Survival and associated factors in 268 adults with Pompe disease prior to treatment with enzyme replacement therapy

Background: Pompe disease is a rare lysosomal storage disorder characterized by muscle weakness and wasting. The majority of adult patients have slowly progressive disease, which gradually impairs mobility and respiratory function and may lead to wheelchair and ventilator dependency. It is as yet unknown to what extent the disease reduces the life span of these patients. Our objective was to determine the survival of adults with P

Performance measures for upper gastrointestinal endoscopy:A European Society of Gastrointestinal Endoscopy (ESGE) Quality Improvement Initiative

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Bereavement help-seeking following an 'expected' death: a cross-sectional randomised face-to-face population survey

© 2008 Currow et al; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Background : This study examines the prevalence and nature of bereavement help-seeking among the population who experienced an "expected" death in the five years before their survey response. Such whole population data are not limited by identification through previous access to specific services nor practitioners. Methods : In a randomised, cross-sectional, state-wide population-based survey, 6034 people over two years completed face-to-face interviews in South Australia by trained interviewers using piloted questions (74.2% participation rate). Respondent demographics, type of grief help sought, and circumstantial characteristics were collected. Uni- and multi-variate logistic regression models were created. Results : One in three people (1965/6034) had experienced an 'expected' death of someone close to them in the last five years. Thirteen per cent sought help for their grief from one or more: friend/family members (10.7%); grief counselors (2.2%); spiritual advisers (1.9%); nurses/doctors (1.5%). Twenty five respondents (1.3%) had not sought, but would have valued help with their grief. In multi-variate regression modeling, those who sought professional help (3.4% of the bereaved) had provided more intense care (OR 5.39; CI 1.94 to14.98; p < 0.001), identified that they were less able to 'move on' with their lives (OR 7.08; CI 2.49 to 20.13; p = 0.001) and were more likely not to be in full- or part-time work (OR 3.75; CI 2.31 – 11.82; p = 0.024; Nagelkerke's R2 = 0.33). Conclusion : These data provide a whole-of-population baseline of bereavement help-seeking. The uniquely identified group who wished they had sought help is one where potentially significant health gains could be made as we seek to understand better any improved health outcomes as a result of involving bereavement services

Dengue Dynamics in Binh Thuan Province, Southern Vietnam: Periodicity, Synchronicity and Climate Variability

Dengue has become a major international public health problem due to increasing geographic distribution and a transition from epidemic transmission with long inter-epidemic intervals to endemic transmission with seasonal fluctuation. Seasonal and multi-annual cycles in dengue incidence vary over time and space. We performed wavelet analyses on time series of monthly notified dengue cases in Binh Thuan province, southern Vietnam, from January 1994 to June 2009. We observed a continuous annual mode of oscillation with a non-stationary 2–3-year multi-annual cycle. We used phase differences to describe the spatio-temporal patterns which suggest that the seasonal wave of infection was either synchronous with all districts or moving away from Phan Thiet district, while the multi-annual wave of infection was moving towards Phan Thiet district. We also found a strong non-stationary association between ENSO indices and climate variables with dengue incidence. We provided insight in dengue population transmission dynamics over the past 14.5 years. Further studies on an extensive time series dataset are needed to test the hypothesis that epidemics emanate from larger cities in southern Vietnam

Hepatitis C Virus Antigenic Convergence

Vaccine development against hepatitis C virus (HCV) is hindered by poor understanding of factors defining cross-immunoreactivity among heterogeneous epitopes. Using synthetic peptides and mouse immunization as a model, we conducted a quantitative analysis of cross-immunoreactivity among variants of the HCV hypervariable region 1 (HVR1). Analysis of 26,883 immunological reactions among pairs of peptides showed that the distribution of cross-immunoreactivity among HVR1 variants was skewed, with antibodies against a few variants reacting with all tested peptides. The HVR1 cross-immunoreactivity was accurately modeled based on amino acid sequence alone. The tested peptides were mapped in the HVR1 sequence space, which was visualized as a network of 11,319 sequences. The HVR1 variants with a greater network centrality showed a broader cross-immunoreactivity. The entire sequence space is explored by each HCV genotype and subtype. These findings indicate that HVR1 antigenic diversity is extensively convergent and effectively limited, suggesting significant implications for vaccine development

Illness cognitions in head and neck squamous cell carcinoma: predicting quality of life outcome

Goals of work: This paper presents an observational study of the longitudinal effects of cancer treatment on quality of life (QoL) in patients treated for head and neck squamous cell carcinoma (HNSCC), and evaluated the contribution of patients' baseline illness cognitions to the prediction of QoL 2 years after diagnosis. Patients and methods: One hundred seventy-seven patients eligible for primary treatment for HNSCC completed the Illness Perception Questionnaire-Revised at baseline and the European Organization for Research and Treatment of Cancer Core Quality of Life Questionnaire-30 at baseline, at 1-year and 2-year follow-ups. Main results Compared to baseline, patients reported better emotional functioning at both follow-ups (p<0.001), worse social functioning at 12 months (p<0.05), and better global health

Targeting histone deacetyalses in the treatment of B- and T-cell malignancies

HDAC inhibitors (HDACI) are now emerging as one of the most promising new classes of drugs for the treatment of select forms of non-Hodgkin’s lymphoma (NHL). They are particularly active in T-cell lymphomas, possibly hodgkin’s lymphoma and indolent B cell lymphomas. Presently, two of these agents, vorinostat and romidepsin, have been approved in the US for the treatment of relapsed and refractory cutaneous T cell lymphomas (CTCL). Initially, these agents were developed with the idea that they affected transcriptional activation and thus gene expression, by modulating chromatin condensation and decondensation. It is now clear that their effects go beyond chromatin and by affecting the acetylation status of histones and other intra-cellular proteins, they modify gene expression and cellular function via multiple pathways. Gene expression profiles and functional genetic analysis has led to further understanding of the various molecular pathways that are affected by these agents including cell cycle regulation, pathways of cellular proliferation, apoptosis and angiogenesis all important in lymphomagenesis. There is also increasing data to support the effects of these agents on T cell receptor and immune function which may explain the high level of activity of these agents in T cell lymphomas and hodgkin’s lymphoma. There is ample evidence of epigenetic dysregulation in lymphomas which may underlie the mechanisms of action of these agents but how these agents work is still not clear. Current HDAC inhibitors can be divided into at least four classes based on their chemical structure. At present several of these HDAC inhibitors are in clinical trials both as single agents and in combination with chemotherapy or other biological agents. They are easy to administer and are generally well tolerated with minimal side effects. Different dosing levels and schedules and the use of isospecific HDAC inhibitors are some of the strategies that are being employed to increase the therapeutic effect of these agents in the treatment of lymphomas. There may also be class differences that translate into specific activity against different lymphoma. HDAC inhibitors will likely be incorporated into combinations of targeted therapies both in the upfront and relapsed setting for lymphomas