Multiple aneurysms in childhood

AbstractArterial aneurysms in children are rare. When present, they are often associated with connective tissue disorders or arteritidies. Idiopathic aneurysms occurring at multiple sites throughout the arterial tree are rare, with only ten cases reported. This report describes a case of multiple arterial aneurysms of uncertain origin involving upper-extremity, extracranial cerebrovascular, aortoiliac, and renal arteries in a 14-year-old boy. The clinical presentation, vascular reconstruction, pathologic findings, and a brief review of the literature are described

The incidence, prevalence and mortality of granulomatosis with polyangiitis in the UK Clinical Practice Research Datalink

Objectives: To estimate the incidence, prevalence and mortality of Granulomatosis with polyangiitis (GPA) in the United Kingdom. Methods: We conducted a historical cohort study using data from the Clinical Practice Research Datalink and Hospital Episode Statistics (CPRD-HES). We calculated incidence rate ratios, adjusted for age, gender and ethnicity, using Poisson regression. Results: We identified 462 cases diagnosed between 1997 and 2013. Our overall estimate of incidence was 11.8 (95% CI 10.7-12.9)/million person-years. Incidence in children (aged <16 years) was 0.88 (95% CI 0.40-1.96), and adults 14.0 (95% CI 12.8-15.4). The incidence was lower in females (adjusted IRR 0.68; 95% CI 0.56-0.81) and highest in the 55-69 year age-group (adjusted IRR 9.5, 95% CI 6.9-13.0; reference group 0-39 years). Incidence was not significantly different in the Black / Minority Ethnic population compared to the white population (adjusted odds ratio 0.78, 95% CI 0.53-1.13, p=0.13). The prevalence in 2013 was 134.9 (121.3-149.6) /million. Mortality was 13.6% at 1-year, and higher in HES than CPRD-identified cases (Hazard ratio 3.16, 95% CI 2.19-4.56, p<0.001). Conclusions: By combining primary and secondary care datasets we have found the incidence and mortality of granulomatosis with polyangiitis to be higher than previously reported. We predict that at present each year in the UK there will be approximately 700 new cases of whom 95 will die within 12 months

Incidence of ANCA-associated vasculitis in a UK mixed ethnicity population

Objectives: We aimed to estimate the incidence of ANCA-associated vasculitis in the UK and how this varied by ethnic group. Methods: We identified incident cases of ANCA-associated vasculitis between March 2007 and June 2013 in the Nottingham–Derby urban area from medical records using multiple sources. We derived the denominator population from the 2011 census, and we calculated incidence rate ratios using Poisson regression. Results: Overall, we identified 107 cases of ANCA-associated vasculitis, giving an incidence of 23.1 per million person-years (95% CI: 18.9, 27.9). The incidence among the white population was 25.8 per million person-years (95% CI: 21.0, 31.3) and among the black and minority ethnic (BME) population 8.4 per million person-years (95% CI: 3.1, 18.3). After adjustment for age and sex, the difference between ethnic groups was not statistically significant (incidence rate ratio 0.7, 95% CI: 0.3, 1.5, P = 0.3). Conclusion: Overall, the incidence of ANCA-associated vasculitis was similar to other epidemiological studies. Crude incidence rates were lower in the BME than in the white population, but this was partly explained by the older age profile among the white compared with BME population

Unusual phase boundary of the magnetic-field-tuned valence transition in CeOs4Sb12

The phase diagram of the filled skutterudite has been mapped in fields H of up to and temperatures T down to using resistivity, magnetostriction, and MHz conductivity. The valence transition separating the semimetallic low-H, low-T L phase from the metallic high-H, high-T H phase exhibits a very unusual, wedge-shaped phase boundary, with a non-monotonic gradient alternating between positive and negative. The expected "elliptical" behavior of the phase boundary of a valence transition with H2∝T2 originates in the H and T dependence of the free energy of the f~multiplet. Here, quantum oscillation measurements suggest that additional energy scales associated with a quantum critical point are responsible for the deviation of the phase boundary of from this text-book behavior at high H and low T. The distortion of the low-H, high-T portion of the phase boundary may be associated with the proximity of to a topological semimetal phase induced by uniaxial stress

Elephant crop-raiding and human-elephant conflict in Cambodia: crop selection and seasonal timings of raids

Elephants are threatened globally by habitat loss, poaching and accelerating levels of human-elephant conflict. For Elephas maximus in Cambodia, crop raiding underlies this conflict. Understanding the timing of raids and selection of crops can help design locally appropriate mitigation and management strategies. This study, using a 4-year database of events, investigated the most frequently raided crops and patterns of raids, over time and seasons and by location. Damage frequency varied significantly by crop, with rice, banana, cassava, sugar cane and papaya most frequently raided. Considering raid events per unit crop area, banana, sugar cane and pineapple were raided more than would be expected based on their availability. There were differences in both crop-raiding events and crop-damage frequencies over study years and there was a peak raiding season in October-December. Nationally, significant differences were found among provinces but not between years. Rates of damage decreased after mitigation strategies such as observation towers, deterrents and fences were implemented. We suggest further mechanisms to improve human-elephant conflict monitoring in relation to crop choice and availability

The incidence, prevalence and survival of systemic sclerosis in the UK Clinical Practice Research Datalink

Objective: To estimate the incidence, prevalence and survival of systemic sclerosis in the United Kingdom. Methods: We conducted a historical cohort study using data from the Clinical Practice Research Datalink (CPRD). We calculated the incidence and survival of systemic sclerosis between 1994 and 2013 and examined its association with age, sex, and socio-economic status. We calculated point prevalence on 1 July 2013, and examined its association with the same exposures. Results: We identified 1,327 cases with incident systemic sclerosis. Annual incidence was 19.4 per million person-years between 1994 and 2013. The incidence was 4.7 times higher in women than in men, was not influenced by socioeconomic status, and has remained stable over the 20 year study period. The peak age of onset was 55-69 years. Survival at 1, 5 and 10 years was 94.2%, 80.0% and 65.7% respectively. The prevalence was 307 (290-323) per million with the highest prevalence in the 70-84 years age group. We estimate there are currently 1180 new cases of systemic sclerosis each year in the UK, and 19,390 people living with systemic sclerosis. Due to the predicted growth and aging of the population, we predict a 24% increase in incident cases and 26% increase in prevalent cases in 20 years’ time. Conclusion: Our estimates of incidence and prevalence are higher than previously reported in the UK, but similar to recent USA and Swedish studies, and do not support a north-south gradient of the occurrence of systemic sclerosis in Europe

Carbon dioxide and ocean acidification observations in UK waters. Synthesis report with a focus on 2010–2015

Key messages: 1.1 The process of ocean acidification is now relatively well-documented at the global scale as a long-term trend in the open ocean. However, short-term and spatial variability can be high. 1.2 New datasets made available since Charting Progress 2 make it possible to greatly improve the characterisation of CO2 and ocean acidification in UK waters. 3.1 Recent UK cruise data contribute to large gaps in national and global datasets. 3.2 The new UK measurements confirm that pH is highly variable, therefore it is important to measure consistently to determine any long term trends. 3.3 Over the past 30 years, North Sea pH has decreased at 0.0035±0.0014 pH units per year. 3.4 Upper ocean pH values are highest in spring, lowest in autumn. These changes reflect the seasonal cycles in photosynthesis, respiration (decomposition) and water mixing. 3.5 Carbonate saturation states are minimal in the winter, and lower in 7 more northerly, colder waters. This temperature-dependence could have implications for future warming of the seas. 3.6 Over the annual cycle, North-west European seas are net sinks of CO2. However, during late summer to autumn months, some coastal waters may be significant sources. 3.7 In seasonally-stratified waters, sea-floor organisms naturally experience lower pH and saturation states; they may therefore be more vulnerable to threshold changes. 3.8 Large pH changes (0.5 - 1.0 units) can occur in the top 1 cm of sediment; however, such effects are not well-documented. 3.9 A coupled forecast model estimates the decrease in pH trend within the North Sea to be -0.0036±0.00034 pH units per year, under a high greenhouse gas emissions scenario (RCP 8.5). 3.10 Seasonal estimates from the forecast model demonstrate areas of the North Sea that are particularly vulnerable to aragonite undersaturation

A Systematic Review and Meta-Analysis of the Incidence Rate of Takayasu Arteritis

Objectives: Takayasu arteritis (TAK), is a rare autoimmune rheumatic disease causing large vessel vasculitis. Onset is typically between the ages of 20-30. It is associated with substantial morbidity and mortality, notably due to its effects on the cardiovascular system. It has a poorly understood global epidemiology. Our objective was to systematically review the available evidence in order to calculate the incidence rate of TAK. Methods: Three databases (Medline, PubMed and Embase) were searched in November 2019 and the results were screened by two reviewers. A random effects meta-analysis was then conducted in R to calculate the overall incidence rate. Heterogeneity was assessed using I2. The quality of the studies was assessed using an adapted Newcastle-Ottawa scale. Further sub-group analyses were performed by quality, sex, research setting and geographical location. Publication bias was assessed using a Begg’s funnel plot. Results: The incidence rate for TAK per million person-years with 95% confidence intervals was 1.11 per million person years (95% CI 0.70 – 1.76). The heterogeneity in the data was extremely high in all analyses, which suggests that there was considerable variation in incidence rates across the different populations studied. TAK was found to be more common in women (incidence rate 2.01 per million person-years, 95% CI 1.39-2.90). Conclusions: TAK is an extremely rare disease. It affects women more commonly than men. There is considerable variation in the incidence rate between populations. We suggest that future research should focus on discrete populations in order to better identify genetic and environmental risk factors

Can granulomatosis with polyangiitis be diagnosed earlier in primary care? A case-control study

Background: People with granulomatosis with polyangiitis (GPA) commonly describe long delays before diagnosis. Aim: To study the natural history of GPA prior to diagnosis using primary care data, and determine whether clinical features could be identified to help earlier diagnosis.DesignCase-control study using the Clinical Practice Research Datalink. Methods: We compared primary care activity and clinical features between cases and 10 matched controls. Results: We identified 757 cases and matched 7,546 controls. Compared to controls, cases had more GP consultations and overall healthcare activity in the five years prior to their diagnosis, with a marked increase in the year before diagnosis, and particularly in the last 3 months. However, consultations were mostly for symptoms that were not specifically related to GPA. In the year prior to diagnosis, the most frequent and strongly predictive clinical features of GPA were Ear Nose and Throat (ENT) symptoms (34.5% of cases, odds ratio (OR) 10.5, 95% confidence intervals (CI) 8.6-12.7), and general (constitutional) symptoms (21.5% of cases, OR 9.0, 95% CI 7.1-11.3). In the year before diagnosis a larger number of cases attended secondary care (382, 50.5%) than had records of clinical features of GPA. Conclusions: After discussing our findings, we conclude it would be difficult to identify cases of GPA earlier in primary care. Our results support a need for heightened awareness of this condition among secondary care clinicians, especially those assessing emergency admissions, and in the clinics which were most frequently attended by cases 3-12 months prior to diagnosis