Survival patterns in cystic fibrosis

This paper describes the Data Registry System of the Cystic Fibrosis Foundation. Through this system an annual up-dated and corrected data base has been prepared for cystic fibrosis patients seen at U.S.A. Centers from 1966 through 1972.From the data base, a large number of life table studies are calculated. A study is specified by its data base, the observation period, analysis year (age year or year since diagnosis), the Centers included, and the condition of patients at birth, and possible additional factors such as sex, age at diagnosis, or race. The annual rates of mortality are measured by a force of mortality technique.Two techniques have been used to appraise the mortality of particular groups, graphing the cumulative survival function for the group together with the cumulative survival function for a comparison group, and calculation of mortality ratios of actual to expected deaths (by an applicable standard mortality table). This latter approach lends itself readily to tests of significance.For the purpose of calculating mortality ratios, two standard tables have been prepared on the basis of the 1972 Data Base and the 1966-1972 observations. The first of these standard tables is for use with age year studies, and the second is for years since diagnosis studies.The paper comments on a number of mortality studies by calendar year, by sex, by condition at birth, by age at diagnosis, and of survival from diagnosis of patients diagnosed in 1966-1972. The calendar year studies indicate an improvement trend in the survival experience from 1966 to 1972 but a change may be occurring. The study by sex exhibits a higher female mortality. The 7% of patients who have meconium ileus at birth show a significantly higher first-year mortality, and a somewhat lower survival rate thereafter even if the first year of life experience is excluded. Mortality data for groups specified by age at diagnosis are given. For patients diagnosed during 1966-1972, there is some indication of better survival than for patients diagnosed prior to 1966.The continuation of these mortality studies on an annual basis should further our understanding of survival patterns for cystic fibrosis patients.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/21939/1/0000346.pd

Investigation of non-uniform airflow signal oscillation during high frequency chest compression

BACKGROUND: High frequency chest compression (HFCC) is a useful and popular therapy for clearing bronchial airways of excessive or thicker mucus. Our observation of respiratory airflow of a subject during use of HFCC showed the airflow oscillation by HFCC was strongly influenced by the nonlinearity of the respiratory system. We used a computational model-based approach to analyse the respiratory airflow during use of HFCC. METHODS: The computational model, which is based on previous physiological studies and represented by an electrical circuit analogue, was used for simulation of in vivo protocol that shows the nonlinearity of the respiratory system. Besides, airflow was measured during use of HFCC. We compared the simulation results to either the measured data or the previous research, to understand and explain the observations. RESULTS AND DISCUSSION: We could observe two important phenomena during respiration pertaining to the airflow signal oscillation generated by HFCC. The amplitudes of HFCC airflow signals varied depending on spontaneous airflow signals. We used the simulation results to investigate how the nonlinearity of airway resistance, lung capacitance, and inertance of air characterized the respiratory airflow. The simulation results indicated that lung capacitance or the inertance of air is also not a factor in the non-uniformity of HFCC airflow signals. Although not perfect, our circuit analogue model allows us to effectively simulate the nonlinear characteristics of the respiratory system. CONCLUSION: We found that the amplitudes of HFCC airflow signals behave as a function of spontaneous airflow signals. This is due to the nonlinearity of the respiratory system, particularly variations in airway resistance

Ocean warming affects the distribution and abundance of resident fishes by changing their reproductive scope

With ocean warming predicted globally, one of the mechanisms driving distributional shifts and changes in the abundance of resident fishes is reproductive output. The relationship between sea surface temperature and the reproductive activity of a eurythermic, resident coastal species, blacktail seabream Diplodus sargus capensis, was examined in the ‘‘ocean warming’’ hotspot of the northern Benguela. Reproductive activity was found to be restricted to periods when the water temperature dropped below 20 _C. A metadata analysis conducted on the D. sargus sub-species complex similarly showed that reproductive activity was restricted to temperatures between 15 and 20 _C, regardless of the range in ambient water temperature. Based on these findings and using satellite derived SST information, we examined D. s. capensis’s total and seasonal ‘‘reproductive scope’’ that is defined as either the area suitable for spawning each year or the duration of its potential spawning season at a fixed geographical locality, respectively. Trends were examined over the last three decades. Reproductive scope by area was found to be shrinking at a rate of 7 % per decade in southern Angola and expanding at a rate of 6 % per decade in northern Namibia. Reproductive scope by season decreased by 1.05 months per decade in Namibe, southern Angola and increased by 0.76 months per decade in Hentiesbaai, northern Namibia. Changes in reproductive scope may be a driving mechanism of distributional shifts in resident fishes, although the rate of the shifts is likely to be slow. More importantly, changes in reproductive scope will not be uniform throughout fish distributions and will most likely result in heterogeneous variations in fish abundance

Ocean warming, a rapid distributional shift, and the hybridization of a coastal fish species

Despite increasing awareness of large-scale climate-driven distribution shifts in the marine environment, no study has linked rapid ocean warming to a shift in distribution and consequent hybridization of a marine fish species. This study describes rapid warming (0.8 °C per decade) in the coastal waters of the Angola-Benguela Frontal Zone over the last three decades and a concomitant shift by a temperature sensitive coastal fish species (Argyrosomus coronus) southward from Angola into Namibia. In this context, rapid shifts in distribution across Economic Exclusive Zones will complicate the management of fishes, particularly when there is a lack of congruence in the fisheries policy between nations. Evidence for recent hybridization between A. coronus and a congener, A. inodorus, indicate that the rapid shift in distribution of A. coronus has placed adults of the two species in contact during their spawning events. Ocean warming may therefore revert established species isolation mechanisms and alter the evolutionary history of fishes. While the consequences of the hybridization on the production of the resource remain unclear, this will most likely introduce additional layers of complexity to their management

Aspects of the biology and fisheries of an economically important sparid Dentex macrophthalmus (Bloch 1791) in the Namibe province, Angola

Dentex macrophthalmus is a small, commercially important sparid fish that is abundant in the deeper waters (range 50–500 m) of the Mediterranean Sea (Trunov 1970), the North-West African shelf (Goode and Bean 1896, Fowler 1936), including the Cape Verde and Canary Islands, and the south-eastern Atlantic from the Equator to just south of Lüderitz (27°40′ S) (Trunov 1970). Because of its high abundance, it is of considerable economic importance and is captured in large numbers in both artisanal and commercial fisheries in many countries. Although it is the most important artisanal species along the entire Angolan coast, it is most abundant in the southern region (Kilongo et al. 2007). Despite its economic importance in Angola and elsewhere, relatively little is known on its abundance, fisheries and biology

An international/multicentre report on patients with cystic fibrosis (CF) over the age of 40 years

AbstractBackgroundThe lifespan of patients with cystic fibrosis (CF) is increasing significantly. The objective of this international pilot study was to study the characteristics of these long-term survivors.MethodsFour centres with large CF clinics from London (UK), Minneapolis (USA), Toronto (Canada) and Verona (Italy) identified 366 patients who had survived 40years and longer.ResultsAt all centres males survived longer than females. There were more pancreatic sufficient patients in Verona (60%) and Toronto (40%) than in London (16%) and Minneapolis (21%). The percentage of ΔF508 homozygous patients varied between 47% in London and 45% in Minneapolis to only 26% in Toronto and 9% in Verona.Average FEV1 and BMI values of the surviving population appeared to stabilise after 40years of age. FEV1 was on average 12% higher in patients who were pancreatic sufficient (p > 0.0001). There was no difference in survival between the centres. The overall median survival after the age of 40 was 13years. The estimated annual death rate was approximately 3.4% from the age of 40–60years.ConclusionsSignificant numbers of patients are now surviving to 40years or more, and it is hoped that an in-depth study of these patients may identify the factors contributing to longer survival

Cotton breeding in Australia : meeting the challenges of the 21st century

The Commonwealth Scientific and Industrial Research Organisation (CSIRO) cotton breeding program is the sole breeding effort for cotton in Australia, developing high performing cultivars for the local industry which is worth∼AU$3 billion per annum. The program is supported by Cotton Breeding Australia, a Joint Venture between CSIRO and the program’s commercial partner, Cotton Seed Distributors Ltd. (CSD). While the Australian industry is the focus, CSIRO cultivars have global impact in North America, South America, and Europe. The program is unique compared with many other public and commercial breeding programs because it focuses on diverse and integrated research with commercial outcomes. It represents the full research pipeline, supporting extensive long-term fundamental molecular research; native and genetically modified (GM) trait development; germplasm enhancement focused on yield and fiber quality improvements; integration of third-party GM traits; all culminating in the release of new commercial cultivars. This review presents evidence of past breeding successes and outlines current breeding efforts, in the areas of yield and fiber quality improvement, as well as the development of germplasm that is resistant to pests, diseases and abiotic stressors. The success of the program is based on the development of superior germplasm largely through field phenotyping, together with strong commercial partnerships with CSD and Bayer CropScience. These relationships assist in having a shared focus and ensuring commercial impact is maintained, while also providing access to markets, traits, and technology. The historical successes, current foci and future requirements of the CSIRO cotton breeding program have been used to develop a framework designed to augment our breeding system for the future. This will focus on utilizing emerging technologies from the genome to phenome, as well as a panomics approach with data management and integration to develop, test and incorporate new technologies into a breeding program. In addition to streamlining the breeding pipeline for increased genetic gain, this technology will increase the speed of trait and marker identification for use in genome editing, genomic selection and molecular assisted breeding, ultimately producing novel germplasm that will meet the coming challenges of the 21st Century

A case–control study of the impact of the East Anglian breast screening programme on breast cancer mortality

Although breast cancer screening has been shown to work in randomised trials, there is a need to evaluate service screening programmes to ensure that they are delivering the benefit indicated by the trials. We carried out a case–control study to investigate the effect of mammography service screening, in the NHS breast screening programme, on breast cancer mortality in the East Anglian region of the UK. Cases were deaths from breast cancer in women diagnosed between the ages of 50 and 70 years, following the instigation of the East Anglia Breast Screening Programme in 1989. The controls were women (two per case) who had not died of breast cancer, from the same area, matched by date of birth to the cases. Each control was known to be alive at the time of death of her matched case. All women were known to the breast screening programme and were invited, at least once, to be screened. There were 284 cases and 568 controls. The odds ratio (OR) for risk of death from breast cancer in women who attended at least one routine screen compared to those who did not attend was 0.35 (CI: 0.24, 0.50). Adjusting for self-selection bias gave an estimate of the breast cancer mortality reduction associated with invitation to screening of 35% (OR=0.65, 95% CI: 0.48, 0.88). The effect of actually being screened was a 48% breast cancer mortality reduction (OR=0.52, 95% CI: 0.32, 0.84). The results suggest that the National Breast Screening Programme in East Anglia is achieving a reduction in breast cancer deaths, which is at least consistent with the results from the randomised controlled trials of mammographic screening

Variability in chemotherapy delivery for elderly women with advanced stage ovarian cancer and its impact on survival

Given the survival benefits of adjuvant chemotherapy for advanced ovarian cancer (OC), we examined the associations of survival with the time interval from debulking surgery to initiation of chemotherapy and with the duration of chemotherapy. Among patients ⩾65 years with stages III/IV OC diagnosed between 1991 and 2002 in the Surveillance, Epidemiology, and End Results-Medicare database, we developed regression models of predictors of the time interval from surgery to initiation of chemotherapy and of the total duration of chemotherapy. Survival was examined with Cox proportional hazards models. Among 2558 patients, 1712 (67%) initiated chemotherapy within 6 weeks of debulking surgery, while 846 (33%) began treatment >6 weeks. Older age, black race, being unmarried, and increased comorbidities were associated with delayed initiation of chemotherapy. Delay of chemotherapy was associated with an increase in mortality (hazard ratio (HR)=1.11; 95% CI, 1.0–1.2). Among 1932 patients in the duration of treatment analysis, the 1218 (63%) treated for 3–7 months had better survival than the 714 (37%) treated for ⩽3 months (HR=0.84; 95% CI, 0.75–0.94). This analysis represents one of the few studies describing treatment delivery and outcome in women with advanced OC. Delayed initiation and early discontinuation of chemotherapy were common and associated with increased mortality

Publisher Correction:Discovery of rare variants associated with blood pressure regulation through meta-analysis of 1.3 million individuals (Nature Genetics, (2020), 52, 12, (1314-1332), 10.1038/s41588-020-00713-x)

In the version of this article originally published, the e-mail address of corresponding author Patricia B. Munroe was incorrect. The error has been corrected in the HTML and PDF versions of the article