Reviews
Cutting edge issues in juvenile sclerosing cholangitis
Sclerosing cholangitis (SC) is a rare chronic disorder characterised by inflammation and progressive obliterative fibrosis of the intrahepatic and/or extrahepatic bile ducts. Diagnosis is based on cholangiogram showing bile duct dilatation, narrowing and obliteration of the biliary tree, and histologically, on the presence of inflammatory bile duct damage leading to periductal fibrosis. In children the most common SC is associated with strong autoimmune features, overlapping with those of autoimmune hepatitis (AIH); this form is known as autoimmune sclerosing cholangitis, ASC.Clinical relevance of skeletal muscle abnormalities in patients with cirrhosis
Recent advances in body composition evaluation have demonstrated abnormalities in skeletal muscle in patients with cirrhosis. Sarcopenia (severe muscle depletion) and myosteatosis (pathological fat accumulation in muscle) are prevalent muscle abnormalities in patients with cirrhosis that confer poor prognosis. Sarcopenia has become a well-defined factor for adverse clinical outcomes pre- and post-liver transplantation and emerging evidence has suggested the prognostic significance of myosteatosis in predicting mortality and overt hepatic encephalopathy in patients with cirrhosis.Recent updates on alcoholic hepatitis
Alcoholic hepatitis (AH) is a unique clinical syndrome that affects patients with chronic and active harmful alcohol consumption, and is associated with a high mortality of up to 40% at 1 month from presentation. It is important to assess disease severity and prognosis at time of presentation to identify patients at risk for high mortality and potential candidates for specific therapies. The cornerstone therapy for AH is enteral nutrition and abstinence. Steroids remain the only pharmacological option for severe AH however, adverse effects and lack of long-term benefit limit their routine use.Common issues in the management of patients in the waiting list and after liver transplantation
The present document contains the recommendations of an expert panel of transplant hepatologists, appointed by the Italian Association for the Study of the Liver (AISF), on how to manage the most common aspects of liver transplantation: the topics covered include: new treatments for HCV in patients on the waiting list for liver transplantation; antiviral treatments in patients with HCV recurrence after liver transplantation; prophylaxis for HBV recurrence after liver transplantation; indications for liver transplantation in alcoholic liver disease; and Immunosuppressive therapy.Liver graft preconditioning, preservation and reconditioning
Liver transplantation is the successful treatment of end-stage liver disease; however, the ischaemia-reperfusion injury still jeopardizes early and long-term post-transplant outcomes. In fact, ischaemia-reperfusion is associated with increased morbidity and graft dysfunction, especially when suboptimal donors are utilized. Strategies to reduce the severity of ischaemia-reperfusion can be applied at different steps of the transplantation process: organ procurement, preservation phase or before revascularization.Comparison of liver transplantation outcomes in biliary atresia patients with and without prior portoenterostomy: A meta-analysis
Portoenterostomy is currently the standard first procedure for biliary atresia, and liver transplantation is reserved as a complementary therapy for those with late diagnosis, rapid hepatic decompensation, or failed portoenterostomy. Many previous publications have analysed the impact of prior portoenterostomy on the clinical outcomes of liver transplantation and the conclusions are discordant.Position paper of the Italian Association for the Study of the Liver (AISF): The multidisciplinary clinical approach to hepatocellular carcinoma
Patients with hepatocellular carcinoma should be managed with a multidisciplinary approach framed in a network where all the diagnostic techniques and therapeutic resources are available in order to provide the optimal level of care. Given this assumption, the Coordinating Committee of the Italian Association for the Study of the Liver nominated a panel of experts to elaborate practical recommendations for the multidisciplinary management of hepatocellular carcinoma aiming to provide: (1) homogeneous and efficacious diagnostic and staging work-up, and (2) the best treatment choice tailored to patient status and tumour stage at diagnosis.Liver transplantation for the treatment of nodular regenerative hyperplasia
Nodular regenerative hyperplasia (NRH) is the leading cause of non-cirrhotic portal hypertension in Western countries. Although some patients are successfully managed medically or with shunting procedures, others require liver transplantation. The aim of this review was to assess the overall results obtained with liver transplantation and to better define its role in this setting.Liver transplantation for patients with alcoholic liver disease: An open question
End-stage alcoholic liver disease is a recognised indication for liver transplantation but some questions on the matter remain open. It is difficult to quantify alcohol consumption, and a single definition of post-transplant relapse is lacking. Moreover, there are no internationally accepted criteria for the selection of candidates for liver transplantation and the eligibility parameters for these patients are controversial. Additional clinical and psychological evaluations are necessary in this setting, especially to establish the risk of alcohol relapse.Practice guidelines for the treatment of hepatitis C: Recommendations from an AISF/SIMIT/SIMAST Expert Opinion Meeting
It is increasingly clear that a tailored therapeutic approach to patients with hepatitis C virus infection is needed. Success rates in difficult to treat and low-responsive hepatitis C virus patients are not completely satisfactory, and there is the need to optimise treatment duration and intensity in patients with the highest likelihood of response. In addition, the management of special patient categories originally excluded from phase III registration trials needs to be critically re-evaluated.Allocation priority in non-urgent liver transplantation: An overview of proposed scoring systems
Given the lack of donors, a correct organ allocation system for candidates to liver transplantation is essential to increase graft and patient survival. The most used organ allocation tools are Child–Turcotte–Pugh and model for end-stage liver disease. It is generally accepted that model for end-stage liver disease score is superior to the Child–Turcotte–Pugh classification in predicting the short-term survival of cirrhotic patients awaiting liver transplantation. Since 2002, model for end-stage liver disease is widely used for liver allocation.Neurological complications after orthotopic liver transplantation
The number of orthotopic liver transplantation performed each year is increasing due to increased safety and logistic facilities. Therefore, the importance of reducing adverse events is progressively growing.Wilson disease—A practical approach to diagnosis, treatment and follow-up
Wilson disease is an inherited, autosomal recessive, copper accumulation and toxicity disorder that affects about 30 individuals per million. This rare disease is caused by mutations in the gene encoding a copper-transporting P-type ATPase, which is important for copper excretion into bile, leading to copper accumulation in the liver. Toxic copper concentrations can also be found in the brain and kidney, and clinical phenotypes include hepatic, haemolytic, neurologic and psychiatric diseases. Diagnosis is based on the combination of clinical features and findings such as increased urinary copper excretion, reduced levels of serum ceruloplasmin, high concentrations of copper in liver tissues and Kayser–Fleischer rings.
