Impact of Cystic Fibrosis on Unaffected Siblings: A Systematic Review

Objective To conduct a systematic review of the evidence to determine the impact of cystic fibrosis (CF) on unaffected siblings. Study design We searched MEDLINE (Ovid interface, from 1946); EMBASE (Ovid interface, from 1946); CINAHL (EBSCO interface); Academic Search Complete (EBSCO interface); Psych Info (EBSCO interface); ProQuest Theses' and Dissertation's (ProQuest); British Index of Nursing (ProQuest); Web of Science (ISI, Web of Knowledge portal); PubMed (PubMed NCBI); BASE (Bielefeld Academic Research Engine); Scopus; EThOS (e-theses online service); Open Grey; and Cochrane Library. Contents pages of the Journal of Cystic Fibrosis, June 2002-April 2017 were hand searched to identify further eligible studies. Reference lists of eligible articles and relevant review papers were screened. Inclusion criteria were full studies published after 1989 in English focusing on the impact of cystic fibrosis on unaffected siblings. Results In total, 13 papers, 4 PhD theses and 1 MSc thesis were included in the review. Four themes were identified; family functioning, psychosocial impact, knowledge of CF, and condition-specific differences. Conclusions Most studies are old and may not accurately represent the impact of CF on unaffected siblings following changes to health care provision including newborn bloodspot screening and the advent of CF transmembrane regulator modulator therapies. Further work is needed directly with siblings rather than using mothers as proxies to determine effect of age, sex, and disease trajectory on unaffected siblings' experiences

Source and quantity of carbon influence its sequestration in Rostherne Mere (UK) sediment: a novel application of stepped combustion radiocarbon analysis

We explored the roles of phytoplankton production, carbon source, and human activity on carbon accumulation in a eutrophic lake (Rostherne Mere, UK) to understand how changes in nutrient loading, algal community structure and catchment management can influence carbon sequestration in lake sediments. Water samples (dissolved inorganic, organic and particulate carbon) were analysed to investigate contemporary carbon sources. Multiple variables in a 55-cm sediment core, which represents the last ~ 90 years of accumulation, were studied to determine historical production rates of algal communities and carbon sources. Fluctuations in net primary production, inferred from sedimentary diatom abundance and high-performance liquid chromatography (HPLC) pigment methods, were linked to nutrient input from sewage treatment works (STW) in the catchment. Stepped combustion radiocarbon (SCR) measurements established that lake sediment contains between 11% (~ 1929 CE) and 69% (~ 1978 CE) recalcitrant carbon, with changes in carbon character coinciding with peaks in accumulation rate and linked to STW inputs. Catchment disturbance was identified by radiocarbon analysis, and included STW construction in the 1930s, determined using SCR analysis, and recent nearby highway construction, determined by measurements on dissolved organic carbon from the lake and outflow river. The quantity of autochthonous carbon buried was related to diatom biovolume accumulation rate (DBAR) and decreased when diatom accumulation rate and valve size declined, despite an overall increase in net carbon production. HPLC pigment analysis indicated that changes in total C deposition and diatom accumulation were related to proliferation of non-siliceous algae. HPLC results also indicated that dominance of recalcitrant carbon in sediment organic carbon was likely caused by increased deposition rather than preservation factors. The total algal accumulation rate controlled the sediment organic carbon accumulation rate, whereas DBAR was correlated to the proportion of each carbon source buried

Source and quantity of carbon influence its sequestration in Rostherne Mere (UK) sediment: a novel application of stepped combustion radiocarbon analysis

We explored the roles of phytoplankton production, carbon source, and human activity on carbon accumulation in a eutrophic lake (Rostherne Mere, UK) to understand how changes in nutrient loading, algal community structure and catchment management can influence carbon sequestration in lake sediments. Water samples (dissolved inorganic, organic and particulate carbon) were analysed to investigate contemporary carbon sources. Multiple variables in a 55-cm sediment core, which represents the last ~ 90 years of accumulation, were studied to determine historical production rates of algal communities and carbon sources. Fluctuations in net primary production, inferred from sedimentary diatom abundance and high-performance liquid chromatography (HPLC) pigment methods, were linked to nutrient input from sewage treatment works (STW) in the catchment. Stepped combustion radiocarbon (SCR) measurements established that lake sediment contains between 11% (~ 1929 CE) and 69% (~ 1978 CE) recalcitrant carbon, with changes in carbon character coinciding with peaks in accumulation rate and linked to STW inputs. Catchment disturbance was identified by radiocarbon analysis, and included STW construction in the 1930s, determined using SCR analysis, and recent nearby highway construction, determined by measurements on dissolved organic carbon from the lake and outflow river. The quantity of autochthonous carbon buried was related to diatom biovolume accumulation rate (DBAR) and decreased when diatom accumulation rate and valve size declined, despite an overall increase in net carbon production. HPLC pigment analysis indicated that changes in total C deposition and diatom accumulation were related to proliferation of non-siliceous algae. HPLC results also indicated that dominance of recalcitrant carbon in sediment organic carbon was likely caused by increased deposition rather than preservation factors. The total algal accumulation rate controlled the sediment organic carbon accumulation rate, whereas DBAR was correlated to the proportion of each carbon source buried

Understanding value creation and word-of-mouth behaviour at cultural events

Cultural value is a highly contested concept, despite its undoubted importance to practitioners and policy makers. Reseach into cultural value has, meanwhile, tended to employ a unidimensional value framework. This has hamprered the understanding of behaviour related to the word-of-mouth (WOM) communication behaviour of cultural values. This paper presents a cultural value segmentation based on a multidimensional value framework, allowing a profile of WOM behaviour (both online and offline) of each segment to be developed. The segmentation has four distinct segments of cultural consumer, each with different combinations of cultural values and WOM communication preferences. In this way, the study challenges current understandings of value creation and transfer in cultural settings. By way of practical recommendations, the study favours the use of market segmentation based on multi-dimensional value ‘constellations’, which can not only achieve better audience development but also to encourage wider WOM communication of the values in question

Understanding the transfer of contemporary temperature signals into lake sediments via paired oxygen isotope ratios in carbonates and diatom silica: problems and potential

Although the oxygen isotope composition (δ18O) of calcite (δ18Ocalcite) and, to a lesser extent, diatom silica (δ18Odiatom) are widely used tracers of past hydroclimates (especially temperature and surface water hydrology), the degree to which these two hosts simultaneously acquire their isotope signals in modern lacustrine environments, or how these are altered during initial sedimentation, is poorly understood. Here, we present a unique dataset from a natural limnological laboratory to explore these issues. This study compares oxygen and hydrogen isotope data (δ18O, δ2H) of contemporary lake water samples at ~2-weekly intervals over a 2-year period (2010–12) with matching collections of diatoms (δ18Odiatom) and calcite (δ18Ocalcite) from sediment traps (at 10 m and 25 m) at Rostherne Mere (maximum depth 30 m), a well-monitored, eutrophic, seasonally stratified monomictic lake in the UK. The epilimnion shows a seasonal pattern of rising temperature and summer evaporative enrichment in 18O, and while there is a temperature imprint in both δ18Odiatom and δ18Ocalcite, there is significant inter-annual variability in both of these signals. The interpretation of δ18Odiatom and δ18Ocalcite values is complicated due to in-lake processes (e.g. non-equilibrium calcite precipitation, especially in spring, leading to significant 18Ocalcite depletion), and for δ18Odiatom, by post-mortem, depositional and possibly dissolution or diagenetic effects. For 2010 and 2011 respectively, there is a strong temperature dependence of δ18Ocalcite and δ18Odiatom in fresh trap material, with the fractionation slope for δ18Odiatom of ca. −0.2‰/°C, in agreement with several other studies. The δ18Odiatom data indicate the initiation of rapid post-mortem secondary alteration of fresh diatom silica (within ~6 months), with some trap material undergoing partial maturation in situ. Diatom δ18O of the trap material is also influenced by resuspension of diatom frustules from surface sediments (notably in summer 2011), with the net effect seen as an enrichment of deep-trap 18Odiatom by about +0.7‰ relative to shallow-trap values. Contact with anoxic water and anaerobic bacteria are potentially key to initiating this silica maturation process, as deep-trap samples that were removed prior to anoxia developing do not show enrichment. Dissolution (perhaps enhanced by anaerobic bacterial communities) may also be responsible for changes to δ18Odiatom that lead to increasing, but potentially predictable, error in inferred temperatures using this proxy. High resolution, multi-year monitoring can shed light on the complex dynamics affecting δ18Odiatom and δ18Ocalcite and supports the careful use of sedimentary δ18Odiatom and δ18Ocalcite as containing valuable hydroclimatic signals especially at a multi-annual resolution, although there remain substantial challenges to developing a reliable geothermometer on paired δ18Odiatom and δ18Ocalcite. In particular, δ18Odiatom needs cautious interpretation where silica post-mortem secondary alteration is incomplete and diatom preservation is not perfect, and we recommend dissolution be routinely assessed on diatom samples used for isotopic analyses

The impacts of changing nutrient load and climate on a deep, eutrophic, monomictic lake

1. Nutrient availability and climate have substantial effects on the structure and function of lakes. Predicted changes to climate (particularly temperature) over the 21st century are expected to adjust physical lake functions, changing thermal and nutrient use processes. Both increasing anthropogenic nutrient inputs and net reductions following remediation will also drive ecological change. Therefore, there is an increasing necessity to disentangle the effects of nutrient and temperature change on lakes to understand how they might act in additive and antagonistic ways. 2. This study quantified internal and external nutrient loads at Rostherne Mere, U.K., a deep (zmax = 30 m), monomictic eutrophic lake (average annual total phosphorus >100 μg/L) that has a long, stable period of stratification (c. 8.5 months). A lake biophysical model (PROTECH) was used to assess the effect of changes in these loads and climate change on lake productivity in a factorial modelling experiment. 3. During the summer, phosphorus released from the sediment is largely restricted to the hypolimnion and phytoplankton production is supported by the external load. On overturn, phosphorus at depth is distributed throughout the water column with the elevated concentration persisting to support algal productivity in the following spring. 4. Consequently, the model showed that internal nutrient loading was the main driver of current and future changes in the concentration of phosphorus (responsible for up to 86% P reduction), phytoplankton chlorophyll a and cyanobacterial blooms. However, although the external phosphorus load had a relatively small influence on annual mean phosphorus concentration, it had a statistically significant effect on chlorophyll a concentration, because it supported algal production during summer stratification. 5. Climate had minimal direct impact, but a substantial indirect impact by altering the timing, depth and length of lake stratification (c. 14 days longer by 2100), and therefore altered nutrient cycling and phosphorus availability. 6. In summary, the recovery trajectory at Rostherne Mere is limited by the annual internal soluble reactive phosphorus load replenishment that realistically is unlikely to change greatly on a shorter time-scale. Therefore, the external soluble reactive phosphorus load has the potential to play an important role as it can be managed further, but is complicated by the indirect impact of climate changing stratification and flushing patterns

In defence of the familiar : understanding conservatism in concert selection amongst classical music audiences

Since the establishment of a classical music canon in the 19th century, classical music culture has historically been focused on a stable set of masterpieces by genius composers predominantly from the classical and romantic periods. A small number of composers continue to dominate programming to this day. Many classical music organisations are keen to programme music beyond this narrow repertoire and to showcase new or unfamiliar works. The need to sell tickets, however, is often an obstacle, with organisations far more confident in the ability of big hits to attract large crowds. This article explores the experiences and opinions of classical music concertgoers in relation to familiar and unfamiliar music, providing a number of reasons as to why audiences may choose to hear well-known pieces rather than new works. This paper reports on one strand of a qualitative study with 42 individuals who booked tickets for one of two concert series consisting of core and populist repertoire, respectively. Semi-structured interviews were carried out to explore the reasons for their choices and their experiences of attending live concerts. These interviews showed that most participants did indeed have a clear preference for hearing music that was familiar to them, and only frequent attenders relished the challenge of unknown music. Participants felt that listening to familiar music was usually a more enjoyable experience than hearing something new. They rarely spoke of becoming bored with over-familiar music, perhaps because the live concert experience brings a sense of freshness to even the most familiar work

On the edge of their seats: Comparing first impressions and regular attendance in arts audiences

This article reports the findings of a questionnaire and interview study with arts audiences in a UK city, and compares the experiences of arts attenders with those of an “audience exchange” group, in which participants were taken to unfamiliar arts events and then discussed their expectations, first impressions, and intentions for future arts engagement. The study considers how the established values and behaviours of regular audience members might be inhibiting or alienating to first-time listeners, and identifies continuums of engagement that draw on psychological frameworks of identity and belonging. Differences between the factors in audience satisfaction in cinema, theatre, and music are explored, and the effects of familiarity with one art form on understanding a first encounter with a new one highlight the range of perspectives that audience members bring to any given event. The implications of these findings for audience development strategies are considered, showing how research with audiences can be a developmental tool in itself, by encouraging reflective discussion of arts experiences amongst new attenders