprotection from mother" (P < 0.05). The only significant difference between ALD-R and NALD-R after liver transplantation was in SSQ scores at 3 months. CONCLUSION: The psychosocial characteristics of ALD-RC and NALD-RC may be similar when evaluated on the basis of Japan's abstinence rule. This result also imply that the psychosocial characteristics of ALD-RC may differ from the previously reported psychosocial characteristics of alcohol dependent patients. These findings have the potential to provide helpful information for the evaluation of ALD-RC.


Assuntos
Alcoolismo , Hepatopatias Alcoólicas , Transplante de Fígado , Humanos , Hepatopatias Alcoólicas/cirurgia , Estudos Prospectivos , Recidiva
19.
Transpl Infect Dis ; 23(5): e13731, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-34500501

RESUMO

BACKGROUND: Chronic high Epstein-Barr virus loads (CHEBV) are commonly observed in pediatric liver transplant patients. However, it is unclear how CHEBV impacts the liver graft. The aim of this study was to clarify the clinical and pathological impacts of CHEBV on the liver graft. METHODS: From 2012 to 2020, we retrospectively investigated 46 pediatric liver transplant patients (under 16 years) who survived ≥6 months. The patients were divided into two groups: CHEBV group (EBV DNA >10 000 IU/ml of whole blood for ≥6 months) and nonchronic high EBV (NCHEBV) group (patients who did not meet CHEBV criteria). Tacrolimus was reduced to <3.0 ng/ml in patients with EBV DNA >5000 IU/ml. Blood biochemistry data and pathological findings, obtained at the time of protocol and episodic biopsies, were compared between the two groups. RESULTS: Out of 46 patients, 28 CHEBV and 18 NCHEBV patients were enrolled. The blood biochemical examination did not show a significant difference between the two groups. In addition, no significant differences between the two groups were found in the pathological findings, including frequency of late acute rejection and the progression of fibrosis at the time of both protocol and episodic biopsies. Appropriate adjustment of immunosuppression for CHEBV management may have contributed to the prevention of the progression of fibrosis. CONCLUSION: CHEBV had little adverse effect on the liver graft. Graft fibrosis might have been avoided through optimal dose modification of tacrolimus. Further long-term monitoring is necessary because CHEBV may affect the pediatric liver graft in the long term.


Assuntos
Infecções por Vírus Epstein-Barr , Transplante de Fígado , Transtornos Linfoproliferativos , Criança , Infecções por Vírus Epstein-Barr/epidemiologia , Rejeição de Enxerto/epidemiologia , Rejeição de Enxerto/prevenção & controle , Herpesvirus Humano 4 , Humanos , Imunossupressores/efeitos adversos , Fígado , Transplante de Fígado/efeitos adversos , Estudos Retrospectivos
20.
Exp Clin Transplant ; 19(9): 935-942, 2021 09.
Artigo em Inglês | MEDLINE | ID: mdl-34545778

RESUMO

OBJECTIVES: In this study, we revisited the reasons for poorer prognosis afterlivertransplantin patients with hepatitis C virus, whose main causes of death were generally known to be recurrent disease. MATERIALS AND METHODS: Between April 2003 and March 2017, among 132 patients who underwent liver transplantbecause ofliver cirrhosis at ourinstitution, 40 patients (30.3%) were positive for hepatis C virus. We retrospectively compared the overall survival afterliver transplantin patients with and without hepatitis C virus infection. Furthermore, we investigated the causes of death in transplant recipients with hepatitis C virus infections. RESULTS: In patients with hepatitis C virus infection, overall survivalwas 82.2%, 75.2%, and50.8% at 1, 5, and 10 years,respectively, afterlivertransplant;these results were lower than those in patients without infection (94.5%, 87.0%, and 87.0% at 1, 5, and 10 years, respectively; P = .001). Among 40 patients with positive infection, 14 patients died after liver transplant. A main reason for death was hepatocellular carcinoma recurrence (3 patients). Surprisingly, only 1 patient died from hepatitis C virus-related complication (fibrosing cholestatic hepatitis); the remaining 10 patients died from reasons other than hepatitis C virus disease progression. CONCLUSIONS: Our results suggest that clinicians should not only be aware of hepatitis C virus recurrence but should also be aware of other unrelated complications in transplant recipients who are positive for this virus.


Assuntos
Hepatite C , Neoplasias Hepáticas , Transplante de Fígado , Hepacivirus , Hepatite C/complicações , Hepatite C/diagnóstico , Humanos , Cirrose Hepática/diagnóstico , Cirrose Hepática/etiologia , Cirrose Hepática/cirurgia , Transplante de Fígado/efeitos adversos , Recidiva Local de Neoplasia/complicações , Prognóstico , Recidiva , Estudos Retrospectivos , Resultado do Tratamento
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