European Cystic Fibrosis Society Standards of Care: Framework for the Cystic Fibrosis Centre

A significant increase in life expectancy in successive birth cohorts of people with cystic fibrosis (CF) is a result of more effective treatment for the disease. It is also now widely recognized that outcomes for patients cared for in specialist CF Centres are better than for those who are not. Key to the effectiveness of the specialist CF Centre is the multidisciplinary team (MDT), which should include consultants, clinical nurse specialist, microbiologist, physiotherapist, dietitian, pharmacist, clinical psychologist, social worker, clinical geneticist and allied healthcare professionals, all of whom should be experienced in CF care. Members of the MDT are also expected to keep up to date with developments in CF through continued professional development, attendance at conferences, auditing and involvement in research. Specialists CF Centres should also network with other Centres both nationally and internationally, and feed Centre data to registries in order to further the understanding of the disease. This paper provides a framework for the specialist CF Centre, including the organisation of the Centre and the individual roles of MDT members, as well as highlighting the value of CF organisations and disease registries

Assessment of MRI scanner performance for preclinical functional studies

Functional Magnetic Resonance Imaging (fMRI) based studies are rapidly expanding in the field of preclinical research. The majority of these studies use Echo Planar Imaging (EPI) to measure Blood Oxygenation Level Dependent (BOLD) signal contrasts in the brain. In such studies the magnitude and statistical significances of these contrasts are then related to brain function and cognition. It is assumed that any observed signal contrast is ultimately due to differences in biological state and that scanner performance is stable and repeatable between subjects and studies. However, due to confounding issues introduced by in vivo subjects, little work has been undertaken to test this basic assumption. As the BOLD signal contrasts generated in such experiments are often very low, even small changes in scanner performance may dominate the BOLD contrast, distorting any biological conclusions drawn. A series of fMRI phantoms were produced to measure scanner performance independent of biological subjects. These phantoms produce specified signal contrast levels on demand during an fMRI scan by means of current-induced magnetic field gradients. These were used to generate data sets that emulated the BOLD signal contrast of in vivo imaging. Two studies examining scanner performance were then conducted on high-field preclinical MRI scanners. Firstly, in a longitudinal study on a single scanner, measurements were taken over a number of days across a week long period and then every two months over a year long period. Secondly, the behaviour of four preclinical scanners (three at 7T, one at 9.4T) was comparatively assessed. Measurements of several imaging parameters including contrast generated and functional contrast to noise ratio (fCNR) were obtained in both studies. If the scanners involved are truly comparable then they should generate similar measurement values. Across both studies parameter measurements showed significant differences for identical contrast settings on the phantom. Although signal contrast itself proved very comparable across the studies fCNR proved to be highly variable. As well as these measurements of longer tem behaviour proving variable, short and mid-term signal stability displayed a wide range of variability. Variations in the level and quality of both signal and noise were observed. Modelling of signal changes based on fundamental physical principles was also performed for comparison. The impact of these behaviours and variations on in vivo studies could result in skewed biological conclusions at any single site, with some sites exhibiting greater problems than others. The multisite results suggest potential difficulties when comparing biological conclusions between sites, even when using identical imaging parameters. In summary, these results suggest that a cautious approach should be taken with the conclusions of both fMRI and associated resting state connectivity studies that use EPI as their acquisition sequence. Improvements to both the experimental design of studies and regular quality monitoring of scanners should be undertaken to minimise these effects. Clinical MRI scanners should also be assessed for similar aberrations in behaviour

ECFS best practice guidelines: the 2018 revision

Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field. In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe

Why so few? : women academics in Australian universities

This book had its genesis in the new wave of consciousness about women's position in society and the women's movement of the 1970s. Its aim is to contribute to the interest in and knowledge about Australian women through a study of a group who share the common situation of women in societies based on a sexual division of roles but who work in the particular locus of a university where they are a minority in both number and status

Conducting large-scale surveys in secondary schools: the case of the Youth On Religion (YOR) project

There are few published articles on conducting large-scale surveys in secondary schools, and this paper seeks to fill this gap. Drawing on the experiences of the Youth On Religion project, it discusses the politics of gaining access to these schools and the considerations leading to the adoption and administration of an online survey. It is concluded that successful research in schools has to be planned carefully in collaboration with key members of staff, and justified as an educational activity. Providing speedy feedback was helpful to ensure schools benefited from the research and to keep them engaged with the project

Effects of early life stress on brain activation.

<p>Within and between group analysis comparing BOLD response to first CS presentation over baseline in rats previously exposed to early life stress (ELS) compared to unstressed controls (CON). KE  =  cluster extent in voxels. T  =  peak voxel t-statistic. Clusters reaching <i>P</i>_corrected <0.05 are shown. R =  right, L =  left, RSG =  retrospenial granular cortex. CON, n =  14; ELS, n = 8.</p

Training and scanning procedure.

<p>Animals were acclimatised to the scanning procedure using a mock scanner, conditioned and then tested during retrieval in the scanner 24 h later.</p

Brain activation maps in response to a fear conditioned stimulus.

<p>(a) Activation in PG in response to CS presentation is observed in right lateral amygdala (LA) and hypothalamus (Hyp). (b) Comparison of response between groups (PG:UG) confirms LA, Hyp and granular insular cortex (GI) activation in PG in response to the CS presentation. Extinction modeling of (c) the response across CS presentations within the PG or (d) between PG and UG groups reveals right LA, Hyp, GI and somatosensory cortex activation (SSC). PG, n = 14; UG, n = 10.</p

Heart rate levels obtained during preliminary testing using a mock scanner.

<p>Data Mean ±SEM, n = 6 rats. Previous telemetry evidence suggests a resting heart rate of around 300–380 bpm in adult rats.</p

Consequence of early life stress on fear associated brain activation.

<p>Greater activation in response to CS presentation is observed in right and left lateral amygdalae (LA), hypothalamus (Hyp) and optic grey (optic) when ELS is compared to CON. (a) Coronal, (b) axial and (c) sagittal sections of rat brain. Control, n = 14; ELS, n = 8.</p