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Introduction: Triple therapy with telaprevir or boceprevir has proven to be effective in the treatment of chronic hepatitis C with response rates of up to 88%. However, the treatment may be associated with important adverse effects and a high economic impact., Objective: To assess the cost-effectiveness and safety of triple therapy with telaprevir or boceprevir for the treatment of chronic hepatitis C., Methods: Retrospective observational study. We included all patients who had started treatment with protease inhibitors before July 31(st), 2013. We evaluated sustained virological response, the cost per patient achieving sustained virological response, and the cost of the supportive treatment for adverse events associated with triple therapy., Results: Fifty-nine patients were included; 35 had been treated with telaprevir (59.3%) and 24 with boceprevir (40.7%). Sustained virological response was achieved by 38 (64.4%) patients: 24 (68.6%) patients in the telaprevir treatment arm and 14 (58.3%) patients in the boceprevir treatment arm. The cost per patient with sustained virological response was 43,555 € (95% CI 35,389-51,722 €). There were no statistically significant differences between the overall costs of therapy with telaprevir, 43,494 € (95% CI 34,795 €-55,092 €) versus boceprevir, 42,005 € (95% CI 32,122-64,243€). The mean cost of supportive care per patient was 1,500 €, while the maximum cost was 11,374 €. Due to adverse events, 8 (13.6%) patients required hospital admission, 22 (37.3%) patients attended the accident and emergency department, and 26 (44.1%) patients needed additional medical consultations., Conclusions: The treatment of triple therapy with telaprevir or boceprevir resulted in high cost per patient with sustained virological response. Due to adverse events, a high number of patients required supportive care, whose costs should be added to those of triple therapy., (Copyright © 2015 Elsevier España, S.L.U. and AEEH y AEG. All rights reserved.)

Introduction: Gastrointestinal hemorrhage due to vascular malformations has a negative impact on patients´ quality of life and consumes an important quantity of resources., Objective: Analyze the cost-effectiveness of long-active releasing octreotide (OCT-LAR) in the treatment of gastrointestinal haemorrhage secondary to vascular malformations., Material and Methods: Retrospective study, including 19 pacients that were treated with mensual injections of OCTLAR between 2008-2013. The number of blood transfusions, hemoglobin levels, hospital admissions and possible side effects during the year before treatment and the year after the start of the treatment were assessed, and cost-effectiveness was analyzed., Results: After the beginning of the treatment with OCTLAR, complete response was observed in 7 patients (36.8 %), partial response in 7 patients (36.8 %) and 5 patients (26.3 %) continued to require admissions, blood transfusions and/or endoscopic treatment. We observed significant reduction in the length of admission per year (in days) before and after the start of the treatment (22.79 versus 2.01 days, p < 0.0001) as well as in the number of blood transfusions administered (11.19 versus 2.55 blood transfusions per year, p = 0.002). The mean haemoglobin levels increased from 6.9 g/dl to 10.62 g/dl (p < 0.0001). We observed reduction of costs of 61.5 % between the two periods (from 36,072.35 € to 13,867.57 € per patient and year, p = 0.01). No side effects related to treatment were described., Conclusion: In conclusion, OCT-LAR seems to be a costefficient and safe pharmacological treatment of gastrointestinal haemorrhage secondary to vascular malformations, mainly in patients in whom endoscopic or surgical treatment is contraindicated.

Wilson's disease is a hereditary autosomal recessive disorder of copper metabolism,characterized by copper accumulation in the liver and brain. This rare entity, which has a broad clinical spectrum, is often difficult to diagnose and should therefore always be suspected in patients with liver disease of unclear cause. We describe two types of manifestation of liver disease in two patients; the first developed fulminant hepatic failure requiring urgent liver transplantation and the second showed advanced chronic liver disease and received standard medical treatment. The objective of this clinical observation is to analyze the diagnosis of Wilson's disease in two patients with distinct onset, illustrating the broad clinical spectrum of the disease, and its treatment., (Copyright © 2012 Elsevier España, S.L. and AEEH y AEG. All rights reserved.)

Introduction and Aims: Spontaneous bacterial peritonitis (SBP) is ascitic fluid (AF) infection in the absence of an intraperitoneal source of infection in patients with liver disease and portal hypertension. The aim of this study was to evaluate the yield of microbiological cultures to optimize their collection and systematic implementation in routine clinical practice., Methods: We analyzed two cohorts: the first consisted of retrospective data from the clinical records of 156 consecutive episodes of SBP from January 2003 to December 2005 (group R), and the second was composed of data collected prospectively from October 2007 to October 2008, consisting of 79 episodes (group P), with systematic inoculation of 10 cc of AF in each blood culture bottle., Results: No significant differences were demonstrated in baseline epidemiological or clinical characteristics among episodes, except the more frequent presence of alcoholic liver disease and the incidence of tense ascites, paralytic ileus and kidney failure at diagnosis in group P. Microbiological isolation in AF was achieved in 12.2% of the episodes in group R compared with 53.2% in group P (p = 0.001) and 8.5 and 26.6% (p = 0.001) in blood culture, respectively. Microbiological isolation was achieved in 65.8% of episodes in group P versus 19.2% in group R (OR 8; 95% CI: 4.4-14.9; p = 0.001). The most frequently isolated organism in AF in group P was Escherichia coli (42.9%)., Conclusions: Optimizing the diagnostic procedure in cirrhotic patients with ascites significantly increases diagnostic microbiologic performance., (Copyright © 2010 Elsevier España, S.L. All rights reserved.)

Introduction and Objective: Hepatocellular carcinoma (HCC) is the sixth leading cause of cancer. In western countries its impact is steadily growing and most of these tumors arise in cirrhotic liver. The aim of this study was to evaluate the incidence and characteristics of HCC developing in noncirrhotic patients., Methods: We conducted a retrospective review of 469 patients diagnosed with HCC between January 2003 and December 2007. Patients who met the histological criteria, or a combination of clinical, laboratory, imaging and hemodynamic criteria for the absence of cirrhosis were included., Results: We identified 29 patients with a diagnosis of HCC in non-cirrhotic liver (6.2%). Most (86.2%) were men and the median age was 61.6 (23 - 82) years. The most frequent histology of the liver was mild-moderate liver fibrosis (68.9%) and 62.1% of the patients were asymptomatic at diagnosis. HCC was a solitary nodule in 86.2%, and the median size of the main nodule was 46 (20 - 150) mm. Macroscopic vascular invasion was demonstrated in two patients and bone metastases in a further two patients. Active treatment was provided in 25 patients, consisting of tumoral resection in 15, radiofrequency ablation in six and transarterial chemoembolization in four. The overall cumulative survival at 1, 2 and 3 years was 84.3%, 67.2% and 50.1%, respectively., Conclusion: HCC arising in non-cirrhotic liver is uncommon and there is wide tumor extension at diagnosis. Nevertheless, in most patients, treatment with curative intent can be applied, achieving better survival than that expected patients with cirrhosis., (Copyright © 2010 Elsevier España, S.L. All rights reserved.)

Aims: Our aim was to study the episodes of spontaneous bacterial peritonitis (SBP) in cirrhotic patients older than 65 years. Their clinical characteristics were compared with a cohort of cirrhotic patients with SBP., Methods: We conducted a retrospective study on all hospital discharged patients diagnosed with SBP in the period from January 2003 to December 2005, obtaining a total of 158 episodes. Two groups of patients were created: those over 65 (group 1: 73 episodes) and those aged 65 years (group 2: 86 episodes). A descriptive study, an analysis of survival by Kaplan Meier curves (compared with log-rank test) and a Cox regression analysis was conducted comparing the two groups., Results: In Group 1, males were 74%, the average age was 72.4 (SD 4.2) years, and the Child-Pugh distribution was 8.2% (A), 68.5% (B) and 23.3% (C).The most usual etiology of cirrhosis was viral (65.8%). In comparison with group 2, there was a superior frequency of renal impairment at admission, hepatocellular carcinoma and a viral etiology. Yet the bilirubin levels were higher in group 2. In-hospital mortality rate was 23.3%. The survival at 3 and 6 months was of 69.5% and 54.8%, while in group 2 it was 80.3% and 78.4% respectively (p=0001). An age> 65 years, the existence of hepatocellular carcinoma and a Mean Arterial Pressure <75 mm Hg at admission, in the overall cohort, were independent predictors of mortality., Conclusion: An age above 65 years is an independent predictor of mortality following the development of an episode of spontaneous bacterial peritonitis in cirrhotic patients.

Neurofibromatosis type 1 (NF1) is a multisystemic disease with an autosomal dominant pattern of inheritance, complete penetrance and variable expressivity. This disease affects one in every 3,000 individuals and one out of every 200 patients with mental retardation. Gastrointestinal bleeding secondary to gastrointestinal mesenchymal tumors is one of the many complications associated with NF1. We report the case of a patient with NF1 admitted to hospital for severe gastrointestinal hemorrhage secondary to a jejunal gastrointestinal stromal tumor (GIST) with favorable outcome after surgical removal.

Objectives: To assess survival and predictive factors of mortality after an episode of spontaneous bacterial peritonitis (SBP) in cirrhotic patients and to analyze the diagnostic and therapeutic measures used., Methods: We retrospectively reviewed the medical records of 158 consecutive episodes of SBP treated between January 2003 and December 2005. Survival was studied by Kaplan-Meier curves, compared by the log-rank test. Independent predictive factors of mortality were obtained by a Cox regression model, while independent predictive factors of in-hospital mortality were obtained by logistic regression analysis., Results: A total of 80.4% of the SBP episodes occurred in men and the mean age was 61.23 +/- 12.49 years. The most frequent etiology of cirrhosis was viral (51.3%), followed by alcoholic (39.9%). The distribution of Child-Pugh classification was 5.7% (A), 63.3% (B) and 31% (C). Overall 3-year survival in the sample was 43.3%. Four variables were identified as independent predictive factors of in-hospital and 3-month mortality: renal impairment, hepatic encephalopathy, diagnosis of hepatocellular carcinoma (HCC) and mean arterial pressure (MAP) < 75 mmHg. At the end of the monitoring period, the results of the analysis were as follows: diagnosis of HCC, MAP < 75 mmHg, and age > 65 years. Microbiological detection was achieved in 21% of the episodes. The most frequent microorganisms detected were Escherichia coli in ascitic fluid and Staphylococcus aureus in blood cultures., Conclusions: SBP has a poor short- and long-term prognosis in cirrhotic patients. Independent predictive factors of short-term survival are renal impairment, hepatic encephalopathy, MAP < 75 mmHg, and the presence of HCC.

The combination of pegylated interferon and ribavirin is the first option treatment for chronic hepatitis C (HCC). Although not frequent, several vascular adverse events, such as Raynaud's phenomenon, thrombotic thrombocytopenic purpura, hemolytic uremic syndrome and central retinal vein occlusion, have been associated with the use of pegylated and standard interferon. We report the cases of two patients with HCC who, while receiving treatment with pegylated interferon and ribavirin, developed decompensated liver disease. Radiological tests revealed acute portal thrombosis. After the diagnosis was established, anticoagulant therapy was started, which resolved the portal thrombosis.

Objective: To evaluate factors associated with poor survival in patients with non-active treatment of hepatocellular carcinoma (HCC)., Material and Methods: Between May 2003 and June 2005, 50 patients with HCC were deemed unsuitable for active treatment, following the Barcelona Clinic Liver Cancer staging system. Symptomatic treatment was provided. Kaplan-Meier curves were constructed and compared by the log-rank test to identify factors associated with poor survival. Independent factors predictive of survival were evaluated by multivariate Cox regression analysis., Results: The mean age was 65.6 +/- 11.9 years and 84% of the patients were men. Forty-eight percent of the patients had hepatitis C infection and 58% were Child-Pugh grade A. HCC was multinodular in 54% and the total tumor size was more than 5 cm in 90% of patients. Thirty-four percent of the patients had malignant portal thrombosis and four patients had metastases. Thirty-eight percent of the patients had received previous treatment. The median follow-up was 9.2 months and 1- and 2-year survival was 46% and 17.5%, respectively. Poor survival was associated with male sex, alpha-fetoprotein values of > 400 ng/ml, albumin levels of < 3 g/dl, and metastases. Independent predictors identified by multivariate Cox regression analysis were male sex, albumin levels of < 3 g/dl, and alpha-fetoprotein values of > 400 ng/ml. The median survival in patients with two or more independent factors was significantly lower than that in patients with none or only one factor (14.2 vs. 4.1 months)., Conclusion: Survival in patients with non-active treatment of hepatocellular carcinoma can be estimated and the factors involved allow separate groups of patients with different short- to medium-term prognoses to be identified.

The prevalence of HCV infection in Spanish prisons is very high (38.5%). The characteristics of the infected patients, particularly the high rate of HIV coinfection, makes it very likely that the morbidity and mortality produced by serious liver disease secondary to this infection will increase considerably in the coming years. A group of Spanish experts with experience in patients who are inmates has been invited to establish a series of recommendations for the diagnosis and treatment of chronic hepatitis C infection in Spanish prisons.

The prevalence of HCV infection in Spanish prisons is very high (38.5%). The characteristics of the infected patients, particularly the high rate of HIV coinfection, makes it very likely that the morbidity and mortality produced by serious liver disease secondary to this infection will increase considerably in the coming years. A group of Spanish experts with experience in patients who are inmates has been invited to establish a series of recommendations for the diagnosis and treatment of chronic hepatitis C infection in Spanish prisons.

The prevalence of systemic thromboembolic complications is higher in patients with inflammatory bowel disease than in the general population. This hypercoagulable state is due to an increased production of procoagulant substances proportionally related to the inflammatory activity of the disease, although recent reports have focused on the presence of inherited thrombophilic disorders in this entity. We present the case of a 32-year-old woman with no relevant medical history who presented with massive abdominal vein thrombosis, including suprahepatic, portal, splenic and superior mesenteric veins, and secondary acute liver failure in her first ulcerative colitis flare and who later developed toxic megacolon requiring emergency total colectomy despite steroids and cyclosporine. Anticoagulant therapy achieved complete resolution of suprahepatic thrombosis and partial resolution in the splenic and superior mesenteric veins, with final cavernous transformation of the portal vein.

Hepatoportal sclerosis is characterized by fibrosis of the intima of the portal vein and its branches leading to the development of presinusoidal portal hypertension. We describe the case of a 58-year-old woman with idiopathic hepatoportal sclerosis, who was admitted to our service due to impairment of liver function, with rapid clinical worsening and finally the development of multiorgan failure. Autopsy showed a diffuse liver angiosarcoma with splenic metastases. The patient had no history of domestic or occupational exposure to substances involved in the development of hepatoportal sclerosis or liver angiosarcoma. The development of liver angiosarcoma in a patient with hepatoportal sclerosis is exceptional, even though both diseases may have a common etiology.

Cyclooxygenase (COX) is a crucial enzyme in the biosynthesis of prostaglandins. There are two COX isoforms: COX-1 is constitutively expressed in a number of cell types and is involved in the homeostatic functions of prostaglandins, whereas COX-2 is inducible by a variety of proinflammatory stimuli, such as cytokines and lipopolysaccharide. In the liver, COX-2 and prostaglandins production has been implicated in hepatic regeneration, liver matrix remodeling and portal hypertension. In animal models of alcoholic-induced liver disease has been demonstrated its relation with necro-inflammatory activity. In viral hepatitis, hepatocellular COX-2 expression was observed and associated with fibrosis progression. More interestingly it has been the demonstration of COX-2 role in the development of hepatocellular carcinoma and cholangiocarcinoma, such in experimental models as in human samples. It has also been demonstrated that COX-2 was implicated in carcinogenesis through apoptosis inhibition and increased proliferation of human tumor cells. Experimental evidences show that selective pharmacologic inhibition of COX-2 could be useful in chemoprevention of primary liver tumors.

Hepatitis C virus (HCV) has been associated with several autoimmune and rheumatologic disorders. The aim of this study was to determine the incidence of these abnormalities in patients with chronic HCV. We studied 56 patients, 29 of whom (52%) had biochemical abnormalities that suggested immunological disorders. Cryoglobulinemia was detected in nine patients (22%), antinuclear antibodies in eleven (20%), rheumatoid factor in seven (19.27%) and hypocomplementemia in fourteen (29.16%). The most common clinical manifestations were: arthralgias (52%), myalgias (16%), xerostomia (28.5%) and xerophthalmia (14%). These results indicate the existence of a relationship between HCV and rheumatologic disorders. We conclude that HCV may play a role in the pathogenesis of these autoimmune phenomena, but more studies are required to define the extent of this role.