Hereditary leiomyomatosis and renal cell carcinoma: a case series and literature review.

BACKGROUND: Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) is a rare genodermatosis characterized by cutaneous leiomyoma (CLM), uterine leiomyoma (ULM) and renal cell carcinoma (RCC). Five HLRCC patients are presented with a compiled database of published HLRCC cases to increase understanding of HLRCC. Furthermore, a surveillance program is suggested. Our review is based on a PubMed search which retrieved case reports and cohort studies published before November 2019. The search yielded 97 original papers with a total of 672 HLRCC patients. RESULTS: CLMs were present in 474 patients (71.5%), developed at the mean age of 28 years. Five patients had cutaneous leiomyosarcomas. ULMs were present in 356 women (83%), while two had uterine leiomyosarcoma. ULMs were diagnosed at a mean age of 32 years, with the youngest diagnosed at age 17 years. The most common surgical treatment for ULMs was hysterectomy, performed at a mean age of 35 years, with the youngest patient being 19 years old. RCCs were present in 189 patients (34.9%), of which half had metastatic disease. The mean age of diagnosis was 36 years with the youngest patient diagnosed with RCC at the age of 11 years. CONCLUSION: We suggest a surveillance program for HLRCC including a dermatological examination once every 2 years, annual magnetic resonance imaging starting at the age of 10 years to monitor for early RCCs, annual gynecological examinations from the age of 15 years and counseling regarding risk of hysterectomy and family planning at the age of 18 years. CLMs are often the earliest manifestation of HLRCC, which is why recognizing these lesions, performing a biopsy, and making a prompt referral to genetic counseling is important in order to diagnose HLRCC early.

The WaterProtect governance guide: Experiences from seven agricultural and drinking water production catchments across Europe.

Although collaborative, multi-level and adaptive governance is put forward as the right approach to manage water resources, it remains a challenge for local water managers to engage with multiple understandings and perspectives and to move towards more transdisciplinary approaches in water governance. In this research, we address this need by proposing a practical guide for water governance assessment and improvement, existing of three successive steps, i.e. (1) an assessment of water governance, (2) the implementation of a multi-actor process and (3) an evaluation of achievements. This guide is tested in the seven action labs of the WaterProtect project, which have in common that they are drinking water catchments suffering from agricultural pollution throughout Europe. By implementing the guide in the different action labs, we find that water managers appreciated the integrated approach of the guide, helping them in the efficient organization of the water governance process while taking into account the human dimension. Furthermore action lab leaders evaluated the governance guide as being hands-on and practical, allowing them to redirect and open up the governance process. Despite this, they welcomed the presence of a facilitator, who boosted the local action lab leaders' confidence and motivation to put things into motion. Looking at the results, we find that over the different action labs a lot of progress has been made towards intermediary goals, however that an improvement of the water quality cannot be expected in the short time frame of a project. We therefore recommend improving the design of the guide with insights from transition management, which will allow local water managers to better address change at different time-scale levels.

[Cardiac CT].

Cardiac CT has become a frequently used diagnostic modality in Denmark, especially for the evaluation of coronary artery disease indicated by angina pectoris/dyspnoea. Indications have recently expanded to include evaluation of valvular heart disease, pre- and post-procedural evaluation related to several invasive procedures as well as some congenital and pediatric cardiac conditions. As described in this review, improved image quality and reduced radiation, owing to technological advances as well as a high number of well-performed scientific studies, have paved the way for this development.

Drivers and barriers for taking account of geological uncertainty in decision making for groundwater protection.

The geological conceptual model is considered a major source of uncertainty in groundwater modelling and well capture zone delineation. However, how to account for it in groundwater policy and management remains largely unresolved. We explore the drivers and barriers to account for geological conceptual uncertainty in groundwater protection amongst decision makers and stakeholders in an agricultural groundwater catchment in Denmark. Using a groundwater model, we analyze the impact of alternative geological conceptual models on capture zone delineation. A focus area, which covers multiple modelled capture zones, is defined and considered for groundwater protection. Model uncertainty and focus area are discussed at two workshops, one with local and national stakeholders and another with local farmers. The drivers to account for model uncertainty include: i) safer drinking water protection by considering a larger area for protection than identified from a single geological model; and ii) stability over time of management plans. The main barrier is the additional cost to the stakeholders for the protection of a larger area. We conclude that integration of geological uncertainty in groundwater protection plans may be improved through: i) better communication between the research community and the national water authority; ii) more constraining guidelines regarding the estimation of geological uncertainty; and iii) the development of a framework ensuring knowledge transfer to the local water authorities and detailing how to integrate uncertainty in management plans.

Prognostic assessment of stable coronary artery disease as determined by coronary computed tomography angiography: a Danish multicentre cohort study.

Aims: To examine the 3.5 year prognosis of stable coronary artery disease (CAD) as assessed by coronary computed tomography angiography (CCTA) in real-world clinical practice, overall and within subgroups of patients according to age, sex, and comorbidity. Methods and results: This cohort study included 16,949 patients (median age 57 years; 57% women) with new-onset symptoms suggestive of CAD, who underwent CCTA between January 2008 and December 2012. The endpoint was a composite of late coronary revascularization procedure >90 days after CCTA, myocardial infarction, and all-cause death. The Kaplan-Meier estimator was used to compute 91 day to 3.5 year risk according to the CAD severity. Comparisons between patients with and without CAD were based on Cox-regression adjusted for age, sex, comorbidity, cardiovascular risk factors, concomitant cardiac medications, and post-CCTA treatment within 90 days. The composite endpoint occurred in 486 patients. Risk of the composite endpoint was 1.5% for patients without CAD, 6.8% for obstructive CAD, and 15% for three-vessel/left main disease. Compared with patients without CAD, higher relative risk of the composite endpoint was observed for non-obstructive CAD [hazard ratio (HR): 1.28; 95% confidence interval (CI): 1.01-1.63], obstructive one-vessel CAD (HR: 1.83; 95% CI: 1.37-2.44), two-vessel CAD (HR: 2.97; 95% CI: 2.09-4.22), and three-vessel/left main CAD (HR: 4.41; 95% CI :2.90-6.69). The results were consistent in strata of age, sex, and comorbidity. Conclusion: Coronary artery disease determined by CCTA in real-world practice predicts the 3.5 year composite risk of late revascularization, myocardial infarction, and all-cause death across different groups of age, sex, or comorbidity burden.

The Western Denmark Cardiac Computed Tomography Registry: a review and validation study.

BACKGROUND: As a subregistry to the Western Denmark Heart Registry (WDHR), the Western Denmark Cardiac Computed Tomography Registry (WDHR-CCTR) is a clinical database established in 2008 to monitor and improve the quality of cardiac computed tomography (CT) in Western Denmark. OBJECTIVE: We examined the content, data quality, and research potential of the WDHR-CCTR. METHODS: We retrieved 2008-2012 data to examine the 1) content; 2) completeness of procedure registration using the Danish National Patient Registry as reference; 3) completeness of variable registration comparing observed vs expected numbers; and 4) positive predictive values as well as negative predictive values of 19 main patient and procedure variables. RESULTS: By December 31, 2012, almost 22,000 cardiac CTs with up to 40 variables for each procedure have been registered. Of these, 87% were coronary CT angiography performed in patients with symptoms indicative of coronary artery disease. Compared with the Danish National Patient Registry, the overall procedure completeness was 72%. However, an additional medical record review of 282 patients registered in the Danish National Patient Registry, but not in the WDHR-CCTR, showed that coronary CT angiographies accounted for only 23% of all nonregistered cardiac CTs, indicating >90% completeness of coronary CT angiographies in the WDHR-CCTR. The completeness of individual variables varied substantially (range: 0%-100%), but was >85% for more than 70% of all variables. Using medical record review of 250 randomly selected patients as reference standard, the positive predictive value for the 19 variables ranged from 89% to 100% (overall 97%), whereas the negative predictive value ranged from 97% to 100% (overall 99%). Stratification by center status showed consistently high positive and negative predictive values for both university (96%/99%) and nonuniversity centers (97%/99%). CONCLUSION: WDHR-CCTR provides ongoing prospective registration of all cardiac CTs performed in Western Denmark since 2008. Overall, the registry data have a high degree of completeness and validity, making it a valuable tool for clinical epidemiological research.

Which exercise test to use for chest pain from an anomalous coronary artery.

Chest pain in children and young people is a frequent cause of contact to general practitioners and outpatient clinics. However, in children, chest pain is typically benign and self-limiting; it is not usually a manifestation of organic disease, and it is very rarely of cardiac origin. The cause of chest pain often remains undiagnosed. There are a number of chronic conditions known to be associated with recurrent chest pain. Symptoms and signs include crushing left-sided precordial pain, pain radiating to the left arm or the jaw, pain onset with exercise and subsiding at rest (with asthma excluded), and an abnormal cardiovascular examination suggests referral for cardiac evaluation. We here report a case of stable angina pectoris in the young.

Current use of impact models for agri-environment schemes and potential for improvements of policy design and assessment.

Agri-Environment Schemes (AES) to maintain or promote environmentally-friendly farming practices were implemented on about 25% of all agricultural land in the EU by 2002. This article analyses and discusses the actual and potential use of impact models in supporting the design, implementation and evaluation of AES. Impact models identify and establish the causal relationships between policy objectives and policy outcomes. We review and discuss the role of impact models at different stages in the AES policy process, and present results from a survey of impact models underlying 60 agri-environmental schemes in seven EU member states. We distinguished among three categories of impact models (quantitative, qualitative or common sense), depending on the degree of evidence in the formal scheme description, additional documents, or key person interviews. The categories of impact models used mainly depended on whether scheme objectives were related to natural resources, biodiversity or landscape. A higher proportion of schemes dealing with natural resources (primarily water) were based on quantitative impact models, compared to those concerned with biodiversity or landscape. Schemes explicitly targeted either on particular parts of individual farms or specific areas tended to be based more on quantitative impact models compared to whole-farm schemes and broad, horizontal schemes. We conclude that increased and better use of impact models has significant potential to improve efficiency and effectiveness of AES.

Landscape changes in agrarian landscapes in the 1990s: the interaction between farmers and the farmed landscape. A case study from Jutland, Denmark.

Recent landscape changes in a farmed landscape are analysed and related to farm and farmer characteristics. It is assumed that farm and farmer characteristics serve as mediators of large scale or macro driving forces of change-in the present case, a changing farming context including demands for a more environmentally friendly farming practise and a reduced output. The results are based on multivariate analyses of data collected from structured interviews of 160 farmers in a case study area, in central Jutland, measuring 5000 ha. The analysis shows that farmers are highly involved in landscape changes. The investigated landscape changes include creation and removal of landscape elements as well as certain management changes. The most common activity was creation of elements: hedgerows, small woodlands and conversion of rotational arable land to permanent grassland, whereas removal of elements, mainly hedgerows and semi-natural grasslands, were seen less frequently. Management changes like abandonment of permanent grassland were widespread. The results indicate a general extensification of the land use and the authors interpret the results partly as an indication of a change from productivism to a more multifunctional agricultural regime. The observed landscape changes at the farm level show a low, but structured relationship with the current farm and farmer characteristics, meaning that landscape changes were undertaken by various farmers and on various farms. On a general level, however, the age of the farmer and the duration of farm ownership seem to have a major influence on the landscape changes.