Results from an online survey of adults with cystic fibrosis: Accessing and using life expectancy information

Cystic fibrosis (CF) is the one of the most common inherited diseases. It affects around 10,000 people in the UK, and the median survival age is 47. Recent developments making use of longitudinal patient registry data are producing more detailed and relevant information about predicted life expectancy in CF based on current age and clinical measurements. The objective of this study was to conduct an online survey of adults with CF living in the UK using a web-based questionnaire to investigate: (i) if and how they access information on life expectancy; (ii) what they use it for; (iii) if they want more personalised information on life expectancy or the time until other milestones. The survey was advertised through the Cystic Fibrosis Trust using social media. There were 85 respondents, covering men (39%) and women (61%) aged 16–65. 75% had received information on life expectancy either from their CF care team (34%) or other sources (71%), the most common being the Cystic Fibrosis Trust website and research literature. Most people who received information found it to be beneficial and reported using it in a variety of ways, including to plan strategies for maintaining as best health as possible and to psychologically manage current health status. 82% of respondents were interested in more personalised information about their life expectancy, and participants also noted interest in other outcomes, including time to needing transplant or reaching a low level of lung function. Themes arising in text responses included the importance of good communication of information, the difficulty of relating general information to one’s own circumstances, and a desire for increased information on factors that impact on survival in CF. As an outcome from this work, research is underway to establish how information on life expectancy can be presented to people with CF in an accessible way

Contemporary temperature-driven divergence in a Nordic freshwater fish under conditions commonly thought to hinder adaptation

BACKGROUND: Evaluating the limits of adaptation to temperature is important given the IPCC-predicted rise in global temperatures. The rate and scope of evolutionary adaptation can be limited by low genetic diversity, gene flow, and costs associated with adaptive change. Freshwater organisms are physically confined to lakes and rivers, and must therefore deal directly with climate variation and change. In this study, we take advantage of a system characterised by low genetic variation, small population size, gene flow and between-trait trade-offs to study how such conditions affect the ability of a freshwater fish to adapt to climate change. We test for genetically-based differences in developmental traits indicating local adaptation, by conducting a common-garden experiment using embryos and larvae from replicate pairs of sympatric grayling demes that spawn and develop in natural cold and warm water, respectively. These demes have common ancestors from a colonization event 22 generations ago. Consequently, we explore if diversification may occur under severely constraining conditions. RESULTS: We found evidence for divergence in ontogenetic rates. The divergence pattern followed adaptation predictions as cold-deme individuals displayed higher growth rates and yolk conversion efficiency than warm-deme individuals at the same temperature. The cold-deme embryos had a higher rate of muscle mass development. Most of the growth- and development differences occurred prior to hatch. The divergence was probably not caused by genetic drift as there was a strong degree of parallelism in the divergence pattern and because phenotypic differentiation (Q(ST)) was larger than estimated genetic drift levels (microsatellite F(ST)) between demes from different temperature groups. We also document that these particular grayling populations cannot develop successfully at temperatures above 12°C, whereas other European populations can, and that increasing the muscle mass development rate comes at the cost of some skeletal trait development rates. CONCLUSIONS: This study shows that genetically based phenotypic divergence can prevail even under conditions of low genetic variation and ongoing gene flow. Furthermore, population-specific maximum development temperatures along with musculoskeletal developmental trade-offs may constrain adaptation

A Rare Cause of Low Back Pain: Report of a Tailgut Cyst

Tailgut cysts, also known as retrorectal cystic hamartomas, are rare developmental abnormalities that typically occur in the retrorectal space. They are believed to arise from remnants of the embryonic hindgut (Hjermstad and Helwig, 1988). They can present as incidental findings during routine examination but over half of patients are thought to present with symptoms. MRI has become the modality of choice to image these frequently misdiagnosed cysts. Biopsy is not recommended. Complete intact surgical excision is advised to avoid the potential complications of these cysts which include infection, fistula formation, and the possibility of malignant transformation (Hjermstad and Helwig (1988), Mathis et al. (2010)). We describe the case of a 46-year-old female who presented with a 6-month history of low back pain. CT and MRI imaging demonstrated a complex retrorectal lesion with supralevator and infralevator components. This was removed using a combined transperineal and transabdominal approach. Histology confirmed a tailgut cyst

Peritoneal Dialysis Catheters

In peritoneal dialysis, a well-functioning catheter is of great importance because a dysfunctional catheter may be associated with exit-site infection, peritonitis, reduced efficiency of dialysis, and overall quality of treatment, representing one of the main barriers to optimal use of peritoneal dialysis. This chapter reviews the literature on indications and contraindications for peritoneal dialysis, peritoneal dialysis catheter design and materials, the techniques of insertion, complications, and method of removal of dialysis catheters

Dynamic Magnetic Resonance Imaging Demonstrates the Integrity of Perineal Reconstruction following Cylindrical Abdominoperineal Excision with Reconstruction of the Pelvic Floor Using Porcine Collagen

A 72-year-old female presented with a six-month history of increased frequency of defecation, rectal bleeding, and severe rectal pain. Digital rectal examination and endoscopy revealed a low rectal lesion lying anteriorly. This was confirmed histologically as adenocarcinoma. Radiological staging was consistent with a T3N2 rectal tumour. Following long-course chemoradiotherapy repeat staging did not identify any metastatic disease. She underwent a laparoscopic cylindrical abdominoperineal excision with en bloc resection of the coccyx and posterior wall of the vagina with a negative circumferential resection margin. The perineal defect was reconstructed with Permacol (biological implant, Covidien) mesh. She had no clinical evidence of a perineal hernia at serial followup. Dynamic MRI images of the pelvic floor obtained during valsalva at 10 months revealed an intact pelvic floor. A control case that had undergone a conventional abdominoperineal excision with primary perineal closure without clinical evidence of herniation was also imaged. This confirmed subclinical perineal herniation with significant downward migration of the bowel and bladder below the pubococcygeal line. We eagerly await further evidence supporting a role for dynamic MR imaging in assessing the integrity of a reconstructed pelvic floor following cylindrical abdominoperineal excision

Effect of Soil Chemistry on Microbial Biodiversity and Functionality in Grassland and Tilled Soils

Microorganisms are excellent indicators of soil health, because of their rapid response to environmental change. Traditional microbiology is ineffective for the study of soil, as \u3c 1% of microorganisms are currently culturable (Torsvik et al., 1996). Nucleic acid based methods, however, allow rapid detection of organisms, or particular genes, directly from soil samples. This work investigated, using polymerase chain reaction (PCR)-based approaches, the relationship between key chemical properties and bacterial biodiversity in grassland and tilled soils, with particular emphasis on the abundance and diversity of ammonium oxidisers

Asymptotic normalization coefficients (nuclear vertex constants) for p+7Be→8Bp+^7Be\to ^8B and the direct 7Be(p,γ)8B^7Be(p,\gamma)^8B astrophysical S-factors at solar energies

A new analysis of the precise experimental astrophysical S-factors for the direct capture $^7Be(p,\gamma)$ $^8B$ reaction [A.J.Junghans et al.Phys.Rev. C 68 (2003) 065803 and L.T. Baby et al. Phys.Rev. C 67 (2003) 065805] is carried out based on the modified two - body potential approach in which the direct astrophysical S-factor, ${\rm S_{17}(E)}$, is expressed in terms of the asymptotic normalization constants for $p+^7Be\to ^8B$ and two additional conditions are involved to verify the peripheral character of the reaction under consideration. The Woods-Saxon potential form is used for the bound ($p+^7Be$)- state wave function and for the $p^7Be$- scattering wave function. New estimates are obtained for the ^{\glqq}indirectly measured\grqq values of the asymptotic normalization constants (the nuclear vertex constants) for the $p+^7Be\to ^8B$ and $S_{17}(E)$ at E$\le$ 115 keV, including $E$=0. These values of $S_{17}(E)$ and asymptotic normalization constants have been used for getting information about the ^{\glqq}indirectly measured\grqq values of the $s$ wave average scattering length and the $p$ wave effective range parameters for $p^7Be$- scattering.Comment: 27 pages, 6 figure

Fucoidan-degrading fungal strains: screening, morphometric evaluation, and influence of medium composition

Ten different fungal strains from the genus Aspergillus, Penicillium, and Mucor were screened for fucoidan hydrolyzing ability aiming to find microorganisms able to produce sulfated fucan-degrading enzymes. Screening was carried out by measuring the strains kinetic and morphometric behavior over plate assays using Laminaria japonica fucoidan as only carbon source, testing three nitrogen sources (urea, peptone, and sodium nitrate). The selected fungal strains were subsequently used in submerged fermentations, which were performed for (1) selection of the strains able to growth over fucoidan medium and (2) media selection, testing the synergy of fucoidan with other sugars for inducing high enzyme titles. Radial expansion and hyphae parameters were observed for Aspergillus niger PSH, Mucor sp. 3P, and Penicillium purpurogenum GH2 grown only over fucoidan-urea medium. A. niger PSH showed the maximum enzymatic activity values, which were significantly different (p<0.05) from those achieved by the other selected fungi. Sucrose addition to fucoidan media proportioned the highest fucoidanase activity values for this fungal strain. This research allowed establishing optimal conditions for metabolites synthesis by fungal stains able to act toward fucoidan ramified matrix.Mexican Science and Technology Council (CONACYT

Choosing party leaders: Anglophone democracies, British parties and the limits of comparative politics

Since 1965, Britain’s major political parties have radically, and repeatedly, changed the ways in which they choose their leaders. Building on a recent comparative study of party leadership selection in the five principal Anglophone (‘Westminster’) parliamentary democracies (Cross and Blais, 2012a), this article first outlines a theoretical framework that purports to explain why the major parties in three of those countries, including Britain, have adopted such reform. It then examines why five major British parties have done so since 1965. It argues that, while Cross and Blais’ study makes a significant contribution to our knowledge and understanding of processes of party leadership selection reform in Anglophone parliamentary democracies, it has limited explanatory power when applied to changes enacted by the major parties in modern and contemporary Britain. Instead, the adoption of such reform in the British context is ultimately best understood and explained by examining both the internal politics and external circumstances of individual parties

Treating Solar Model Uncertainties: A Consistent Statistical Analysis of Solar Neutrino Models and Data

We describe how to consistently incorporate solar model uncertainties, along with experimental errors and correlations, when analyzing solar neutrino data to derive confidence limits on parameter space for proposed solutions of the solar neutrino problem. Our work resolves ambiguities and inconsistencies in the previous literature. As an application of our methods we calculate the masses and mixing angles allowed by the current data for the proposed MSW solution using both Bayesian and frequentist methods, allowing purely for solar model flux variations, to compare with previous work. We consider the effects of including metal diffusion in the solar models and also discuss implications for future experiments.Comment: 29 pages (incl figs), latex, 6 figures (appended as separate uuencoded file. To embed figures in text, uncomment 6 \epsfysize lines which appear before bibliography), CWRU-P5-94, CfPA-94-TTH-29, Fermilab-Pub-94/176-