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Background: Data on survival and safety of sorafenib for hepatocellular carcinoma recurrence after liver transplant are still equivocal., Aim: We performed a meta-analysis of published studies, with the aim of estimating the 1-year rates of survival, analysing the variability in survival rates and, finally, identifying the factors associated with a longer survival., Methods: Data from 8 of the 17 selected studies were pooled, while the other 9 were excluded because survival rates were missing. All included studies were retrospective., Results: Overall, the 1-year survival ranged from 18% to 90%. Tumour progression was the main cause of death. The second cause was bleeding, reported only in patients undergoing m-Tor inhibitor therapy. The pooled estimate of 1-year survival was 63%. There was a significant heterogeneity among studies (P < 0.0001). Among the 34 variables assessed by univariate meta-regression, 5 were associated with an increase in the 1-year survival rate: (1) male gender (P = 0.001); (2) Time to progression (P = 0.038); and adverse drug events, divided in (3) gastrointestinal (P = 0.038), (4) cardiovascular (P = 0.029), and (5) dermatological (P = 0.014)., Conclusions: Additional data from multicentre prospective studies are required to clearly determine if sorafenib is a safe and acceptable treatment in hepatocellular carcinoma recurrence after liver transplant. Nevertheless, its association with m-Tor inhibitors should be discouraged., (Copyright © 2015 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.)

The topic of this paper is to report an update on management of Budd-Chiari syndrome (BCS). Actually, the flow-chart of BCS management comes from experts opinion and is not evidence-based due to the rarity of BCS. Management of BCS follows a step-wise strategy. Anticoagulation and medical therapy should be the first line treatment. Revascularization or TIPS in case of no response to medical therapy. OLT as a rescue therapy. Surgery has limited but important space, especially in cases with high inferior vena cava obstruction not suitable for endovascular treatment. However, no clear indication can actually be given about the timing of different treatments. Moreover, there is some concern about treatment of some subgroup of patients, especially regarding the risk of recurrence after liver transplantation. This paper propose a new algorithm of BCS management suggesting an earlier therapeutic approach when clinical signs are evident.

Portopulmonary hypertension (PPHTN) is a known complication of cirrhosis. Moderate-to-severe PPHTN implies an extremely poor prognosis. It occurs in 5%-10% of patients referred for liver transplantation (LT), and probably with an higher incidence in patients with large portosystemic shunts. Patients with moderate-to-severe pulmonary hypertension have been previously excluded from LT because of the extremely high surgical risk and since the post-transplant outcome reported was poor. Recently, new perspectives in the management of patients with portopulmonary hypertension are emerging. In fact, some pulmonary vasoactive drugs have become routine in the treatment of patients with idiopathic pulmonary hypertension. These drugs, particularly epoprostenol, have been recently introduced in the treatment of patients with PPHTN, and have been shown to be effective in reducing pulmonary artery pressure as well as pulmonary vascular resistances. Furthermore, recent studies seem to demonstrate that treatment with pulmonary vasoactive drugs could allow liver transplantation with acceptable surgical risks and excellent survival. Although there are not large series nor prospective studies addressing this topic, the clinical scenario of patients with PPHTN seems to be positively changing., (© 2013 Baishideng Publishing Group Co., Limited. All rights reserved.)

Aims: The aim of this study was to assess the safety and efficacy of sorafenib, with or without everolimus, in the treatment of recurrent hepatocellular carcinoma (HCC) after an orthotopic liver transplantation (OLT)., Methods: We reviewed the outcome of our consecutive cohort series of patients. Eleven patients (nine men) with recurrent HCC after OLT were treated. Four patients received cyclosporine plus sorafenib at a starting dose of 400 mg twice daily; seven received the combination of sorafenib (same dosage) and everolimus. Sorafenib was reduced or stopped according to the drug label., Results: The median time to recurrence was 12 months (range 2-66). The mean age at the start of treatment was 57 ± 9 years. Sorafenib was withdrawn because of intolerance or side-effects in four (36%) patients. Dose reduction because of adverse events or intolerance was required in 91% of patients after 26 ± 11 days from the start of treatment. The average length of treatment was 68 days (range 15-444). One patient died because of a massive gastrointestinal bleeding while receiving sorafenib and everolimus. The most frequent adverse events were fatigue (54%), skin toxicity (45%), and hypophosphatemia (36%). Two patients (18%) showed a radiological partial response, one (9%) had a stable disease, and six (54%) showed a progressive disease. None of the patients achieved a complete response. Treatment response could not be assessed in two (18%) patients. The overall median survival since the start of treatment was 5 months. One-year survival was 18%., Conclusion: Sorafenib, with or without mammalian target of rapamycin inhibitors, is poorly tolerated and rarely effective in the treatment of recurrent HCC after OLT.

Background: Hepatocellular carcinoma as a complication is linked to improved outcomes of thalassemia.Main Body: Published data suggest an incidence of HCC in thalassemia of about 2%. However, since thalassemia is endemic in many under-developed countries where patients have not probably been screened for HCC yet, the burden of the disease could be higher. Prevention of HCV infection through blood transfusion, HCV treatment and adequate iron chelation are all tools to prevent HCC in thalassemia. In presence of risk factors, HCC screening seems appropriate for thalassemia. Management of HCC should not be different from that indicated for non thalassemics. However, liver transplantation can be challenging and should be reserved to highly selected cases, due to coexistence of relevant comorbidities. Decisions in the management of HCC in thalassemia should follow a multidisciplinary effort. Moreover, due to the paucity of published data about the issue, future multicenter international studies will be helpful.Short Conclusion: In BMC Gastroenterology results of a commendable effort to guidelines for the management of HCC in thalassemia are reported by an Italian panel of experts. However, due to the paucity of published data about the topic, some conclusions rely on grey areas and are reason of debate. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article "Hepatocellular carcinoma as an emerging morbidity in the thalassemia syndromes: a comprehensive review," by H. M. Moukhadder and colleagues.