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Objectives: The aim of this study is to present updated data on liver transplantation (LT) for hepatocellular carcinoma (HCC) of Inonu University, Liver Transplantation Institute, one of the largest volume liver transplant centers in the world. Methods: The data of 492 LT patients with HCC were analyzed retrospectively from the databank which is recorded prospectively and sequentially. Post-transplant recurrence rates and patient survival according to Milan, Malatya and Expanded Malatya criteria were calculated. Milan Expansion rate of the Malatya and Expanded Malatya criteria were also calculated. Results: Median follow-up period of the total cohort was 11.1±1.6 years (8.0-14.2, 95% CI) and the recurrence rate was 18.5 % (91/492). 5-year OS according to Milan, Malatya and Expanded Malatya criteria in our cohort were 80%, 79.3% and 78.4%, respectively. Post-transplant recurrence rates within these criteria were 3.2%, 3.8%, and 4.7%, respectively. Milan expansion rates were 25.2% for Malatya criteria and 35.2% for Expanded Malatya criteria. Conclusion: Milan criteria can be expanded reasonably by Expanded Malatya criteria. Low GGT and low AFP are good prognostic biomarkers that predict survival following LT in patients with HCC. Patients within Expanded Malatya Criteria had 78.4% 5-year OS, 4.7% post-transplant recurrence rate and Expanded Malatya criteria expanded the Milan criteria by 35.2%. Thus, 88 patients were beyond Milan criteria and were within Expanded Malatya criteria and so had an opportunity for LT. [ABSTRACT FROM AUTHOR]

Abstract Background Contrast-enhanced magnetic resonance imaging (MRI) plays a crucial role in the diagnosis of hepatocellular carcinoma (HCC). This study aims to assess the performance of MRI features for evaluating hepatocellular carcinoma (HCC) aggressiveness in living liver transplantation in patients. Material and methods This retrospective study included patients who underwent liver transplantation in our hospital between 2015 and 2020. Abdominal contrast-enhanced MRIs of these patients were reviewed, and clinical, radiological, and histopathological findings of HCCs were recorded. The prognostic features of HCCs as determined by MRI were compared with Edmondson-Steiner (E-S) grades. Liver parenchyma fibrosis based on an apparent diffusion coefficient (ADC) map was correlated with histological subclassification of cirrhosis using the Laennec staging system. Results The study subjects included 37 men and 8 women with a mean age of 59.56 ± 7.81 (range: 25–72). The mean tumour size was 37.33 ± 22.27 mm (range: 10–118 mm), and nine tumours (23.1%) involved portal vein tumour thrombosis. There was a significant correlation between tumour grade and size (p = 0.007) and intratumoral fat (p = 0.014) even though no significant correlations between grade and mean ADC value, capsule appearance, presence of satellite lesions, smooth margin, imaging of the tumour feeding artery, and corona enhancement of HCC (p > 0.05) were found. There was a statistically significant correlation between mild (stage 4A) and moderate (stage 4B) fibrosis of non-tumorous liver parenchyma and ADC value (p

Background: Contrast-enhanced magnetic resonance imaging (MRI) plays a crucial role in the diagnosis of hepatocellular carcinoma (HCC). This study aims to assess the performance of MRI features for evaluating hepatocellular carcinoma (HCC) aggressiveness in living liver transplantation in patients. Material and methods: This retrospective study included patients who underwent liver transplantation in our hospital between 2015 and 2020. Abdominal contrast-enhanced MRIs of these patients were reviewed, and clinical, radiological, and histopathological findings of HCCs were recorded. The prognostic features of HCCs as determined by MRI were compared with Edmondson-Steiner (E-S) grades. Liver parenchyma fibrosis based on an apparent diffusion coefficient (ADC) map was correlated with histological subclassification of cirrhosis using the Laennec staging system. Results: The study subjects included 37 men and 8 women with a mean age of 59.56 ± 7.81 (range: 25–72). The mean tumour size was 37.33 ± 22.27 mm (range: 10–118 mm), and nine tumours (23.1%) involved portal vein tumour thrombosis. There was a significant correlation between tumour grade and size (p = 0.007) and intratumoral fat (p = 0.014) even though no significant correlations between grade and mean ADC value, capsule appearance, presence of satellite lesions, smooth margin, imaging of the tumour feeding artery, and corona enhancement of HCC (p > 0.05) were found. There was a statistically significant correlation between mild (stage 4A) and moderate (stage 4B) fibrosis of non-tumorous liver parenchyma and ADC value (p < 0.001). Conclusion: Our study found that ADC values can be used to distinguish mild cirrhotic livers from moderate cirrhotic livers. Diffusion MRI might be used to diagnose the degree of liver fibrosis without histopathological analysis. According to our results, only intralesional fat and tumour size correlated with tumour grade, and as such, these parameters could be used as prognostic MRI biomarkers for HCC. [ABSTRACT FROM AUTHOR]

Background: Lichen planus (LP) is a chronic mucocutaneous inflammatory condition characterized by white striations and plaques or erythematous/erosive lesions in the oral cavity. LP has affected about 0.5 -1% of world population. Apart from the potential of autoimmune origin, the WHO classified oral LP (OLP) as a premalignant condition, with an increased risk of developing squamous cell carcinoma of the head and neck (SCCHN). Patients with LP have exhibited a higher susceptibility to other autoimmune diseases (about 25%). Case Presentation: A 51-year-old woman with a history of OLP and concurrent lupus erythematosus, transplanted Liver, and ulcerative colitis was referred to the Oral and Maxillofacial Medicine Department of Tehran Dental Faculty. A noteworthy point in this study, which was not commonly observed in previous reports, was the existence of OLP with three autoimmune diseases. The lichenoid oral ulcers of the patient were managed by modifying her drug regimen under consultation with a physician using systemic and topical corticosteroids, and, finally, cryotherapy. Conclusions: This case, not the first case of autoimmune diseases, illustrates the management of the challenges of an oral lesion accompanying other systemic diseases and highlights the importance of awareness of the oral manifestations of systemic diseases and their side effects. According to previous studies, the response of oral lesions to different treatments is unique, and the patient’s lifestyle and his/her other systemic diseases influence the treatment success or failure. The patient’s management protocol and its validity can be re-evaluated in future studies as clinical trial projects. [ABSTRACT FROM AUTHOR]

ABSTRACT BACKGROUND: Immunosuppressive drugs have important role in transplant of solid grafts, it aim avoid episodes of acute and chronic rejection and improving graft survival and patient survival. In Brazil, in 2016, liver transplantation was the third most frequent, with 1,880 transplants performed, of which 150 in Rio Grande do Sul. Several studies evaluated the association between variability in blood levels of immunosuppressive tacrolimus and late acute cellular graft rejection. OBJECTIVE: To investigate the association of tacrolimus blood levels with clinical outcomes late acute cellular rejection, death, patient survival and graft survival in patients undergoing liver transplantation. METHODS: This is a retrospective longitudinal study including patients submitted to adult liver transplantation by the Liver Transplantation Group in the Santa Casa de Misericórdia Hospital of Porto Alegre, from January 2006 to January 2013, and who used tacrolimus as immunosuppressive therapy. RESULTS: Of the 127 patients included in the study, the majority were male (70.1%), 52-60 years old (33.9%) at the transplant. The most frequent causes of liver transplantation in this series were hepatitis C virus and hepatocellular carcinoma (24.4%) and alcohol (15.7%). Thirteen patients had late acute cellular rejection (10.2%); of these, three had two episodes. Regarding severity classification, seven patients had mild late acute cellular rejection. The mean time of rejection after liver transplantation was 14 months (ranging from 8 to 33 months). Overall survival was 8.98 years. Regarding tacrolimus blood levels, 52 patients with a variation ≥2 standard deviations were identified. Of these patients, eight had rejection; however, the association was not significant (P=0.146). A significant association was found between variation ≥2 standard deviations in tacrolimus blood levels and death (P=0.023) and survival (P=0.019). Regarding 5-year follow-up of graft survival, being two standard deviations above increases by 2.26 times the risk of transplanted graft loss, and for each unit of increase of standard deviation of tacrolimus blood levels there is a two-fold increase in the risk of graft loss in 5 years. CONCLUSION: Increased risk of graft loss associated with increased standard deviations of tacrolimus blood levels may indicate the need for more rigorous and prospective monitoring of tacrolimus blood levels.

Introduction: Interest in fosfomycin, an old antibiotic, has been reignited because of the use of its intravenous formulation (fosfomycin sodium) in resistant infections. The aim of this study was to screen for carbapenem resistance in Enterobacterales isolates from the bloodstream, and determine the frequency of carbapenamases types and fosfomycin resistance genes in carbapenem-resistant Enterobacterales (CRE) strains. Materials and Methods: Enterobacterales isolates from the bloodstream of liver transplant patients aged 18 years and older were screened for carbapenem resistance between 2017 and 2019. In isolates that were resistant to at least one carbapenem antibiotic were further screened for fosfomycin susceptibility. Carbapenem susceptibility was tested for by the E-test, fosfomycin susceptibility was tested for by the agar dilution methods, and evaluated in accordance with European Committee on Antimicrobial Susceptibility Testing criteria. The frequency of OXA-48, NDM, KPC, VIM, and IMP type carbapenemases and fosA, fosA3, and fosC2 fosfomycin resistance genes were screened for using the polymerase chain reaction method. Results: A total of 115 Enterobacterales isolates from bloodstream infections were obtained. Carbapenem resistance was detected in 34 (29.3%) isolates, 41.2% of them were Escherichia coli and 58.8% of them were Klebsiella pneumoniae. Out of the 34 isolates, 61.8% produced carbapenemases, and OXA-48 was the most common type of carbapenemase. The fosfomycin resistance rate was 73.5%. Among the 34 carbapenem-resistant isolates, the frequency of the fosA gene was 5.9%. The genes fosA3 and fosC2 were not detected in any isolates. FosA + OXA-48 and fosA + NDM genes were detected in CRE isolates. Conclusion: This is the first study reporting on the screening for fosfomycin resistance genes in blood isolates of liver transplant patients in Turkey. Despite high fosfomycin resistance, detection of only two resistance genes reveals that there may be fosfomycin resistance due to other resistance mechanisms.

Background: Nutrition is an integral part of health maintenance. The liver is the largest and most important metabolic organ, playing a pivotal role in integrating several biochemical pathways of carbohydrate, fat, protein, and vitamin metabolism. Malnutrition is a common complication of end- stage liver disease (ESLD). Progressive deterioration of nutritional status has been associated with poor outcome in cirrhotic patients. Liver transplantation (LT) revolutionized the management of liver disease. Nutritional therapy is an integral part of liver transplant care. Objective: To identity the efficacy of different dietary nutritional supplements as zinc, vit D and immunonutrients (glutamine, omega 3, arginine and ribonucleic acid) for recipient at early post operative course. Material and Methods: In this systematic review and meta-analysis we enrolled a total of 7 studies for assessing the clinical outcomes of ICU patients underwent liver transplantation surgery and supplied with dietary supplements during early postoperative period. The studies also assessed the laboratory assessment of patients postoperatively during ICU admission period. Results: Regarding postoperative dietary supplementations with HMB, we found significant difference for appendicular skeletal muscle mass (ASMI) regarding the baseline and the postoperative ASMI in the intervention group with HMB. But we did not report significant difference regarding the baseline and the postoperative Fat-free mass index (FFMI) in both intervention and control group with HMB. Also we did not find significant difference between base line and postoperative in both groups for Mid-arm muscle-circumference (MAMC), Fat mass index (FMI), 6-minute walk test (6MWT), and Handgrip (HG). Data Sources: Medline databases (PubMed, Medscape, ScienceDirect. EMF-Portal) and all materials available in the Internet till 2021. Conclusion: BCAA as dietary supplementation showing a significant favorable effect regarding the baseline and the postoperative BTR levels, also for BCAA levels, Tyrosine levels, prealbumin levels, retinol binding protein and PTINR levels in both intervention and control groups. For HMB, we found significant difference for appendicular skeletal muscle mass (ASMI) regarding the baseline and the postoperative ASMI in the intervention group with HMB. But we did not report significant difference regarding the baseline and the postoperative Fat-free mass index (FFMI) in both intervention and control group with HMB. [ABSTRACT FROM AUTHOR]

The shortage of donor organs has compelled transplant centers to use organs from non-standard donors. The Korean Network for Organ Sharing data showed that there were 5,804 potential recipients on the waiting list, and only 1,579 patients underwent liver transplant in 2019. Reuse of a graft that has been transplanted previously to other recipients could be an option in this situation. However, given the susceptibility of hepatic grafts to ischemic damage, their reuse must be considered extremely carefully. In this retrospective, observational study, we investigated the outcomes of six cases of hepatic graft reuse in Korea since the year 2000, from information gathered from patient medical records from ten transplant centers. Only three of the six reused hepatic grafts functioned well. Among the three successful transplants, two had minimal ischemic damage owing to a longer interval between the first and second transplants, and because they were obtained from living donors. Two of the five cadaveric transplants were successful. The outcome of reusing hepatic grafts in Korea has not been ideal. However, in patients with limited choices, it can be carefully considered, provided the graft is thoroughly checked for ischemic damage and the recipient status is ascertained.

Atrial Fibrillation (AF) is the most commonly diagnosed cardiac arrhythmia. It is associated with significant morbidity and mortality, as it is a major risk factor for cerebral vascular accidents (CVA). Our aim was to determine the prevalence of pre-existing and new-onset AF among patients undergoing liver transplantation (LT) and its impact on post-transplant outcomes. Medline and Embase were searched. Single-arm analysis was conducted using the generalized linear mixed model to determine the prevalence of pre-existing and new-onset AF. Logistic regression was performed to analyze risk factors. Comparative meta-analysis in odds ratio was conducted for binary outcomes. Twenty articles were included, with 17 studies on pre-existing AF, and 7 including data on new-onset AF post-LT. The prevalence of pre-existing AF was 3.3% (CI 2.3–4.7) (14 studies, 45,070 patients) in pooled analysis. Significantly higher prevalence of pre-existing AF patients from North America was noted when compared to Europe (4.5%, CI 3.4–5.8 vs 1.5%, CI 0.8–2.7; p = 0.001). Body mass index (BMI), history of hypertension, diabetes, coronary artery disease (CAD), and cerebrovascular accidents (CVA) were risk factors for pre-existing AF. Pre-existing AF was significantly associated with major adverse cardiac or cerebrovascular events (MACCE) postoperatively (OR 8.02, 95%CI 5.40–11.90, p < 0.001). New-onset AF post-LT had an incidence of 6.8% (CI 4.9–9.3), and was associated with increased risk of mortality (OR 2.31, 95% CI 1.76–3.02, p < 0.001) and graft failure (OR 2.98, CI 1.99–4.47, p < 0.001). AF is relatively more common among patients undergoing LT compared to the general non-transplant population. Additionally, it is associated with adverse outcomes including MACCE, thus warranting clinical attention. Thorough cardiac assessment, and close surveillance of post-operative AF may be clinically prudent. [ABSTRACT FROM AUTHOR]

Khaled Elzorkany,1,2 Mahmoud Abd-Elaziz Kora,1 Aliaa Sabry Abdel Wahed,3 Hassan El-Sayed Zaghla,3 Ahmed Mohamed Zahran,1 Yassein Salah Yassein,1 Asmaa Zaki El Naggar,4 Abdallah Essa,5 Abdelnaser Abdelaty Gadallah1 1Internal Medicine Department, Faculty of Medicine, Menoufia University, Shibin El-Kom, Egypt; 2Internal Medicine Department, College of Medicine, King Faisal University, Al-Ahsa, Saudi Arabia; 3Hepatology and Gastroenterology Department, National Liver Institute, Menoufia University, Shibin El- Kom, Egypt; 4Menshawy Hospital, Tanta, Egypt; 5Tropical Medicine Department, Faculty of Medicine, Menoufia University, Shibin El-Kom, EgyptCorrespondence: Khaled ElzorkanyInternal Medicine Department, Faculty of Medicine, Menoufia University, Shebin El-Kom, Menoufia, EgyptTel 00201002827630Email khaledelzorkany@med.menofia.edu.egPurpose: Direct acting antiviral agents (DAAs) have greatly improved the clearance of hepatitis C virus (HCV) infection. The effect of DAAs on renal function in post-liver transplant HCV-positive patients remains questionable, especially considering the possibility of drug interactions between immunosuppressants and DAAs.Patients and methods: A retrospective observational study included 84 post-liver transplant patients with HCV infection. Patients were divided into two groups: group I received sofosbuvir plus ribavirin for 24 weeks, group II received sofosbuvir plus daclatasvir for 12 weeks. Laboratory data and eGFR were determined before, at the end, and 6 months after completion of treatment.Results: The treatment was well tolerated with 100% sustained virologic response (SVR 12). There was no statistically significant difference between the two groups regarding clinical and laboratory data before treatment. Mean eGFR significantly reduced from 87.36 mL/min to 76.16 mL/min in group I (P=0.001). However, within 6 months after treatment, mean eGFR recovered to 81.51 mL/min, which was not significant when compared to baseline eGFR (P=0.09). Mean eGFR in group II showed non-significant change. There were no significant changes in immunosuppressive drug levels and eGFR in either group of patients, who received either ciclosporin or tacrolimus before and at the end of treatment.Conclusion: DDAs in post-liver transplant patients with HCV infection were well tolerated and associated with stable renal function. Moreover, sofosbuvir plus daclatasvir regimen showed relatively better renal safety compared to sofosbuvir plus ribavirin.Keywords: renal function, hepatic transplantation, direct acting antiviral agents, hepatitis C treatment

Acute severe hepatitis is a rare complication of adult-onset Still's disease (AOSD). This condition is poorly characterized. We performed a review of the medical literature to describe clinical, biological, pathological, and treatment characteristics from AOSD patients with acute severe hepatitis. Their characteristics were compared with AOSD patients without severe hepatitis. Twenty-one cases were collected including a new case reported here. Patients with severe hepatitis were mostly young adults with a median age of 28 years (range: 20 to 55 years). Overall, patients with severe hepatitis had less arthritis, macular rash, sore throat, lymphadenopathy, or splenomegaly than patients without severe hepatitis. Cytopenia was more frequent in case of severe hepatitis. Most patients were treated with steroids, and the use of biotherapies has increased over the last decade. Despite treatment, 49% of patients required liver transplantation and 24% died. Key Points • Acute severe hepatitis in adult-onset Still's disease (AOSD) is associated with liver transplantation and/or death in, respectively, 43% and 24% of cases. • Severe hepatitis is the inaugural manifestation of AOSD in half of cases. Diagnosis is difficult when extra-hepatic clinical manifestations are lacking. • The mechanism of hepatic necrosis in AOSD with severe hepatitis is unknown. Liver biopsy is not specific and should not delay treatment initiation. [ABSTRACT FROM AUTHOR]

This paper reviews the past 50 years of liver transplantation in children from the perspective of patient demographics, perioperative patient management, surgical techniques, immunosuppression and patient outcomes. [ABSTRACT FROM AUTHOR]

ABSTRACT Background: Biliary reconstitution has been considered the Achilles’s heel of liver transplantations due to its high rate of postoperative complications. Aim: To evaluate the risk factors for occurrence of biliary strictures and leakages, and the most efficient methods for their treatment. Method: Of 310 patients who underwent liver transplantation between 2001 and 2015, 182 medical records were retrospectively analyzed. Evaluated factors included demographic profile, type of transplantation and biliary reconstitution, presence of vascular and biliary complications, their treatment and results. Results: 153 (84.07%) deceased donor and 29 (15.93%) living donor transplantations were performed. Biliary complications occurred in 49 patients (26.92%): 28 strictures (15.38%), 14 leakages (7.7%) and seven leakages followed by strictures (3.85%). Hepatic artery thrombosis was present in 10 patients with biliary complications (20.4%; p=0,003). Percutaneous and endoscopic interventional procedures (including balloon dilation and stent insertion) were the treatment of choice for biliary complications. In case of radiological or endoscopic treatment failure, surgical intervention was performed (biliodigestive derivation or retransplantation (32.65%). Complications occurred in 25% of patients treated with endoscopic or percutaneous procedures and in 42.86% of patients reoperated. Success was achieved in 45% of patients who underwent endoscopic or percutaneous procedures and in 61.9% of those who underwent surgery. Conclusion: Biliary complications are frequent events after liver transplantation. They often require new interventions: endoscopic and percutaneous procedures at first and surgical treatment when needed. Hepatic artery thrombosis increases the number of biliary complications.

Background: Surgical site infection is an important complication in the postoperative period among liver transplant recipients. However, little is known about the risk factors in this patient group. Therefore, the objective of this study was to analyze the incidence and risk factors for surgical site infections among adult liver transplant recipients. Methods: Medical records of adult liver transplant recipients from January 1, 2009, to December 31, 2015, were analyzed in this retrospective cohort study. Results: We enrolled 156 recipients' medical records. Forty-two (26.9%) cases of surgical site infections were identified. The main isolated microorganisms were methicillin-resistant Staphylococcus species, extended spectrum β-lactamase-producing Klebsiella species, carbapenem-resistant Pseudomonas aeruginosa, carbapenem-resistant Acinetobacter baumannii, and vancomycin-susceptible Enterococcus faecalis. We found that long operative times (≥487 minutes) and differences in body mass index between donor and recipient (≥1.3 kg/m2) increased the risk for surgical site infections by approximately 5 times (odds ratio [OR], 5.5; 95% confidence interval [CI], 2.5-11.8), and capillary glycemia ≥175 mg/dL in the first 96 postoperative hours increased the risk by approximately 3 times (OR, 2.97; 95% CI, 1.43-6.17). Conclusions: There was a high incidence of surgical site infections among the studied population and that some risk factors identified differ from those reported in the scientific literature. [ABSTRACT FROM AUTHOR]

The continuously decreasing willingness for liver donation aggravates treatment of end-stage liver diseases requiring organ transplantation as the only curative strategy. Cell therapy approaches using human hepatocytes or stem cell-derived hepatocyte-like cells may be a therapeutic option out of this dilemma. ABCB5-positive mesenchymal stromal cells from human skin featured promising potential to treat immune-mediated diseases. Since most of chronic liver diseases involve exaggerating immune mechanisms, it was the aim to demonstrate in this study, whether ABCB5 + stem cells may serve as a resource to generate hepatocytic cells for application in liver cell transplantation. Using an established single-step protocol, which had been successfully applied to differentiate mesenchymal stromal cells into the hepatocytic lineage, ABCB5 + skin-derived stem cells did not gain significant characteristics of hepatocytes. Yet, upon culture in hepatocytic differentiation medium, ABCB5 + stem cells secreted immunomodulatory and anti-fibrotic factors as well as proteins, which may prompt hepatic morphogenesis besides others. Hepatic transplantation of ABCB5 + stem cells, which had been prior cultured in hepatocytic differentiation medium, did not cause any obvious deterioration of liver architecture suggesting their safe application. Thus, human ABCB5 + skin-derived stem cells secreted putative hepatotropic factors after culture in hepatocytic differentiation medium. [ABSTRACT FROM AUTHOR]

Background: Milan criteria are used for patient selection in liver transplantation for hepatocellular carcinoma (HCC). Hangzhou criteria have been shown in China to enable access to liver transplantation for more patients when compared to Milan criteria without negative effects on long-term survival. The purpose of this study was to evaluate the Hangzhou criteria in a German cohort.Methods: One hundred fifty-nine patients transplanted for HCC between 1975 and 2010 were investigated. Patients were categorized into four groups depending on the fulfillment of Milan and Hangzhou criteria. General and tumor baseline characteristics were compared. Overall and tumor-free survival rates were investigated with the Kaplan-Meier analysis.Results: One-, 3-, 5-, and 10-year survival rates for patients fulfilling Milan criteria (n = 68) were 89.7, 83.7, 75.8, and 62.1%, respectively, versus 89.8, 82.2, 75.2, and 62.6% for patients fulfilling Hangzhou criteria (n = 109) (p = 0.833). When comparing patients exceeding Milan or Hangzhou criteria, survival rates were 75.3, 53.2, 48.1, and 41.1% versus 63.3, 31.4, 26.9, and 22.1%, respectively (p = 0.019). The comparison of tumor-free survival rates in patients fulfilling Milan or Hangzhou criteria was statistically not significant (p = 0.785), whereas the comparison of the groups exceeding the criteria showed significantly worse survival for patients outside Hangzhou criteria (p = 0.007). The proportion of patients fulfilling Hangzhou criteria (68.6%) was significantly larger as compared to the proportion fulfilling Milan criteria (42.8%) (p < 0.001).Conclusion: Hangzhou criteria are more accurate in predicting long-term survival after liver transplantation for HCC in Germany. Deployment of the Hangzhou criteria for patient selection could enlarge the pool of transplantable patients. [ABSTRACT FROM AUTHOR]

Citrullinemia or ASS deficiency in its classical form presents in the neonatal period with poor feeding, hyperammonemia, encephalopathy, seizures, and if untreated can be fatal. Despite advances in medical therapy, neurocognitive outcomes remain suboptimal. LT has emerged as a potential management option. A retrospective singlecenter review identified 7 children with a median age of 1.1 years (range, 0.6-5.8) at referral. Five children presented clinically, and 2 were treated prospectively from birth due to positive family history. All patients received standard medical and dietary therapy prior to LT. The indications for LT were frequent metabolic decompensations in 4, elective in 2, and ALF in 1. The median age at LT was 2.4 years (range, 1.3-6.5). Five patients received 6 left lateral segment grafts, one a live unrelated donor left lateral segment as an APOLT graft, and one a cadaveric whole liver graft as APOLT. One child required retransplantation due to hepatic artery thrombosis. Graft and patient survival were 86% and 100%, respectively. Median follow-up is 3.1 years (range, 0.1-4.1), and the median age at follow-up is 5.5 years (range, 4.0-9.8). There have been no metabolic decompensations in 6 children, while 1 patient (with APOLT) developed asymptomatic hyperammonemia with no clinical or histological signs of liver injury, requiring additional medical therapy. Our medium-term experience following LT in citrullinemia is favorable, demonstrating a positive transformation of the clinical phenotype. [ABSTRACT FROM AUTHOR]

Background Surgical site infection (SSI) is an important complication in the postoperative period of recipients of liver transplantation. The purpose of this integrative literature review is to summarize the knowledge available about the risk factors contributing to the development SSI among adults undergoing liver transplantation. Methods We reviewed the Medical Literature Analysis and Retrieval System Online/PubMed, the Cumulative Index to Nursing and Allied Health Literature, the Latin American and Caribbean Health Sciences Literature, Scopus, and Web of Science databases. Results Two hundred sixteen articles were identified and the final sample of 9 articles was analyzed in full length. The SSI rate found in the investigations ranged between 9.6% and 35.5%. Risk factors for SSI were grouped into categories related to the preoperative period, such as Model for End-Stage Renal Disease score > 35 and ventilated support on day of transplant; to the intraoperative period activity, such as transfusion of packed red blood cells, extended surgical time, hyperglycemia >200 mg/dL, use of vasopressor drugs, and ascites flow >1 L; and to the donor/recipient relationship, such as age differences >10 years, ratio of donor liver mass to recipient body mass < 0.01. Additionally, centers that annually perform <50 transplants appear to have higher rates of SSI. Conclusions Few studies have addressed the subject of SSI in relation to liver transplantation in the scientific literature. Risk factors for SSI in patients who underwent liver transplantation vary between institutions. [ABSTRACT FROM AUTHOR]

Hepatic cavernous hemangioma accounts for 73% of all benign liver tumors with a frequency of 0.4-7.3% at autopsy and is the second most common tumor seen in the liver after metastases. Patients affected by hemangioma usually have their tumor diagnosed by ultrasound abdominal examination for a not well defined pain, but pain persist after treatment of the hemangioma. The causes of pain can be various gastrointestinal pathologies including cholelithiasis and peptic ulcer disease.The malignant trasformation is pratically inexistent. Different imaging modalities are used to diagnosis liver hemangioma including ultrasonography, computed tomography (CT), magnetic resonance (MR) imaging, and less frequently scintigraphy, positron-emission tomography combined with CT (PET/CT) and angiography. Imaging-guided biopsy of hemangioma is usually not resorted to except in extremely atypical cases. The right indications for surgery remain rupture, intratumoral bleeding, Kasabach-Merritt syndrome and organ or vessels compression (gastric outlet obstruction, Budd-Chiari syndrome, etc.) represents the valid indication for surgery and at the same time they are all complications of the tumor itself. The size of the tumor do not represent a valid indication for treatment. Liver hemangiomas, when indication exist, have to be treated firstly by surgery (hepatic resection or enucleation, open, laproscopic or robotic), but in the recent years other therapies like liver transplantation, radiofrequency ablation, radiotherapy, trans-arterial embolization, and chemotherapy have been applied.

Background: The model of end-stage liver disease (MELD) score is the standard tool for prioritizing patients awaiting liver transplantation. There is currently no definite high cutoff score reflecting disease severity that might exclude patients from transplantation. Furthermore, the age limit that used to disqualify patients from eligibility to transplantation was recently withdrawn in several countries.Aims: The aims of this study were to assess the effects of MELD score and age at time of transplantation on patients' short- and long-term survival.Methods: We conducted a retrospective single-center study on a cohort of patients transplanted for the first time due to non-fulminant liver failure.Results: Four hundred and seventeen patients (mean age 50.2 years, 63% males) who underwent liver transplantation for the first time were included. Both higher patients' and donors' age were significantly associated with increased long-term mortality (p = 0.007, 95% CI 1.006-1.038 for patient age, p = 0.02, 95% CI 1.002-1.023 for donor age). Patients' age remained significantly associated with survival at 1 year post-transplantation, as well. We found no association between higher MELD score at transplantation and long-term mortality (p = 0.189, 95% CI 0.99-1.051) irrespective of patients' age. Specifically, when patients were divided according to their MELD score at transplantation (MELD < 15, MELD 15-25 and MELD > 25), no significant differences in long-term survival were detected between these three subgroups. Results did not differ significantly in a subgroup analysis of patients without hepatocellular carcinoma at the time of transplantation.Conclusions: Patients' and donors' age rather than patients' MELD score at transplantation determine survival following liver transplantation. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Hepatic artery thrombosis should always be considered on a liver graft recipient with mild and nonspecific symptoms, even after a decade of the transplantation. [ABSTRACT FROM AUTHOR]

Sinus of valsalva aneurysm (SVA) is a rare cardiac disease. The most common complication of SVA is rupture into the right atrium or right ventricle. Rupture into the left chambers is very rare. Patients with ruptured SVA are likely to die of heart failure or endocarditis. We present a 29-year-old man who was hospitalized for hepatic transplantation with rupture of SVA. Transthoracic echocardiography and transesophageal echocardiography showed rupture of a noncoronary SVA into the left atrium. Mitral valve infective endocarditis developed and surgery was planned for the patient, but the patient died due to multiple organ dysfunction syndrome. [ABSTRACT FROM AUTHOR]

Liver transplantation protocols have been extended to people over 65 years of age who had previously been excluded from protocols due to the increased morbidity and mortality rates associated with age. This study aims to identify survival rates and complications in patients over 65 years who have undergone liver transplantation. Medical records of patients older than 65 who underwent liver transplantation in the period between September 2004 and November 2010 at the Hospital Pablo Tobón Uribe in Medellin, Colombia were analyzed. We studied 27 patients with an average age of 67 years. The 30 day post-transplant survival rate was 85.2%, at one year the survival rate was 70.4%, and at five years it was 63%. An evaluation of post-transplant complications found that the most frequent complications were massive bleeding and nosocomial infections. The main causes of death were cardiovascular events. Conclusion: Proper patient selection allows those over 65 years of age to become candidates for liver transplantation with a good actuarial 5-year survival rate and better quality of life. [ABSTRACT FROM AUTHOR]

Hepatic cavernous hemangioma accounts for 73% of all benign liver tumors with a frequency of 0.4-7.3% at autopsy and is the second most common tumor seen in the liver after metastases. Patients affected by hemangioma usually have their tumor diagnosed by ultrasound abdominal examination for a not well defined pain, but pain persist after treatment of the hemangioma. The causes of pain can be various gastrointestinal pathologies including cholelithiasis and peptic ulcer disease. The malignant trasformation is pratically inexistent. Different imaging modalities are used to diagnosis liver hemangioma including ultrasonography, computed tomography (CT), magnetic resonance (MR) imaging, and less frequently scintigraphy, positronemission tomography combined with CT (PET/CT) and angiography. Imaging-guided biopsy of hemangioma is usually not resorted to except in extremely atypical cases. The right indications for surgery remain rupture, intratumoral bleeding, Kasabach-Merritt syndrome and organ or vessels compression (gastric outlet obstruction, Budd-Chiari syndrome, etc.) represents the valid indication for surgery and at the same time they are all complications of the tumor itself. The size of the tumor do not represent a valid indication for treatment. Liver hemangiomas, when indication exist, have to be treated firstly by surgery (hepatic resection or enucleation, open, laproscopic or robotic), but in the recent years other therapies like liver transplantation, radiofrequency ablation, radiotherapy, trans-arterial embolization, and chemotherapy have been applied. [ABSTRACT FROM AUTHOR]