Role for mycobacterial infection in pathogenesis of primary biliary cirrhosis?

Primary biliary cirrhosis (PBC) is a progressive cholestatic liver disease characterized by the immune-mediated destruction of biliary epithelial cells in small intrahepatic bile ducts. The disease is characterized by circulating antimitochondrial antibodies (AMAs) as well as disease-specific antinuclear antibodies, cholestatic liver function tests, and characteristic histological features, including granulomas. A variety of organisms are involved in granuloma formation, of which mycobacteria are the most commonly associated.

Role of disease severity, illness perceptions, and mood on quality of life in muscle disease.

Introduction: The aim of this study was to describe the effect of muscle disease upon QoL and to explore the influence of disease severity, mood, and illness perception on the QoL of these patients.

Methods: Validated questionnaires assessing QoL (Individualized Neuromuscular QoL and SF-36), disease severity (Health Assessment Questionnaire), mood (Hospital Anxiety and Depression Scale) and illness perception (Illness Perception Questionnaire) were sent to adults with muscle disease.

Results: We received 302 responses (return rate 75%).

Familial primary biliary cirrhosis: like mother, like daughter?

The reasons underlying why autoimmune diseases overwhelmingly affect women more than men are not clear. Nor are the reasons why autoimmune disease is more prevalent in families. This review uses primary biliary cirrhosis (PBC) as a model autoimmune disease to discuss the familial risk, focusing mainly on mother-daughter pairs.

Immunopathogenesis of primary biliary cirrhosis: an old wives' tale.

Primary biliary cirrhosis (PBC) is a cholestatic liver disease characterised by the autoimmune destruction of the small intrahepatic bile ducts. The disease has an unpredictable clinical course, but may progress to fibrosis and cirrhosis. Although medical treatment with urseodeoxycholic acid is largely successful, some patients may progress to liver failure requiring liver transplantation.

Skeletal muscle dysfunction in critical care: wasting, weakness, and rehabilitation strategies.

Understanding the trajectory of skeletal muscle loss, evaluating its relationship to the subsequent functional impairment, and understanding the underlying mechanisms of skeletal muscle wasting will provide goals for novel treatment strategies in the intensive care setting. A focused approach on the effect of critical illness on muscle morphology, muscle protein turnover, and the associated muscle-signaling pathways during the early and recovery stages of critical illness is required. This could potentially lead to targeted pharmacologic and nonpharmacologic strategies to treat, or even prevent, peripheral muscle wasting and weakness.

Perivascular cells as mesenchymal stem cells.

Importance Of The Field: Mesenchymal stem cells are multipotent adult stem cell populations that have broad differentiation plasticity and immunosuppressive potential that render them of great importance in cell-based therapies. They are identified by in vitro characteristics based on their differentiation potential for clinical approaches while their biological properties and in vivo identities are often less understood.

Areas Covered In This Review: Recent research carried out in the last decade on mesenchymal stem cell biology suggests that mesenchymal stem cells from various tissues reside in a perivascular location and these can be identified as pericytes that function as mural cells in microvessels.

Allogeneic hematopoietic stem-cell transplantation for patients 50 years or older with myelodysplastic syndromes or secondary acute myeloid leukemia.

Purpose: This study was performed to examine the characteristics of transplant activity for patients with myelodysplastic syndromes (MDS) older than 50 years within the European Group for Blood and Marrow Transplantation, and to evaluate the factors predicting outcome within this group of patients.

Patients And Methods: We performed a retrospective multicenter analysis of 1,333 MDS patients age 50 years or older who received transplantation within the EBMT since 1998. The median recipient age was 56 years, with 884 patients (66%) age 50 to 60 years and 449 (34%) patients older than 60 years.

Changing the treatment paradigm in myelodysplastic syndromes.

The treatment algorithm for the patient with myelodysplastic syndrome (MDS) is in the process of being revitalized based on recent results of clinical trials. Historically, the goal for lower-risk patients was hematologic improvement, and disease modification was reserved for patients in the higher-risk category. Recent data now favor shifting emphasis away from supportive care alone and toward altering the disease course and prolonging survival, particularly in patients with intermediate-2 and high-risk disease.

Urodynamic assessment of poor responders after botulinum toxin-A treatment for overactive bladder.

Objectives: Botulinum toxin-A (BTX-A) is effective in treating overactive bladder (OAB) refractory to anticholinergics. Most patients have improvements in symptoms, urodynamic parameters, and quality of life, and a poor response is uncommon. The aim of this study was to determine whether poor responders could be predicted from preoperative urodynamic parameters.

Outcome of BEAM-autologous and BEAM-alemtuzumab allogeneic transplantation in relapsed advanced stage follicular lymphoma.

The role of haematopoietic stem cell transplantation (HSCT) in relapsed follicular lymphoma remains controversial. This study analysed 126 patients with relapsed, advanced stage follicular lymphoma who received BEAM (BCNU [carmustine], cytarabine, etoposide, melphalan)-alemtuzumab allogeneic HSCT (BEAM-allo) (n = 44) or BEAM-autologous HSCT (BEAM-auto) (n = 82). The BEAM-allo group had a younger median age (48 years vs.

Robotic approaches to renal cancer.

Purpose Of Review: Robotic-assisted laparoscopic surgery has had considerable impact on the management of localized prostate cancer. The role of robot assistance in laparoscopic renal surgery is less well defined, however.

Recent Findings: There have been few recently published papers regarding the robotic approach to management of renal cancer.

Clonal gammopathies following alemtuzumab-based reduced intensity conditioning haematopoietic stem cell transplantation: association with chronic graft-versus-host disease and improved overall survival.

The presence of clonal gammopathies (CG) has been reported following both conventional myeloablative and autologous haematopoietic stem cell transplantation (HSCT). We monitored the occurrence of CG in a cohort of patients with myeloid malignancies receiving FBC (fludarabine-busulphan-alemtuzumab)-based reduced intensity conditioned (RIC) HSCT, and assessed its correlation with infections, graft-versus-host disease (GvHD) and survival. Serial serum protein electrophoresis was analysed in a total of 138 patients and CG were detected in 49 patients (36%).

Delayed attainment of full donor chimaerism following alemtuzumab-based reduced-intensity conditioning haematopoeitic stem cell transplantation for acute myeloid leukaemia and myelodysplastic syndromes is associated with improved outcomes.

This prospective study evaluated the kinetics of lymphoid (CD3) engraftment in 110 patients with acute myeloid leukaemia (AML) and myelodysplastic syndromes (MDS) after allogeneic transplantation and conditioning with fludarabine, busulphan and alemtuzumab, using ciclosporin for post-transplant immunosuppression. Declining donor CD3 chimaerism beyond day+100 was treated with pre-emptive donor lymphocyte infusion (pDLI). The median age of patients was 53.

Impaired executive functioning in young adults born very preterm.

Individuals born very preterm (VPT) are at increased risk of perinatal brain injury and long-term cognitive and behavioral problems. Executive functioning, in particular, has been shown to be impaired in VPT children and adolescents. This study prospectively assessed executive function in young adults who were born VPT (<33 weeks of gestation) [n = 61; mean age, 22.

Outcomes of patients with haematological malignancies admitted to intensive care unit. A comparative review of allogeneic haematopoietic stem cell transplantation data.

The outcomes of 55 consecutive haemato-oncology patients admitted to the intensive care unit (ICU) were retrospectively analysed. Twenty-eight patients were admitted following haemopoietic stem cell transplantation (HSCT). Thirty-nine patients were admitted with respiratory failure, and all patients required respiratory support.

Outcomes of alemtuzumab-based reduced intensity conditioning stem cell transplantation using unrelated donors for myelodysplastic syndromes.

This prospective study evaluated the outcomes of 75 successive patients receiving a FBC (fludarabine, busulphan, alemtuzumab) reduced-intensity conditioning (RIC) regimen for myelodysplastic syndromes (MDS) using volunteer unrelated donors(VUD). The prognostic significance of a variety of clinical variables including the recently described haematopoietic cell transplantation co-morbidity index (HCT-CI) was assessed. The median age of the cohort was 52.

Successful treatment of refractory angioimmunoblastic T-cell lymphoma with thalidomide and dexamethasone.

Angioimmunoblastic T cell lymphoma (AITL) is a peripheral T-cell lymphoma characterized morphologically by lymphadenopathy with a polymorphic infiltrate, marked vascular and follicular dendritic cell proliferation. Patients usually present with advanced disease and the overall prognosis is poor. While intensive chemotherapy has been shown to induce complete remissions in 50-70% of patients, the majority of patients subsequently relapse.

Toxoplasmosis following alemtuzumab based allogeneic haematopoietic stem cell transplantation.

Alemtuzumab is a humanised monoclonal antibody against CD52 used as an immunosuppressive agent in allogeneic HSCT. Regimens including alemtuzumab for allogeneic haematopoietic stem cell transplant (HSCT) conditioning have been associated with an increased incidence of viral complications. Patients with prior toxoplasma exposure undergoing alemtuzumab containing HSCT could therefore be expected to be at a higher risk of toxoplasma reactivation.

Allogeneic transplantation for myelodysplastic syndrome (MDS).

Haematopoietic stem cell transplantation (HSCT) remains the only curative option for patients with myelodysplastic syndrome (MDS). Developing conditioning regimens with low toxicity, at the same time as preserving an effective graft versus tumour response, is pivotal to expanding the scope for allogeneic transplantation in older patients with MDS. With the introduction of reduced intensity conditioned regimens, transplant centres worldwide are able to offer allogeneic HSCT to a much larger cohort of patients.

Patient confidentiality: implications for teaching in undergraduate medical education.

Respect for patient confidentiality is one of the core concepts of professionalism and should have high priority in medical education. Confidentiality should be introduced early in the curriculum so that students understand their ethical, professional, and legal obligations throughout their medical studies and later professional career. Anatomists have important opportunities for teaching professional values including confidentiality and should be major contributors to a multidisciplinary teaching on professionalism.

Evidence in action: fostering growth of research-based practice in children's cancer nursing.

It is an honour to be the first children's nurse to be asked to deliver the Robert Tiffany Lecture. However, the invite also provoked some anxiety: the responsibility weighs heavy. An opportunity to both celebrate the success of the speciality and explore the challenges that still face children's cancer nurses could not be missed.