Long-term impact of liver transplantation on respiratory function and nutritional status in children and adults with cystic fibrosis.

Early liver transplant (LT) has been advocated for patients with cystic fibrosis liver disease (CFLD) and evidence of deterioration in nutritional state and respiratory function to prevent further decline. However, the impact of single LT on long-term respiratory function and nutritional status has not been adequately addressed. We performed a retrospective analysis of the outcomes of 40 (21 adult/19 pediatric) patients with CFLD transplanted between 1987 and 2009 with median follow-up of 47.8 months (range 4-180). One and five-year actuarial survival rates were 85%/64% for adult and 90%/85% for pediatric LT cohorts, respectively. Lung function remained stable until 4 years (FEV(1) % predicted; pretransplant 48.4% vs. 45.9%, 4 years posttransplant) but declined by 5 years (42.4%). Up to 4 years posttransplant mean annual decline in FEV(1) % was lower (0.74%; p = 0.04) compared with the predicted 3% annual decline in CF patients with comorbidity including diabetes. Number of courses of intravenous antibiotics was reduced following LT, from 3.9/year pretransplant to 1.1/year, 5 years posttransplant. Body mass index was preserved posttransplant; 18.0 kg/m(2) (range 15-24.3) pretransplant versus 19.6 kg/m(2) (range 16.4-22.7) 5 years posttransplant. In conclusion, LT is an effective treatment for selected patients with cirrhosis due to CFLD, stabilizing aspects of long-term lung function and preserving nutritional status.

Current therapeutic strategies in non-alcoholic fatty liver disease.

Non-alcoholic fatty liver disease (NAFLD) encompasses a spectrum of disease ranging from simple steatosis through steatohepatitis (NASH) to increasing fibrosis and eventual cirrhosis. NAFLD is the hepatic manifestation of the metabolic syndrome and has now become the most common cause of liver disease in Western countries, with the more advanced stages of disease being associated with an increased risk of liver-related morbidity and mortality. The optimal management of patients with NAFLD remains a clinical challenge. The aim of this study is to describe established and emerging strategies for the treatment of NAFLD. Relevant research and review articles were identified by searching PubMed. Selected articles referenced in these publications were also examined. Good quality randomized controlled studies have demonstrated the need for multifaceted lifestyle interventions in patients with NAFLD including the need for diet, exercise and behavioural counselling. Despite several trials of pharmacological agents, no highly effective treatment yet exists, with surgery representing the mainstay for advanced disease. A multidisciplinary approach, with a major focus on lifestyle change, represents best treatment pending the development of new therapeutic options.

Systematic review: the diagnosis and staging of non-alcoholic fatty liver disease and non-alcoholic steatohepatitis.

BACKGROUND: Non-alcoholic fatty liver disease (NAFLD) has become the most prevalent cause of liver disease in Western countries. The development of non-alcoholic steatohepatitis (NASH) and fibrosis identifies an at-risk group with increased risk of cardiovascular and liver-related deaths. The identification and management of this at-risk group remains a clinical challenge. AIM: To perform a systematic review of the established and emerging strategies for the diagnosis and staging of NAFLD. METHODS: Relevant research and review articles were identified by searching PubMed, MEDLINE and EMBASE. RESULTS: There has been a substantial development of non-invasive risk scores, biomarker panels and radiological modalities to identify at-risk patients with NAFLD without recourse to liver biopsy on a routine basis. These modalities and algorithms have improved significantly in their diagnosis and staging of fibrosis and NASH in patients with NAFLD, and will likely impact on the number of patients undergoing liver biopsy. CONCLUSIONS: Staging for NAFLD can now be performed by a combination of radiological and laboratory techniques, greatly reducing the requirement for invasive liver biopsy.