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Adequate portal vein flow after liver transplantation.

Fri, 12/15/2017 - 13:45

Adequate portal vein flow after liver transplantation.

Liver Transpl. 2017 Dec 14;:

Authors: Gastaca M, Prieto M, Palomares I, Valdivieso A

PMID: 29240285 [PubMed - as supplied by publisher]

Successful Removal of Intracardiac Thrombus With a Poole Tip Suction Device Through the Inferior Vena Cava of a Patient With Cardiovascular Collapse During Liver Transplant: A Case Report.

Fri, 12/15/2017 - 13:45

Successful Removal of Intracardiac Thrombus With a Poole Tip Suction Device Through the Inferior Vena Cava of a Patient With Cardiovascular Collapse During Liver Transplant: A Case Report.

A A Case Rep. 2017 Dec 11;:

Authors: Vogt MNP, Amundson AW, Heimbach JK, Martin DP

Abstract
Intracardiac thrombus occurs in 1.2%-6.3% of patients undergoing orthotopic liver transplant and is associated with a high mortality rate. The pathophysiology and risk factors for development of this complication are not well understood. No consensus treatment guidelines exist, and specific therapies are associated with serious risks. We present the timely and successful use of a Poole tip surgical suction device advanced into the right atrium through a cavotomy created in the inferior vena cava to remove a large right atrial thrombus during liver transplant. The thrombus was identified with transesophageal echocardiography and was causing cardiovascular collapse.

PMID: 29240017 [PubMed - as supplied by publisher]

Maternal liver transplant: another cause of discordant fetal sex determination using cell free DNA.

Fri, 12/15/2017 - 13:45

Maternal liver transplant: another cause of discordant fetal sex determination using cell free DNA.

Prenat Diagn. 2017 Dec 14;:

Authors: Neofytou M, Brison N, Van den Bogaert K, Dehaspe L, Devriendt K, Geerts A, Vermeesch JR

Abstract
NIPT can very accurately determine fetal sex during pregnancy. We present an exceptional case where NIPT contradicts the ultrasound based sex determination. The pregnant woman was recipient of a liver transplant from a male donor. Graft-derived cell-free DNA released into the maternal circulation clouded the NIPT based sex determination. Hence, NIPT is not advisable when the pregnant mother underwent an organ transplant.

PMID: 29239474 [PubMed - as supplied by publisher]

Outcomes of Salvage Liver Transplantation and Re-resection/Radiofrequency Ablation for Intrahepatic Recurrent Hepatocellular Carcinoma: A New Surgical Strategy Based on Recurrence Pattern.

Fri, 12/15/2017 - 13:45
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Outcomes of Salvage Liver Transplantation and Re-resection/Radiofrequency Ablation for Intrahepatic Recurrent Hepatocellular Carcinoma: A New Surgical Strategy Based on Recurrence Pattern.

Dig Dis Sci. 2017 Dec 14;:

Authors: Zhang X, Li C, Wen T, Peng W, Yan L, Yang J

Abstract
BACKGROUND: The treatment of intrahepatic recurrent hepatocellular carcinoma (HCC) has been poorly investigated, and the optimal treatment strategy remains unclear.
AIMS: The aim of this study was to compare outcomes between salvage liver transplantation (SLT) and re-resection (RR)/radiofrequency ablation (RFA) for intrahepatic recurrent HCC according to recurrence pattern.
METHODS: Based on postoperative histopathological examination, 122 patients with intrahepatic recurrent HCC were divided into an intrahepatic metastasis (IM, n = 75) group and a multicentric occurrence (MO, n = 47) group. The demographic, clinical, and primary and recurrent tumor characteristics of the IM group and the MO group were collected and compared. Overall survival (OS) and disease-free survival (DFS) were analyzed, and subgroup analysis according to retreatment type (SLT vs. RR/RFA) was conducted. Twenty-nine clinicopathological variables potentially related to prognostic factors affecting survival were analyzed using a Cox proportional hazard model.
RESULTS: The patients that received SLT treatment exhibited favorable DFS compared to patients that received RR/RFA (P = 0.002). OS (P < 0.001) and DFS (P = 0.008) rates were significantly increased in the MO group compared with in the IM group. Subgroup analysis revealed that DFS was significantly improved for patients in the MO group treated with SLT compared to patients treated with RR/RFA (P = 0.017). Recurrence pattern was an independent prognostic factor for both OS [hazard ratio (HR) = 0.093, 95% confidence interval (CI): 0.026-0.337, P < 0.001] and DFS (HR = 0.318, 95% CI: 0.125-0.810, P = 0.016; HR = 3.334, 95% CI: 1.546-7.18, P = 0.002).
CONCLUSIONS: For patients with intrahepatic recurrent HCC, an MO recurrence pattern is associated with better long-term outcomes than the IM pattern. SLT is the preferred option for intrahepatic recurrent HCC, especially for MO cases.

PMID: 29238896 [PubMed - as supplied by publisher]

Cryptogenic cholestasis in young and adults: ATP8B1, ABCB11, ABCB4, and TJP2 gene variants analysis by high-throughput sequencing.

Fri, 12/15/2017 - 13:45
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Cryptogenic cholestasis in young and adults: ATP8B1, ABCB11, ABCB4, and TJP2 gene variants analysis by high-throughput sequencing.

J Gastroenterol. 2017 Dec 13;:

Authors: Vitale G, Gitto S, Raimondi F, Mattiaccio A, Mantovani V, Vukotic R, D'Errico A, Seri M, Russell RB, Andreone P

Abstract
BACKGROUND: Mutations in ATP-transporters ATPB81, ABCB11, and ABCB4 are responsible for progressive familial intrahepatic cholestasis (PFIC) 1, 2 and 3, and recently the gene for tight junction protein-2 (TJP2) has been linked to PFIC4.
AIM: As these four genes have been poorly studied in young people and adults, we investigated them in this context here.
METHODS: In patients with cryptogenic cholestasis, we analyzed the presence of mutations by high-throughput sequencing. Bioinformatics analyses were performed for mechanistic and functional predictions of their consequences on biomolecular interaction interfaces.
RESULTS: Of 108 patients, 48 whose cause of cholestasis was not established were submitted to molecular analysis. Pathogenic/likely pathogenic mutations were found in ten (21%) probands for 13 mutations: two in ATP8B 1, six in ABCB11, two in ABCB4, three in TJP2. We also identified seven variants of uncertain significance: two in ATP8B1, one in ABCB11, two in ABCB4 and two in TJP2. Finally, we identified 11 benign/likely benign variants. Patients with pathogenic/likely pathogenic mutations had higher levels of liver stiffness (measured by FibroScan®) and bile acids, as well as higher rates of cholestatic histological features, compared to the patients without at least likely pathogenic mutations. The multivariate analysis showed that itching was the only independent factor associated with disease-causing mutations (OR 5.801, 95% CI 1.244-27.060, p = 0.025).
CONCLUSIONS: Mutations in the genes responsible for PFIC may be involved in both young and adults with cryptogenic cholestasis in a considerable number of cases, including in heterozygous status. Diagnosis should always be suspected, particularly in the presence of itching.

PMID: 29238877 [PubMed - as supplied by publisher]

Laparoscopy-Assisted versus Open Hepatectomy for Live Liver Donor: Systematic Review and Meta-Analysis.

Fri, 12/15/2017 - 13:45
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Laparoscopy-Assisted versus Open Hepatectomy for Live Liver Donor: Systematic Review and Meta-Analysis.

Can J Gastroenterol Hepatol. 2017;2017:2956749

Authors: Zhang B, Pan Y, Chen K, Maher H, Chen MY, Zhu HP, Zhu YB, Dai Y, Chen J, Cai XJ

Abstract
Objective: To assess the feasibility, safety, and potential benefits of laparoscopy-assisted living donor hepatectomy (LADH) in comparison with open living donor hepatectomy (ODH) for liver transplantation.
Background: LADH is becoming increasingly common for living donor liver transplant around the world. We aim to determine the efficacy of LADH and compare it with ODH.
Methods: A systematic search on PubMed, Embase, Cochrane Library, and Web of Science was conducted in May 2017.
Results: Nine studies were suitable for this analysis, involving 979 patients. LADH seemed to be associated with increased operation time (WMD = 24.85 min; 95% CI: -3.01~52.78, P = 0.08), less intraoperative blood loss (WMD = -59.92 ml; 95% CI: -94.58~-25.27, P = 0.0007), similar hospital stays (WMD = -0.47 d; 95% CI: -1.78~0.83, P = 0.47), less postoperative complications (RR = 0.70, 95% CI: 0.51~0.94, P = 0.02), less analgesic use (SMD = -0.22; 95% CI: -0.44~-0.11, P = 0.04), similar transfusion rates (RR = 0.82; 95% CI: 0.24~3.12, P = 0.82), and similar graft weights (WMD = 7.31 g; 95% CI: -23.45~38.07, P = 0.64).
Conclusion: Our results indicate that LADH is a safe and effective technique and, when compared to ODH.

PMID: 29238704 [PubMed - in process]

The role of TIPS in the management of liver transplant candidates.

Fri, 12/15/2017 - 13:45
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The role of TIPS in the management of liver transplant candidates.

United European Gastroenterol J. 2017 Dec;5(8):1100-1107

Authors: Unger LW, Stork T, Bucsics T, Rasoul-Rockenschaub S, Staufer K, Trauner M, Maschke S, Pawloff M, Soliman T, Reiberger T, Berlakovich GA

Abstract
Background: Transjugular intrahepatic portosystemic shunt (TIPS) implantation is used for treatment of several complications in patients with liver cirrhosis. Recent studies have identified a survival benefit for patients on the waiting list after TIPS implantation, but the optimal time point for TIPS implantation prior to orthotopic liver transplantation (OLT) has not been established.
Study: This study retrospectively assessed patients undergoing TIPS implantation before or after listing for OLT at the Medical University of Vienna. n = 98 patients with TIPS on the waiting list between January 1993 and December 2013 were identified (n = 73 (74.5%) pre-listing TIPS, n = 25 (25.5%) post-listing TIPS). A matched control group at the time of OLT without TIPS (n = 60) was included.
Results: More patients with post-listing TIPS (28.0%, 7/25) showed clinical improvement and went off-list than patients with pre-listing TIPS (8.2%, 6/73, p = .0119). A similar proportion of patients with pre-listing TIPS (19.2%, 14/73) and post-listing TIPS (20.0%, 5/25) died on the OLT waiting list. Transplant surgery time was similar in patients with and without TIPS: 348(±13) vs. 337(±10) minutes (p = .5139). Estimated 1-year post-transplant survival was similar across all groups (pre-listing TIPS: 76.2%, post-listing TIPS: 86.0%, no TIPS: 91.2%, log-rank p = .1506).
Conclusion: TIPS should be considered in all liver transplant candidates, since it can obviate the need for OLT and optimize bridging to OLT.

PMID: 29238588 [PubMed]

Persistent hypocalcemia and hungry bone syndrome after parathyroidectomy and renal transplantation in a patient with end-stage renal disease.

Fri, 12/15/2017 - 13:45
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Persistent hypocalcemia and hungry bone syndrome after parathyroidectomy and renal transplantation in a patient with end-stage renal disease.

Niger Med J. 2017 Jan-Feb;58(1):50-52

Authors: Tayyebi-Khosroshahi H, Farnood F, Ghorbanian M, Karkon-Shayan F, Naghavi-Behzad M

Abstract
Hungry bone syndrome (HBS) defines as persistent and severe hypocalcemia after parathyroidectomy surgery. It is treated by oral or venous discrimination of calcium carbonate. The present treatment is mostly effective. Hereby, we describe a 60-year-old man who had developed hyperparathyroidism secondary to end-stage renal disease and then parathyroidectomy was performed for him twice before renal transplantation. Up to 500 vials of calcium gluconate (100 mg/ml calcium gluconate 10%) were administered for him to control serum calcium level after parathyroidectomy and renal transplantation. Furthermore, high-dose calcium carbonate was administered for his outpatient care. Therefore, HBS, which was resistant to standard treatment, was detected for him.

PMID: 29238129 [PubMed - in process]

Direct-acting agents for hepatitis C virus before and after liver transplantation.

Fri, 12/15/2017 - 13:45
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Direct-acting agents for hepatitis C virus before and after liver transplantation.

Biosci Trends. 2017 Dec 14;:

Authors: Sugawara Y, Hibi T

Abstract
Chronic hepatitis C virus (HCV) infection remains a widespread public health concern and many people are infected with HCV. HCV is one of the leading indications for liver transplantation. Direct-acting antiviral agents (DAAs) against HCV have changed the course of chronic HCV infection, however, making it a curable disease. DAA treatment may be initiated before or after liver transplantation. In the present review, we present the available data on DAA treatment of HCV in liver transplant recipients.

PMID: 29238003 [PubMed - as supplied by publisher]

Human fetal liver cultures support multiple cell lineages that can engraft immunodeficient mice.

Fri, 12/15/2017 - 13:45
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Human fetal liver cultures support multiple cell lineages that can engraft immunodeficient mice.

Open Biol. 2017 Dec;7(12):

Authors: Fomin ME, Beyer AI, Muench MO

Abstract
During prenatal development the liver is composed of multiple cell types with unique properties compared to their adult counterparts. We aimed to establish multilineage cultures of human fetal liver cells that could maintain stem cell and progenitor populations found in the developing liver. An aim of this study was to test if maturation of fetal hepatocytes in short-term cultures supported by epidermal growth factor and oncostatin M can improve their ability to engraft immunodeficient mice. Fetal liver cultures supported a mixture of albumin+ cytokertin-19+ hepatoblasts, hepatocytes, cholangiocytes, CD14++CD32+ liver sinusoidal endothelial cells (LSECs) and CD34+CD133+ haematopoietic stem cells. Transplantation of cultured cells into uPA-NOG or TK-NOG mice yielded long-term engraftment of hepatocytes, abundant LSEC engraftment and multilineage haematopoiesis. Haematopoietic engraftment included reconstitution of B-, T- and NK-lymphocytes. Colonies of polarized human hepatocytes were observed surrounded by human LSECs in contact with human CD45+ blood cells in the liver sinusoids. Thus, fetal liver cultures support multiple cell lineages including LSECs and haematopoietic stem cells while also promoting the ability of fetal hepatocytes to engraft adult mouse livers. Fetal liver cultures and liver-humanized mice created from these cultures can provide useful model systems to study liver development, function and disease.

PMID: 29237808 [PubMed - in process]

Long-term follow-up of hepatitis-associated aplastic anaemia.

Fri, 12/15/2017 - 13:45
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Long-term follow-up of hepatitis-associated aplastic anaemia.

BMJ Case Rep. 2017 Dec 13;2017:

Authors: Gonçalves C, Ferreira S, Nobre S, Gonçalves I

Abstract
Prognosis of hepatitis-associated aplastic anaemia (HAAA) was improved with haematopoietic stem cell transplantation (HSCT) and immunosuppression, but the long-term outcome remains undefined. Case 1: a girl aged 3 years with acute liver failure (ALF) submitted to orthotopic liver transplantation (OLT) subsequently developed aplastic anaemia and HSCT from a compatible sibling was performed. Post-HSCT, the patient developed post-transplant lymphoproliferative disorder and rituximab was administered with good response. Fifteen years later, both grafts show good outcome. Case 2: a girl aged 10 years submitted to OLT due to ALF, developed pancytopenia 2 months later. Due to the absence of a human leucocyte antigen compatible donor, she was treated with ciclosporin and antithymocyte globulin with very good long-term outcome. These clinical cases suggest that, for patients with HAAA that underwent OLT, aggressive therapy with HSCT or immunosuppression may provide a benign long-term outcome.

PMID: 29237659 [PubMed - in process]

Crigler-Najjar syndrome: current perspectives and the application of clinical genetics.

Fri, 12/15/2017 - 13:45
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Crigler-Najjar syndrome: current perspectives and the application of clinical genetics.

Endocr Metab Immune Disord Drug Targets. 2017 Dec 13;:

Authors: Ebrahimi A, Rahim F

Abstract
Crigler-Najjar syndrome (CNS, OMIM: 218800) is the paradigm of an inborn error of metabolism and a rare genetic disease with an estimated incidence of 0.6-1.0 per million live births. Discrimination between CNS subtypes is usually done on the basis of the clinical criteria, such as response to phenobarbital treatment and other molecular and functional characteristics. The identification of four novel pathogenic mutations and the analysis of residual activity of missense in UGT1A1 gene are useful for clinical diagnosis, and may reveal a new insight in enzyme activity, whereas the identification of pathogenic mutations will accelerate genetic counseling for newly identified CNS patients. Phototherapy, orthotropic liver transplantation, liver cell transplantation and gene therapy are treatment choices and candidates to fight back this syndrome. Due to the promising reports of gene therapy in small animal models, gene therapy approaches are expected to continue in preclinical research for developing safe and effective treatment of CNS. Gene transfer vectors using recombinant viruses, such as Adenovirus have been applied successfully in transferring UGT1A1 gene to the liver of Gunn rat model of CNS. In spite of remaining safety and efficiency issues, gene therapy promises to be a realistic treatment modality for CNS during the future decade.

PMID: 29237388 [PubMed - as supplied by publisher]

De Novo Malignancies After Liver Transplantation: A Single Institution Experience.

Fri, 12/15/2017 - 13:45
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De Novo Malignancies After Liver Transplantation: A Single Institution Experience.

Exp Clin Transplant. 2017 Dec 14;:

Authors: Egeli T, Unek T, Ozbilgin M, Agalar C, Derici S, Akarsu M, Unek IT, Aysin M, Bacakoglu A, Astarcıoglu I

Abstract
OBJECTIVES: Our objective was to analyze characteristics, risk factors, and incidence of de novo malignancies after liver transplant.
MATERIALS AND METHODS: The hospital records of 557 patients who underwent liver transplant were analyzed from the point of de novo malignancy development. We evaluated the demographic features and survival of these patients retrospectively.
RESULTS: The research covered 429 patients, 9 (2%) of whom developed de novo malignancy. All of these patients were male (100%), and their mean (SD) age was 51.33 (4.69) years (range, 45-65 y). Indications for transplant included alcohol related in 4 cases, chronic hepatitis B in 2 cases, chronic hepatitis B and C in 1 case, chronic hepatitis B and D in 1 case, and chronic hepatitis C and alcohol-related cirrhosis in 1 case. The mean (SD) time from transplant to cancer diagnosis was 63.41 (37.10) months (range, 17-122 mo). The types of tumors were lung cancer, lymphoma, neuroendocrine tumor of lung, nasopharyngeal cancer, and squamous cell carcinoma of the skin. Seven cases received chemotherapy with or without radiotherapy. Two cases received surgery and radiotherapy. One patient underwent surgical treatment. One patient died before treatment was started.
CONCLUSIONS: In recent years, improvements in surgical techniques and immunosuppressive therapies have helped prolong survival of patients who undergo liver transplant. However, this also has led to a rise in the incidence of long-term complications such as de novo malignancy. These patients are more likely to develop de novo malignancy than the general population, for which chronic immunosuppression is identified as a major risk factor. Early diagnosis and treatment of de novo malignancies can help obtain better prognosis and higher survival rates in these patients.

PMID: 29237362 [PubMed - as supplied by publisher]

Preoperative Prediction of Microvascular Invasion in Hepatocellular Carcinoma Using Quantitative Image Analysis.

Fri, 12/15/2017 - 13:45
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Preoperative Prediction of Microvascular Invasion in Hepatocellular Carcinoma Using Quantitative Image Analysis.

J Am Coll Surg. 2017 Dec;225(6):778-788.e1

Authors: Zheng J, Chakraborty J, Chapman WC, Gerst S, Gonen M, Pak LM, Jarnagin WR, DeMatteo RP, Do RKG, Simpson AL, Hepatopancreatobiliary Service in the Department of Surgery of the Memorial Sloan Kettering Cancer Center, Research Staff in the Department of Surgery at Washington University School of Medicine

Abstract
BACKGROUND: Microvascular invasion (MVI) is a significant risk factor for early recurrence after resection or transplantation for hepatocellular carcinoma (HCC). Knowledge of MVI status would help guide treatment recommendations, but is generally identified after operation. This study aims to predict MVI preoperatively using quantitative image analysis.
STUDY DESIGN: One hundred and twenty patients from 2 institutions underwent resection of HCC from 2003 to 2015 were included. The largest tumor from preoperative CT was subjected to quantitative image analysis, which uses an automated computer algorithm to capture regional variation in CT enhancement patterns. Quantitative imaging features by automatic analysis, qualitative radiographic descriptors by 2 radiologists, and preoperative clinical variables were included in multivariate analysis to predict histologic MVI.
RESULTS: Histologic MVI was identified in 19 (37%) patients with tumors ≤5 cm and 34 (49%) patients with tumors >5 cm. Among patients with tumors ≤5 cm, none of the clinical findings or radiographic descriptors were associated with MVI; however, quantitative features based on angle co-occurrence matrix predicted MVI with an area under curve of 0.80, positive predictive value of 63%, and negative predictive value of 85%. In patients with tumors >5 cm, higher α-fetoprotein level, larger tumor size, and viral hepatitis history were associated with MVI, and radiographic descriptors were not. However, a multivariate model combining α-fetoprotein, tumor size, hepatitis status, and quantitative feature based on local binary pattern predicted MVI with area under curve of 0.88, positive predictive value of 72%, and negative predictive value of 96%.
CONCLUSIONS: This study reveals the potential importance of quantitative image analysis as a predictor of MVI.

PMID: 28941728 [PubMed - indexed for MEDLINE]

Efficacy and safety of everolimus with reduced tacrolimus in living-donor liver transplant recipients: 12-month results of a randomized multicenter study.

Thu, 12/14/2017 - 13:45

Efficacy and safety of everolimus with reduced tacrolimus in living-donor liver transplant recipients: 12-month results of a randomized multicenter study.

Am J Transplant. 2017 Dec 13;:

Authors: Jeng LB, Lee SG, Soin AS, Lee WC, Suh KS, Joo DJ, Uemoto S, Joh J, Yoshizumi T, Yang HR, Song GW, Lopez P, Kochuparampil J, Sips C, Kaneko S, Levy G

Abstract
In a multicenter, open-label, study, 284 living-donor liver transplant patients were randomized at 30±5 days post-transplant to start everolimus+reduced tacrolimus (EVR+rTAC) or continue standard tacrolimus (TAC Control). EVR+rTAC was non-inferior to TAC Control for the primary efficacy endpoint of treated BPAR, graft loss or death at 12 months post-transplant: difference -0.7% [90%CI -5.2%, 3.7%]; p<0.001 for non-inferiority. tBPAR occurred in 2.2% and 3.6% of patients, respectively. The key secondary endpoint, change in estimated GFR from randomization to month 12, achieved non-inferiority (p<0.001 for non-inferiority), but not superiority and was similar between groups overall (mean -8.0 versus -12.1mL/min/1.73m2 ,p=0.108), and in patients continuing randomized treatment (-8.0 versus -13.3mL/min/1.73m2 ,p=0.046). In the EVR+rTAC and TAC control groups, study drug was discontinued in 15.5% and 17.6%, adverse events with suspected relation to study drug occurred in 57.0% and 40.4%, and proteinuria ≥1 g/24h in 9.3% and 0%, respectively. Everolimus did not negatively affect liver regeneration. At 12 months, hepatocellular recurrence was only seen in the standard TAC-treated patients (5/62; 8.1%). In conclusion, early introduction of EVR+rTAC was non-inferior to standard tacrolimus in terms of efficacy and renal function at 12 months, with hepatocellular carcinoma recurrence only in TAC Control patients. This article is protected by copyright. All rights reserved.

PMID: 29237235 [PubMed - as supplied by publisher]

Transplantation for Alcohol-related Liver Disease: Is It Fair?

Thu, 12/14/2017 - 13:45

Transplantation for Alcohol-related Liver Disease: Is It Fair?

Alcohol Alcohol. 2017 Dec 11;:

Authors: Mellinger JL, Volk ML

Abstract
Aims: Alcohol-related liver disease (ALD) is the second leading cause of liver transplantation performed in the USA and Europe. We aimed to provide a narrative review of the major ethical issues governing transplantation for ALD.
Methods: We performed a narrative review of the ethical concepts in organ allocation for ALD, including alcoholic hepatitis.
Results: Ethical concerns regarding organ allocation for ALD involve issues of urgency, utility and justice. Post-transplant outcomes for ALD patients are good and ethical considerations limiting organs solely because of alcohol etiology do not bear scrutiny.
Conclusion: ALD will continue to be a major cause for liver failure. The main criteria for transplant in ALD should be the patient's risk of return to harmful drinking, alongside standard assessments of physical and psychosocial fitness for transplant.

PMID: 29236944 [PubMed - as supplied by publisher]

Dietary copper restriction in Wilson's disease.

Thu, 12/14/2017 - 13:45

Dietary copper restriction in Wilson's disease.

Eur J Clin Nutr. 2017 Nov 06;:

Authors: Russell K, Gillanders LK, Orr DW, Plank LD

Abstract
Dietary copper restriction has long been considered an important aspect of treatment for Wilson's disease (WD). However, evidence supporting this approach is limited. There are no published randomised controlled trials examining this recommendation due to rarity of the disease and variable presentation. This review summarises current knowledge on the absorption and regulation of copper in humans and its relevance to patients with WD. Studies have demonstrated that as the level of dietary copper increases, the proportion absorbed decreases. This observation implies that 'high copper' foods that WD patients are generally advised to avoid would need to be consumed in large amounts to impact markedly on the quantity absorbed. Dietary copper restriction is unlikely to reduce the amount absorbed significantly and is not only difficult to manage but restricts food groups unnecessarily, detracting from the provision of substrates essential for improving nutritional status in a nutritionally compromised group. Medical management for WD is effective in compliant patients, allowing stabilisation of the liver disease. Based on current evidence, dietary copper restrictions in stable WD patients who are adherent to medical therapy are unnecessary with two food exceptions (shellfish and liver).

PMID: 29235558 [PubMed - as supplied by publisher]

Upregulation of B7-H4 promotes tumor progression of intrahepatic cholangiocarcinoma.

Thu, 12/14/2017 - 13:45

Upregulation of B7-H4 promotes tumor progression of intrahepatic cholangiocarcinoma.

Cell Death Dis. 2017 Dec 13;8(12):3205

Authors: Xie N, Cai JB, Zhang L, Zhang PF, Shen YH, Yang X, Lu JC, Gao DM, Kang Q, Liu LX, Zhang C, Huang XY, Zou H, Zhang XY, Song ZJ, Sun HX, Fu BM, Ke AW, Shi GM

Abstract
Recent reports show that B7-H4 is highly expressed in a variety of tumor cells, functions as a negative regulator of T cells and then promotes tumor progression. However, its expression and role in intrahepatic cholangiocarcinoma (ICC) remain unclear. In present study, B7-H4 expression in ICC and peritumoral tissues was determined at the level of mRNA and protein, and its bioactivity in ICC cells was studied after modification of B7-H4 expression. Then, the mechanism related to tumor progression induced by B7-H4 expression in ICC cells was explored. Finally, clinical significance of B7-H4 expression in ICC patients was further analyzed. The results showed that B7-H4 expression in ICC was much higher than that in peritumoral tissues at the level of both mRNA and protein. The high level of B7-H4 in ICC cells induced epithelial-to-mesenchymal transitions and promoted invasion and metastasis of tumor cells through activation of ERK1/2 signaling. The elevated B7-H4 expression was associated with the downregulated Bax, upregulated Bcl-2 expression, and activation of caspase-3. Clinically, high B7-H4 expression in tumor samples was significantly related to malignant phenotype, such as lymph node metastasis, high tumor stage, and poor differentiation. ICC patients with high expression of B7-H4 had shorter overall survival (OS) and disease-free survival. Moreover, the B7-H4 expression was an independent prognostic factor for predicting OS and tumor recurrence of ICC patients after operation. In conclusion, high expression of B7-H4 promotes tumor progression of ICC and may be a novel therapeutic target for ICC patients.

PMID: 29235470 [PubMed - in process]

Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant.

Thu, 12/14/2017 - 13:45
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Liver transplant for metastatic pancreatoblastoma: 7-year event-free survival after chemotherapy, pancreatectomy, complete hepatectomy, and liver transplant.

Pediatr Transplant. 2017 Dec 13;:

Authors: Ghaffarian AA, Book L, Meyers RL

Abstract
Pancreatoblastoma is a rare malignant tumor in children. Surgical resection of the tumor is necessary for cure; however, due to its aggressive nature, it is often unresectable at presentation due to tumor size, local invasion, and/or metastasis. Because it is a rare tumor, there is currently no standard treatment regimen. We report a case of a 4-year-old boy who presented with metastatic pancreatoblastoma with multiple large metastases involving all four sectors of the liver. We began treatment with chemotherapy (cisplatin, 5FU, vincristine, and doxorubicin), which significantly reduced the tumor burden in both the pancreas and liver. We then performed a staged subtotal pancreatectomy, complete hepatectomy, and living donor left lateral segment liver transplant. This was followed by postoperative adjuvant chemotherapy. Our patient is alive and healthy and has now been tumor-free for 7 years with no tumor relapse.

PMID: 29235221 [PubMed - as supplied by publisher]

Pre-transplant serum procalcitonin level for prediction of early post-transplant sepsis in living donor liver transplantation.

Thu, 12/14/2017 - 13:45
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Pre-transplant serum procalcitonin level for prediction of early post-transplant sepsis in living donor liver transplantation.

Hepatol Res. 2017 Dec 13;:

Authors: Hara T, Soyama A, Hidaka M, Natsuda K, Adachi T, Ono S, Okada S, Hamada T, Takatsuki M, Eguchi S

Abstract
AIM: Infections are frequent causes of in-hospital mortality after liver transplantation (LT). Elimination of possible risks in the pre-transplant period, early diagnosis of post-transplant sepsis, and prompt administration of antimicrobial agents are important. The objectives of this study were to analyze the impact of early post-transplant sepsis on outcomes and to clarify the value of predictive factors for early post-transplant sepsis.
METHODS: The study included 136 patients who underwent initial living donor LT (LDLT) at our institute from April 2009 to December 2016. Sepsis was defined using the third international consensus criteria. The results of biochemical tests at the introduction of anesthesia before LDLT were collected for pre-transplant evaluation.
RESULTS: Post-transplant sepsis was found in 37 patients (27.2%). More patients had a pre-transplant serum procalcitonin (PCT) level >0.5 ng/ml in the sepsis group than in the non-sepsis group (11 [29.7%] vs. 10 [10.1%]; P=0.007). The 1-year survival rates in the sepsis group were significantly lower than those in the non-sepsis group (53.8% vs. 87.2%; P <0.001). Multivariate analysis identified pre-transplant serum PCT >0.5 ng/ml (odds ratio 3.8, 95% confidence interval 1.3-10.9; P=0.01) as the only independent risk factor for post-transplant sepsis.
CONCLUSIONS: Survival of patients with early post-transplant sepsis was poor and the incidence of sepsis was associated with the pre-transplant serum PCT level. Re-evaluation of the general condition and rescheduling of LT are considered in a patient with pre-transplant serum PCT >0.5 ng/ml.

PMID: 29235211 [PubMed - as supplied by publisher]

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