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A retrospective data analysis on liver transplantation for Wilson's disease (WD) was performed among Italian Liver Transplant Centers. Thirty-seven cases were identified. The main indication for liver transplantation was chronic advanced liver disease in 78% of patients. Mixed hepatic and neuropsychiatric symptoms were recorded in 32.3%. Eight patients presented with fulminant liver failure; 44.8% were on medical treatment. Patient and graft survival at 3 months, 12 months, 3 years, 5 years, and 10 years after transplantation were, respectively, 91.8%, 89.1%, 82.9%, 75.6%, and 58.8%, and 85.3%, 83.0%, 77.1%, 70.3%, and 47.2%. Neurological symptoms significantly improved after orthotopic liver transplantation (OLT), but the survival of patients with mixed hepatic and neuropsychiatric involvement was significantly lower than in patients with liver disease alone (P = 0.04). WD characterized by hepatic involvement alone is a rare but good indication for liver transplantation when specific medical therapy fails. Patients with neuropsychiatric signs have a significantly shorter survival even though liver transplantation has a positive impact on neurological symptoms. In conclusion, a combination of hepatic and neuropsychiatric conditions deserves careful neurological evaluation, which should contraindicate OLT in case of severe neurological impairment.

The capacity of endoscopic ultrasound (EUS) to distinguish the different wall layers of the gastrointestinal (GI) tract and the possibility to obtain samples of suspicious lesions or lymph nodes by means of EUS-guided fine-needle aspiration (EUS-FNA), make EUS an ideal staging modality for GI cancers. After an endoscopic and histological diagnosis of gastric cancer (GC), an accurate preoperative evaluation is essential to choose the correct management decision, because for this malignancy various and radically different stage-oriented therapies can be performed. Even if EUS is inserted in the last guidelines for the management of GC as an essential pretherapeutic staging modality, in the literature the reported accuracy, the imaging features and the performances of the technique are variable. In this review, we synthesize the current status and the imaging findings of EUS when describing and staging GC, with a particular attention to the early GC that represents till today a diagnostic and therapeutic challenge. Currently, the EUS study is mandatory for the preoperative staging, to assess with a good accuracy the tumor depth of wall invasion, the presence of suspicious lymph-nodes and of ascites (predictive of peritoneal involvement). The main limitations for a correct EUS staging remain some features of the lesion or its localization, so more attention should be paid when these characteristics are present.

Treatment with peginterferon α-2a (PegIFN) for 48 weeks is the standard of care for selected HBeAg-negative patients chronically infected with hepatitis B virus (HBV), but with limited treatment efficacy. A study was undertaken to investigate whether treatment extension to 96 weeks improves the outcome in this patient population.128 HBeAg-negative patients (120 genotype D) were randomised to weekly 180 μg PegIFN for 48 weeks (group A, n=51), 180 μg PegIFN for 48 weeks followed by 135 μg weekly for an additional 48 weeks (group B, n=52) or 180 μg PegIFN plus lamivudine (100 mg/day) for 48 weeks then 135 μg PegIFN for 48 weeks (group C, n=25). Endpoints were alanine aminotransferase normalisation plus HBV DNA3400 IU/ml (primary), HBV DNA2000 IU/ml and HBsAg clearance at 48 weeks after treatment.Forty-eight weeks after treatment, six patients in group A and 13 in group B achieved alanine aminotransferase normalisation plus HBV DNA3400 IU/ml (11.8% vs 25.0%, p=0.08), 6 vs 15 patients had HBV DNA2000 IU/ml (11.8% vs 28.8%, p=0.03), 0 vs 3 achieved HBsAg clearance (0% vs 5.8%, p=0.24) and 0 vs 5 had HBsAg10 IU/ml (0% vs 9.6%, p=0.06). While extended PegIFN treatment was the strongest independent predictor of response, the combination with lamivudine did not improve responses. Discontinuation rates were similar among the groups (19.6%, 23.1%, 32.0%, p=0.81) and were mostly due to PegIFN-related adverse events.In HBeAg-negative genotype D patients with chronic hepatitis B, PegIFN treatment for 96 weeks was well tolerated and the post-treatment virological response improved significantly compared with 48 weeks of treatment.http://ClinicalTrials.gov registration number: NCT01095835.

In up to 80% of cases primary sclerosing cholangitis (PSC) is associated with inflammatory bowel diseases (IBD). The efficacy of azathioprine (AZA), in the maintenance of remission of IBD has been suggested by several studies. However, AZA tends to exter varied well-known toxicity. Since the rate of hepato-pancreatic side-effects in patients with IBD and PSC is still unclear, we investigated this issue.Consecutive subjects who underwent Outpatient Clinic admission for both IBD and PSC were included. Both conditions were diagnosed according to International Guidelines.Data of 43 patients were elaborated. Twelve of them underwent therapy with AZA. Five (41.7%) presented hepatic (n=4) or pancreatic toxicity. Eighty percent of the patients with hepato-pancreatic reactions versus 28.6% of those without (p0.001) were males, with 60% affected by ulcerative colitis and 40% by Crohn's disease versus 57% and 43%, respectively. Forty percent of patients with reactions versus 43% of those without needed an operation for IBD, and the same percentage underwent orthotopic liver transplantation, with a 100% versus 66.7% (p0.001) need of second transplantation. Colonic neoplasia (20%) was detected only in the former group while cholangiocarcinoma (28.6%) only in the latter.The occurrence of hepato-pancreatic reactions from AZA in our caseload is higher (41.7%) compared to that reported in literature (4%). Therefore, the presence of PSC, in association to IBD, may strongly affect AZA tolerability compared to presence of IBD only.

Background. Liver transplantation is the treatment of choice for end-stage liver disease in both adult and paediatric patients. The Italian experience in paediatric liver transplantation during the period 1988–1999 is reported herein. Patients and methods. This report concerns 228 liver transplantations performed in 207 patients (100 male, 107 female, mean age 5.1±4.4 years) in 11 Italian centres. The mean waiting time on the transplantation list was 6. 1±8.9 months and the main indications for the procedure were biliary atresia, inborn metabolic disorders, liver cirrhosis, liver neoplasms, Alagille syndrome, and fulminant hepatic failure. Results. The cumulative survival rate was 77%, 76%, 73%, and 71 at 1, 3, 5, and 7 years. The overall prevalence of acute rejection was 54%. Survival was significantly affected by re-transplantation (p=0.0002), by United Network for Organ Sharing 4 status at transplantation (p=0.016), and, among the indications for the procedure, by fulminant hepatic failure (p=0.004). Fifty patients (24%) died during the observation period. The main causes of death were primary non-function of the graft and sepsis Conclusions. This study shows that liver transplantation in paediatric age, in Italy, is an effective procedure providing a 5-year survival rate comparable to that attained in the largest published series.

Background. Liver transplantation is the standard treatment for patients with end-stage liver disease no longer responsive to conventional medical treatment Aims . To report the longterm experience of liver transplantation in Italy. Patients and Methods . Data were obtained retrospective by means of a multiple-item form collected from 15 Italian liver transplant centres. The filing centre was centralized. Results . A total of 3323 liver transplants were performed on 3026 patients, with a cumulative proportional survival of 72.4%. Three, 5 and 10 years' patient survival rates were 72.3%, 68.8% and 61.3%, respectively. The most common indication for liver transplantation were hepatitis B virus (± hepatitis D virus)- and hepatitis C virus-related cirrhosis (59.4%). Excellent survival rates were observed particularly in controversial indications, such as alcoholic cirrhosis, hepatitis B virus-related cirrhosis and hepatocellular carcinoma. Retransplantation was required in 8.9% of the cases. The overall prevalence of acute cellular rejection episodes was 43.5%. In our study population, primary non-function and disease recurrence were the most common causes of graft failure (28.7% and 25.4%, respectively). Infections and/or sepsis were the most common causes of death after transplantation (42%). Conclusion. This study confirms that patients with controversial indications to liver transplantation such as alcoholic cirrhosis, HBV-related cirrhosis and hepatocellular carcinoma can achieve excellent survival when properly selected.

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Endoscopic variceal ligation (EVL) is recommended for the treatment of esophageal variceal bleeding. The aim of this study was to assess the most cost-effective timing of endoscopic follow-up after variceal eradication.Cirrhotics with esophageal varices treated between January 2008 and January 2009 until reached variceal obliteration were retrospectively analyzed for technical aspects and for outcomes.Out of 127 patients treated with EVL, 103 were included. Number of sessions to achieve variceal obliteration and number of bands for each session were 2.8±1.3 (range 1-7) and 4.6±1 (range 2-7), respectively. The placement of5 bands per session was not associated with higher incidence of complications (19.6% vs. 17.8%, P=ns). Esophageal ulcers were observed in 42% of patients when the interbanding interval was20 days (versus 15% for interval20 days, P0.05). Once obliteration was achieved, varices reappeared in 28% of patients; the early appearance of small varices was not associated with bleeding.A longer interbanding interval reduces the incidence of procedural-related complications. After variceal obliteration an early endoscopic control is not useful because it does not influence the approach and does not change the patient outcome.

Worldwide, the hepatitis B virus (HBV) and the hepatitis C virus (HCV) cause, respectively, 600 000 and 350 000 deaths each year. Viral hepatitis is the leading cause of cirrhosis and liver cancer, which in turn ranks as the third cause of cancer death worldwide. Within the WHO European region, approximately 14 million people are chronically infected with HBV, and nine million people are chronically infected with HCV. Lack of reliable epidemiological data on HBV and HCV is one of the biggest hurdles to advancing policy. Risk groups such as migrants and injecting drug users (IDU) tend to be under-represented in existing prevalence studies; thus, targeted surveillance is urgently needed to correctly estimate the burden of HBV and HCV. The most effective means of prevention against HBV is vaccination, and most European Union (EU) countries have universal vaccination programmes. For both HBV and HCV, screening of individuals who present a high risk of contracting the virus is critical given the asymptomatic, and thereby silent, nature of disease. Screening of migrants and IDUs has been shown to be effective and potentially cost-effective. There have been significant advances in the treatment of HCV and HBV in recent years, but health care professionals remain poorly aware of treatment options. Greater professional training is needed on the management of hepatitis including the treatment of liver cancer to encourage adherence to guidelines and offer patients the best possible outcomes. Viral hepatitis knows no borders. EU Member States, guided by the EU, need to work in a concerted manner to implement lasting, effective policies and programmes and make tackling viral hepatitis a public health priority.

Celiac disease (CD) is a chronic, immune-mediated disorder, characterized by small intestinal malabsorption of nutrients after the ingestion of gluten by genetically susceptible individuals. The discovery of the wide variations in the nature and intensity of clinical presentation of CD has transformed its status, long considered a rare disease, to that of a common health problem. As patients with CD get older, they tend to present with complaints not directly referable to the gastrointestinal tract. Neurologic symptoms, caused by lesions of the central or peripheral nervous system occasionally occur in patients with CD and are poorly understood. This review focalizes on the present knowledge of the potential relationship between CD and epilepsy. The prevalence of CD among patients with epilepsy is not homogeneously distributed, probably because epilepsy encompasses a heterogeneous group of disorders. In fact, the clinical spectrum of epilepsy related to CD ranges from benign syndromes to intractable epilepsy. The precise mechanism of the potential association between CD and epilepsy is also still under discussion.

Aim: Endoscopic ultrasound (EUS) is a valuable diagnostic tool in pancreatic diseases and its high negative predictive value (NPV) in excluding malignancies is universally recognized. Moreover, EUS with fine-needle aspiration (EUS-FNA) can significantly impact on diagnosis and management of many clinical conditions. However, there are circumstances in which EUS-FNA cannot or should not be performed. We evaluated the factors that prevented us from performing or induced us not to perform FNA. Methods: The study was conducted in a tertiary university hospital. A total of 211 patients suspected of having solid pancreatic malignancy on the basis of clinical presentation and computed tomography and/or magnetic resonance imaging was included. When FNA was withheld because not deemed necessary by the operator, the NPV of EUS was calculated. Results: In 9 patients (4.3% of the procedures), FNA was withheld because of contraindications that should have been foreseen by the referring physician. In 30 subjects, FNA was not accomplished as no lesions requiring biopsy were actually found at EUS exploration. In this group, EUS reached a NPV of 96.7% in excluding malignancy, but it reached 100% in patients without chronic pancreatitis. Conclusion: In a cohort of patients with high pre-test probability of malignancy, the high NPV of EUS was confirmed. False negative results should be expected in patients with chronic pancreatitis and they need a strict follow-up.

Objective: the term "microscopic colitis" includes lymphocytic colitis (LC) and collagenous colitis, bearing common clinical presentation distinguishable only by histopathological examination of colonic biopsies. This study reports on demographic and clinical characteristics, and outcome of a cohort of patients with LC. Methods: demographic, clinical and histopathological data were reviewed. Every patient underwent total colonoscopy with multiple biopsies examined by an expert pathologist. Diagnosis of LC was confirmed if histopathological criteria were present. Routine laboratory tests were collected to rule out other diagnosis. Results: we included 80 patients (28 males; mean age: 46.4 years). At diagnosis, 71 patients (88%) reported diarrhea, 46 (58%) abdominal pain, 21 (36%) weight loss, 10 (13%) nausea. Regarding autoimmune or inflammatory diseases accompanying LC, thyroid disorders and celiac disease (CD) ranked first. Moreover, in over 10% of patients who underwent esophagogastroduodenoscopy, duodenal biopsies showed villi alterations classified as Marsh I damage, without clinical and serological data for diagnosis of CD. Mesalazine and oral topical steroids (budesonide or beclomethasone) were used to treat LC in 34 (43%) and 32 (39%) of patients, respectively, with similar percentages of clinical response (approximately 80%). Conclusions: the need for total colonoscopy with multiple biopsies in all patients with chronic watery diarrhea was confirmed. Since the association between CD and LC exists, additional tests should be performed in patients not responding to gluten-free diet or to LC specific therapy to exclude the other condition. Mesalazine obtained a similar outcome than oral steroids in this cohort.

Over the past fifteen years, numerous observations have linked Helicobacter pylori (H. pylori) infection to ischemic heart disease (IHD). Despite the controversial literature data, it has been postulated that if a role is plausible, it will be in the early events of the acute coronary syndrome. According to this model, we focused on the potential pathogenic mechanisms relating H. pylori to IHD like platelet aggregation and thrombosis. To identify all publications in this field, a MEDLINE search of studies published in English from 1965 to 2009 was conducted. Although very few investigations were found, these showed data of paramount importance. In particular, it has been demonstrated that some strains of H. pylori bind von Willebrand factor and interact with glycoprotein Ib to induce platelet aggregation in humans. In experiments from animal models, such infection promoted the formation of platelet aggregates by both a marked increase in the flux of rolling leukocytes and the appearance of platelet and leukocyte-platelet aggregates in gastric venules. This aggregate formation was abrogated by antibodies against specific adhesion molecules (L- and P-selectin). The future challenge is to gain more knowledge in this field and to translate these information into clinical practice.