European Cystic Fibrosis Society Standards of Care: Quality Management in cystic fibrosis

Since the earliest days of cystic fibrosis (CF) treatment, patient data have been recorded and reviewed in order to identify the factors that lead to more favourable outcomes. Large data repositories, such as the US Cystic Fibrosis Registry, which was established in the 1960s, enabled successful treatments and patient outcomes to be recognized and improvement programmes to be implemented in specialist CF centres. Over the past decades, the greater volumes of data becoming available through Centre databases and patient registries led to the possibility of making comparisons between different therapies, approaches to care and indeed data recording. The quality of care for individuals with CF has become a focus at several levels: patient, centre, regional, national and international. This paper reviews the quality management and improvement issues at each of these levels with particular reference to indicators of health, the role of CF Centres, regional networks, national health policy, and international data registration and comparisons. 

Influence of tropical easterlies in southern Africa's winter rainfall zone during the Holocene

South Africa's southwestern Cape occupies a critical transition zone between Southern Hemisphere temperate (winter) and tropical (summer) moisture-bearing systems. In the recent geological past, it has been proposed that the relative influence of these systems may have changed substantially, but little reliable evidence regarding regional hydroclimates and rainfall seasonality exists to refine or substantiate the understanding of long-term dynamics. In this paper we present a mid-to late Holocene multi-proxy record of environmental change from a rock hyrax midden from Katbakkies Pass, located along the modern boundary between the winter and summer rainfall zones. Derived from stable carbon and nitrogen isotopes, fossil pollen and microcharcoal, these data provide a high resolution record of changes in humidity, and insight into changes in rainfall seasonality. Whereas previous work concluded that the site had generally experienced only subtle environmental change during the Holocene, our records indicate that significant, abrupt changes have occurred in the region over the last 7000 years. Contrary to expectations based on the site's location, these data indicate that the primary determinant of changes in humidity is summer rather than winter rainfall variability, and its influence on drought season intensity and/or length. These findings are consistent with independent records of upwelling along the southern and western coasts, which indicate that periods of increased humidity are related to increased tropical easterly flow. This substantially refines our understanding of the nature of temperate and tropical circulation system dynamics in SW Africa, and how changes in their relative dominance have impacted regional environments during the Holocene. 

Holocene “turn-on” and evolution of the Southern Great Barrier Reef: Revisiting reef cores from the Capricorn Bunker Group

Extensive drilling of the Great Barrier Reef (GBR) in the 70s and 80s illuminated the main factors controlling reef growth during the Holocene. However, questions remain about: (1) the precise nature and timing of reef "turnon" or initiation, (2) whether consistent spatio-temporal patterns occur in the bio-sedimentologic response of the reef to Holocene sea-level rise then stability, and (3) how these factors are expressed in the context of the different evolutionary states (juvenile-mature-senile reefs). Combining 21 new C14-AMS and 146 existing recalibrated radiocarbon and U/Th ages, we investigated the detailed spatial and temporal variations in sedimentary facies and coralgal assemblages in fifteen cores across four reefs (Wreck, Fairfax, One Tree and Fitzroy) from the Southern GBR. Our newly defined facies and assemblages record distinct chronostratigraphic patterns in the cores, displaying both lateral zonation across the different reefs and shallowing upwards sequences, characterised by a transition from deep (Porites/faviids) to shallow (Acropora/Isopora) coral types. The revised reef accretion curves show a significant lag period, ranging from 0.7-2 ka, between flooding of the antecedent Pleistocene substrate and Holocene reef turn-on. This lag period and dominance of more environmentally tolerant early colonizers (e.g., domal Porites and faviids), suggests initial conditions that were unfavourable for coral growth. We contend that higher input of fine siliciclastic material from regional terrigenous sources, exposure to hydrodynamic forces and colonisation in deeper waters are the main factors influencing initially reduced growth and development. All four reefs record a time lag and we argue that the size and shape of the antecedent platform is most important in determining the duration between flooding and recolonisation of the Holocene reef. Finally, our study of Capricorn Bunker Group Holocene reefs suggests that the size and shape of the antecedent substrate has a greater impact on reef evolution and final evolutionary state (mature vs. senile), than substrate depth alone. 

Nong Thale Pron - a key site from southern Thailand for studying monsoon variability during the past 15000 years

Studies of marine sediments, cave speleothemes, annually laminated corals, and tree rings from Asian monsoon regions have added knowledge to our understanding of the factors that control inter-annual to millennial monsoon variability in the past and have provided important constraints for climate modeling scenarios. In contrast, the spatial and temporal pattern of sub-millennial scale monsoon variability and its impact on land cover in SE Asia are still unresolved. This shortcoming stems from the fact that temporally well-resolved paleo-environmental studies are missing from large parts of SE Asia, especially from Thailand. Given that global and regional climate models are increasingly using terrestrial paleo- data to test their performance, past changes in land cover are therefore important variables to better understand feedbacks between different Earth systems. We obtained sediments from Lake Nong Thale Pron, in southern Thailand (8º 10`N, 99 º23`E; 380 m.asl). The aim of our study is to reconstruct lake status changes and to evaluate whether the extent of these changes are linked to known shifts in monsoon intensity and variability. Preliminary results show that lake infilling started more than 15,000 years ago and that the sediments cover the last deglaciation and the Holocene. Current analyses include Itrax XRF core scanning, loss-on-ignition (LOI at 950 and 550ºC), CN elemental and isotopic composition. We expect that our results will be able to give a picture of how the lake's status has changed over time and whether the extent of these changes is linked to known shifts in monsoon intensity and variability.

The Type IIP Supernova 2012aw in M95: Hydrodynamical Modeling of the Photospheric Phase from Accurate Spectrophotometric Monitoring

We present an extensive optical and near-infrared photometric and spectroscopic campaign of the Type IIP supernova SN 2012aw. The data set densely covers the evolution of SN 2012aw shortly after the explosion through the end of the photospheric phase, with two additional photometric observations collected during the nebular phase, to fit the radioactive tail and estimate the ⁵⁶Ni mass. Also included in our analysis is the previously published Swift UV data, therefore providing a complete view of the ultraviolet-optical- infrared evolution of the photospheric phase. On the basis of our data set, we estimate all the relevant physical parameters of SN 2012aw with our radiation-hydrodynamics code: envelope mass M _env ∼ 20 M _⊙, progenitor radius R ∼ 3 × 10¹³ cm (∼430 R_⊙), explosion energy E ∼ 1.5 foe, and initial ⁵⁶Ni mass ∼0.06 M_⊙. These mass and radius values are reasonably well supported by independent evolutionary models of the progenitor, and may suggest a progenitor mass higher than the observational limit of 16.5 ± 1.5 M _⊙of the Type IIP events. 

The frequency of Environmental Quality Standard (EQS) exceedance for chlorinated organic pollutants in rivers of the Humber catchments

The southern industrial rivers (Aire, Calder, Don and Trent) feeding the Humber estuary were routinely monitored for a range of chlorinated micro- organic contaminants at least once a week over a 1.5-year period. Environmental Quality Standards (EQSs) for inland waters were set under the European Economic Community for a limited number of problematic contaminants (18). The results of the monitoring program for seven classes of chlorinated pollutants on the EQS list are presented in this study. All compounds were detected frequently with the exception of hexachlorobutadiene (where only one detectable measurement out of 280 individual samples occurred). In general, the rivers fell into two classes with respect to their contamination patterns. The Aire and Calder carried higher concentrations of micro- pollutants than the Don and Trent, with the exception of hexachlorobenzene (HCB). For Σ hexachlorocyclohexane (HCH) isomers (α + γ) and for dieldrin, a number of samples (~ 5%) exceeded their EQS for both the Aire and Calder. Often, ΣHCH concentrations were just below the EQS level. Levels of p,p'- DDT on occasions approached the EQS for these two rivers, but only one sample (out of 140) exceeded the EQS. No compounds exceeded their EQS levels on the Don and Trent. Analysis of the ratio of γ HCH/αHCH indicated that the source of HCH for the Don and Trent catchments was primarily lindane (γHCH) and, to a lesser extent, technical HCH (mixture of HCH isomers, dominated by α HCH), while the source(s) for the Aire and Calder had a much higher contribution from technical HCH.

Characterization of a reference material for BCR-ABL (M-BCR) mRNA quantitation by real-time amplification assays:towards new standards for gene expression measurements

Monitoring of BCR-ABL transcripts has become established practice in the management of chronic myeloid leukemia. However, nucleic acid amplification techniques are prone to variations which limit the reliability of real-time quantitative PCR (RQ-PCR) for clinical decision making, highlighting the need for standardization of assays and reporting of minimal residual disease (MRD) data. We evaluated a lyophilized preparation of a leukemic cell line (K562) as a potential quality control reagent. This was found to be relatively stable, yielding comparable respective levels of ABL, GUS and BCR-ABL transcripts as determined by RQ-PCR before and after accelerated degradation experiments as well as following 5 years storage at -20 degrees C. Vials of freeze-dried cells were sent at ambient temperature to 22 laboratories on four continents, with RQ-PCR analyses detecting BCR-ABL transcripts at levels comparable to those observed in primary patient samples. Our results suggest that freeze-dried cells can be used as quality control reagents with a range of analytical instrumentations and could enable the development of urgently needed international standards simulating clinically relevant levels of MRD.

An evaluation of palliative care services for cancer patients in the Southern Health and Social Services Board of Northern Ireland.

An interview study of 55 lay carers of people who died from cancer in the Southern Board of Northern Ireland was undertaken using a combination of closed-format and open-ended questions. The aim of the study was to evaluate palliative care services delivered in the last six months of their lives to cancer patients who died either at home or in hospital. Two-thirds of the deaths (36) occurred in the domestic home, 45 of the deceased were admitted as hospital inpatients, and the great majority were in receipt of community nursing (53) and general practitioner (54) services. Open-ended questions were used to allow respondents to give their views about services in some detail and their views about good and bad aspects of services were sought. While they were generally satisfied with services specific areas of difficulty were identified in each aspect of care addressed by the study. The most favourable assessments were made of community nursing with the greatest number of negative comments being made about inpatient hospital care. Differing interests between some of those who were dying and their lay carers were found in two areas: the receipt of help from nonfamily members and the information that the deceased received about their terminal status.

Max Weber and the First World War: Protestant and Catholic living standards in Germany, 1914-1919

We assess informal institutions of Protestants and Catholics by investigating their economic resilience in a natural experiment. The First World War constitutes an exogenous shock to living standards since the duration and intensity of the war exceeded all expectations. We assess the ability of Protestant and Catholic communities to cope with increasing food prices and wartime black markets. Literature based on Weber (1904, 1905) suggests that Protestants must be more resilient than their Catholic peers. Using individual height data on some 2,800 Germans to assess levels of malnutrition during the war, we find that living standards for both Protestants and Catholics declined; however, the decrease of Catholics’ height was disproportionately large. Our empirical analysis finds a large statistically significant difference between Protestants and Catholics for the 1915–19 birth cohort, and we argue that this height gap cannot be attributed to socioeconomic background and fertility alone.

Max Weber and the First World War: Protestant and Catholic living standards in Germany, 1915-1919

We assess informal institutions of Protestants and Catholics by investigating their economic
resilience in a natural experiment. The First World War constitutes an exogenous shock to living standards since the duration and intensity of the war exceeded all expectations. We assess the ability of Protestant and Catholic communities to cope with increasing food prices and wartime black markets. Literature based on Weber (1904, 1905) suggests that Protestants must be more resilient than their Catholic peers. Using individual height data on some 2,800 Germans to assess levels of malnutrition during the war, we find that living standards for both Protestants and Catholics declined; however, the decrease of Catholics’ height was disproportionately large. Our empirical analysis finds a large statistically significant difference between Protestants and Catholics for the 1914-19 birth cohort, and we argue that this height gap cannot be attributed to socioeconomic background and fertility alone.

A stacked Late Quaternary fluvio-periglacial sequence from the Axe valley, southern England with implications for landscape evolution and Palaeolithic archaeology

The current model of mid-latitude late Quaternary terrace sequences, is that they are uplift-driven but climatically controlled terrace staircases, relating to both regional-scale crustal and tectonic factors, and palaeohydrological variations forced by quasi-cyclic climatic conditions in the 100 K world (post Mid Pleistocene Transition). This model appears to hold for the majority of the river valleys draining into the English Channel which exhibit 8–15 terrace levels over approximately 60–100 m of altitudinal elevation. However, one valley, the Axe, has only one major morphological terrace and has long-been regarded as anomalous. This paper uses both conventional and novel stratigraphical methods (digital granulometry and terrestrial laser scanning) to show that this terrace is a stacked sedimentary sequence of 20–30 m thickness with a quasi-continuous (i.e. with hiatuses) pulsed, record of fluvial and periglacial sedimentation over at least the last 300–400 K yrs as determined principally by OSL dating of the upper two thirds of the sequence. Since uplift has been regional, there is no evidence of anomalous neotectonics, and climatic history must be comparable to the adjacent catchments (both of which have staircase sequences) a catchment-specific mechanism is required. The Axe is the only valley in North West Europe incised entirely into the near-horizontally bedded chert (crypto-crystalline quartz) and sand-rich Lower Cretaceous rocks creating a buried valley. Mapping of the valley slopes has identified many large landslide scars associated with past and present springs. It is proposed that these are thaw-slump scars and represent large hill-slope failures caused by Vauclausian water pressures and hydraulic fracturing of the chert during rapid permafrost melting. A simple 1D model of this thermokarstic process is used to explore this mechanism, and it is proposed that the resultant anomalously high input of chert and sand into the valley during terminations caused pulsed aggradation until the last termination. It is also proposed that interglacial and interstadial incision may have been prevented by the over-sized and interlocking nature of the sub-angular chert clasts until the Lateglacial when confinement of the river overcame this immobility threshold. One result of this hydrogeologically mediated valley evolution was to provide a sequence of proximal Palaeolithic archaeology over two MIS cycles. This study demonstrates that uplift tectonics and climate alone do not fully determine Quaternary valley evolution and that lithological and hydrogeological conditions are a fundamental cause of variation in terrestrial Quaternary records and landform evolution.

Review of Protestant Churches and the Formation of Political Consciousness in Southern Mozambique (1930–1974).

Book review of Protestant Churches and the Formation of Political Consciousness in Southern Mozambique (1930–1974). By TERESA CRUZ E SILVA. Introduction by DAVID HEDGES. Basel: P. Schlettwein Publishing, 2001. Pp. xvii+210. CHF 48; €32 (ISBN 3-908193-09-5).

Causes and five-year incidence of blindness and visual impairment in urban southern China:The Liwan Eye Study

PURPOSE. We determined the causes and five-year incidence of blindness and visual impairment (VI) in an adult, urban Chinese population. METHODS. Participants underwent a comprehensive eye examination at baseline in 2003 and then five years later. The World Health Organization (WHO) and United States (US) definitions were used to define incident blindness (WHO visual acuity [VA] < 20/400 in the better-seeing eye, US VA ≤ 20/200) and incident VI (WHO VA < 20/60-20/400, US VA < 20/40->20/200). RESULTS. Among 1405 baseline participants, 924 (75%) of 1232 survivors (87.7%) participated in the 5-year follow-up. The incidences of VI and blindness were 5.38% (95% confidence interval [CI] 3.99% ~ 7.07%) and 0.33% (95% CI 0.07% ~ 0.95%), respectively, based on the WHO definition, and 9.85% (95% CI 7.96% ~ 12.0%) and 1.42% (95% CI 0.76% ~ 2.41%), respectively, based on the US definition. Incidence of blindness and VI (WHO definition) increased significantly with older age (P < 0.001) and poorer baseline presenting VA in the worse-seeing eye (P < 0.001). The leading cause of best-corrected VI (WHO definition) was cataract (64.6%), whereas the main causes of presenting VI were refractive error (40.4%) and cataract (38.4%). CONCLUSIONS. The incidence of VI in urban Southern China is high. The major causes are unoperated cataract and undercorrected refractive error, reflecting the need for better surgical and refractive care, even in this urban setting. © 2013 The Association for Research in Vision and Ophthalmology, Inc.