A rare cause of acute liver failure due to haemophagocytic lymphohistiocytosis secondary to diffuse large B-cell lymphoma.

Acute liver failure is a life-threatening condition commonly caused by drug-induced hepatotoxicity or viral hepatitides. However, there are a number of rarer causes such as haemophagocytic lymphohistiocytosis. Haemophagocytic lymphohistiocytosis is a syndrome of uncontrolled immune cell activation, triggered by infection or malignancy, which carries a high mortality.

Mapping chronic liver disease questionnaire scores onto SF-6D utility values in patients with primary sclerosing cholangitis.

Purpose: The chronic liver disease questionnaire (CLDQ) is a frequently used liver-specific quality of life instrument, but it does not provide information on preference-adjusted health status, which is essential for cost-utility analysis. We aimed to develop a mapping function deriving utilities from the CLDQ in primary sclerosing cholangitis (PSC).

Methods: Short form-6D (SF-6D) utilities were calculated from SF-36 data collected in a recent prospective study in which unselected patients with PSC also completed the CLDQ.

Outcomes after ileal pouch anal anastomosis in patients with primary sclerosing cholangitis.

Background And Aims: Outcomes after ileal pouch anal anastomosis (IPAA) are not well established in patients with primary sclerosing cholangitis (PSC). We conducted a comprehensive outcomes assessment in these patients.

Methods: A retrospective case note review of complications in all PSC-IPAA (n=21) and matched ulcerative colitis patients with IPAA (UC-IPAA; n=79) after surgery in Oxford (1983-2012) was conducted, and functional outcomes (Öresland score) were evaluated (2012).

Cholangiocarcinoma complicating secondary sclerosing cholangitis from cryptosporidiosis in an adult patient with CD40 ligand deficiency: case report and review of the literature.

A 43-year-old man with a hyper-immunoglobulin M syndrome due to CD40 ligand deficiency presented with insidious onset of recurrent diarrhoea and deranged liver function tests. Standard stool microscopy was repeatedly negative for cryptosporidia but immunofluorescent testing and polymerase chain reaction demonstrated the presence of infection eventually. Despite both paromomycin and nitazoxanide, he developed sclerosing cholangitis secondary to cryptosporidial infection.

Factors that reduce health-related quality of life in patients with primary sclerosing cholangitis.

Background & Aims: Health-related quality of life (HRQL) is frequently reduced in patients with chronic liver disease, but there are limited data from patients with primary sclerosing cholangitis (PSC). We aimed to evaluate HRQL and its potential determinants in 2 population-based cohorts of patients with PSC and to study the prevalence of fatigue among these patients.

Methods: Validated questionnaires were used to measure quality of life (the Short-Form 36 [SF-36] and the chronic liver disease questionnaire), fatigue (the fatigue impact scale), and psychological distress (the hospital anxiety and depression scale) in 182 PSC patients residing in Sweden or England.

HCV genotype-3a T cell immunity: specificity, function and impact of therapy.

Background: Hepatitis C virus (HCV) genotype-3a infection is now the dominant strain in South Asia and the UK. Characteristic features include a favourable response to therapy; the reasons for this are unknown but may include distinct genotype-3a-specific T cell immunity. In contrast to genotype-1 infection, T cell immunity to this subtype is poorly defined.

The clinical impact of primary sclerosing cholangitis in patients with an ileal pouch-anal anastomosis for ulcerative colitis.

Introduction: Primary sclerosing cholangitis (PSC), a chronic inflammatory disease affecting the liver, is associated with ulcerative colitis (UC) in up to 75% of Northern European patients. These patients are at increased risk for the development of colorectal cancer, and the operation of choice is restorative proctocolectomy with an ileal pouch-anal anastomosis. However, complications such as pouchitis can occur, and studies have suggested that PSC is an independent risk factor for the development of pouchitis.