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There is uncertainty regarding Wilson's disease (WD) management., Objectives: To assess, in a multicenter Spanish retrospective cohort study, whether the approach to WD is homogeneous among centers., Methods: Data on WD patients followed at 32 Spanish hospitals were collected., Results: 153 cases, 58% men, 20.6 years at diagnosis, 69.1% hepatic presentation, were followed for 15.5 years. Discordant results in non-invasive laboratory parameters were present in 39.8%. Intrahepatic copper concentration was pathologic in 82.4%. Genetic testing was only done in 56.6% with positive results in 83.9%. A definite WD diagnosis (Leipzig score ≥4) was retrospectively confirmed in 92.5% of cases. Chelating agents were standard initial therapy (75.2%) with frequent modifications (57%), particularly to maintenance zinc. Enzyme normalization was not achieved by one third, most commonly in the setting of poor compliance, lack of genetic mutations and/or presence of cardiometabolic risk factors. Although not statistically significant, there were trends for sex differences in number of diagnosed cases, age at diagnosis and biochemical response., Conclusions: Significant heterogeneity in diagnosis and management of WD patients emerges from this multicenter study that includes both small and large reference centers. The incorporation of genetic testing will likely improve diagnosis. Sex differences need to be further explored., (Copyright © 2022 Elsevier España, S.L.U. All rights reserved.)

Background: Bacterial infections remain one of the main complications in cirrhosis and worsen patients' prognosis and quality of life. An increase in multidrug resistant microorganism (MDRM) infections among patients with cirrhosis, together with infection-related mortality rates, have been reported in recent years. Therefore, adaptation of the initial empiric antibiotic approach to different factors, particularly the local epidemiology of MDRM infections, has been recommended. We aim to describe the main features, outcomes and risk factors of MDRM infections in patients with cirrhosis., Methods: Prospective registry of all episodes of in-hospital infections occurring among cirrhotic patients admitted within a 2-year period at a single center. Clinical and microbiological data were collected at the time of infection diagnosis, and the in-hospital mortality rate of the infectious episode was registered., Results: A total of 139 infectious episodes were included. The disease-causing microorganism was identified in 90 episodes (65%), of which 31 (22%) were caused by MDRM. The only two factors independently associated with MDRM infections were rectal colonization by MDRM and a nosocomial or healthcare-associated source. The infection-related mortality rate was 18.7%. MDRM infection and a past history of hepatic encephalopathy were independently associated with in-hospital mortality., Conclusions: Almost one fourth of bacterial infections occurring in admitted cirrhotic patients were due to MDRM. Rectal colonization was the most important risk factor for MDRM infections in decompensated cirrhosis. Screening for MDRM rectal colonization in patients admitted for decompensated cirrhosis should be assessed as a tool to improve local empiric antibiotic strategies., (Copyright © 2022 Elsevier España, S.L.U. All rights reserved.)

Background: Acute-on-chronic liver failure (ACLF) is a common syndrome that occurs in patients with advanced chronic liver disease. It consists of the rapid failure of various organs and is associated with high short-term mortality. We aim to describe the main features and outcomes of inpatients who developed ACLF and to identify the factors associated with in-hospital and 28-day mortality., Patients and Methods: All patients meeting ACLF criteria with advanced chronic liver disease admitted for decompensation from January 2014 to December 2016 were identified. Clinical and biological data were collected at the time of ACLF diagnosis and at 3-7 days thereafter, as well as in-hospital and 28-day mortality., Results: Eighty nine out of 354 admission episodes (28%) developed ACLF, which was present at the time of admission in 72% of cases. A precipitating factor was identified in 83% of cases, the most frequent being infection (53%) and gastrointestinal bleeding (19%). In the multivariate regression analysis, the ACLF grade at 3-7 days after diagnosis was predictive of in-hospital mortality and 28-day mortality, and lower creatinine and bilirubin levels at the time of ACLF diagnosis and a precipitating factor other than bacterial infection were associated with ACLF reversion at 3-7 days., Conclusions: ACLF is a frequent complication among patients with chronic liver disease admitted for acute decompensations and is associated with a high mortality rate and is related to the number of organs involved. Bacterial infection is the most frequent precipitating factor of ACLF and probably entails a worse prognosis., (Copyright © 2021 Elsevier España, S.L.U. All rights reserved.)

Interferon-free regimens achieve sustained virologic response (SVR) rates of over 90%, have generally well-tolerated adverse effects and involve 12-week treatment durations for most patients with chronic hepatitis C, including naive or previously treated patients and patients with or without cirrhosis. However, some of the treatment options recommended by the guidelines require the addition of ribavirin (RBV) or extend the duration of treatment to increase efficacy. The use of RBV is a useful tool in those difficult-to-cure patients such as patients with decompensated or genotype-3-infected cirrhosis and those who have not achieved SVR after treatment with direct-acting antivirals (DAA). Overall, adding RBV to the different combinations causes adverse effects related to a decrease in haemoglobin and involves inconveniences such as its dosage, which requires patients to take several tablets twice daily. However, severe anaemia is rare and easily manageable with a dose reduction. In addition, RBV is teratogenic. In practice, because RBV is inexpensive and well tolerated when combined with an interferon-free regimen, it continues to be a useful tool to optimise the results of some HCV treatment regimens. RBV-free regimens eliminate RBV-related adverse effects related, resulting in better tolerability, improving patient adherence and quality of life and reducing the cost of treatment., (Copyright © 2017 Elsevier España, S.L.U. All rights reserved.)

Hepatocarcinoma (HCC) is the most frequent primary malignant hepatic tumour. These tumours usually develop in cirrhotic liver; for this reason, periodic screening using alphafetoprotein determination and abdominal ultrasonography is considered in cirrhotic patients with preserved hepatocellular function. This strategy allows early detection of HCC, increasing the proportion of curable tumours. The most frequent metastasic dissemination is portal vein neoplasic thrombosis, being unusual the occurrence of spread metastases in other organs. We present 3 cases of atypical HCC metastasis with non specific clinical manifestations which initial diagnosis was wrong. Because of a longer survival of these patients in recent years, spread metastasis might be considered in patients with known HCC and non specific symptoms.