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Publisher Copyright: © This work is licensed under a Creative Commons Attribution 4.0 International License Background: the prevalence of venous thromboembolism (VTE) in Behcet’s disease (BD) is around 40%, though recognition of BD in a thrombosis clinic has been poorly addressed. Objective: to evaluate the prevalence of signs and symptoms leading to the diagnosis of BD in a thrombosis clinic compared to patients attending a general haematology clinic and to healthy controls. Design: cross-sectional case-double control anonymous questionnaire survey. Participants: consecutive patients with spontaneous VTE (n=97) attending a thrombosis clinic, consecutive patients from a general haematology (GH) clinic (n=89) and controls (CTR). Results: BD was diagnosed in 1.03% of VTE participants, in 2.2% of GH participants and in 1.2% of healthy CTR. Exhaustion was more common reported in participants from the VTE group (15.6%) than in those from the GH group (10.3%) and from the healthy CTR (3%) (p=0.06); the sum of signs and symptoms of BD clustered in the VTE group (89.5%) compared to the GH (72.4%) and the CTR (59.7%)( p

Objective: To compare age at occlusion and plasma homocysteine (HC) in patients with idiopathic portal vein thrombosis (PVT) genotyped for methylene tetrahydrofolate reductase (MTHFR) rs1801133 (C®T667 transition), to identify which clinical or laboratory variables influenced age at first PVT, including the prothrombin rs1799963 PT (G®A transition at position 20210) (PT) mutation. Methods: Retrospective cross-sectional cohort survey on 17 MTHFR TT, 45 MTHFR TC and 24 MTHFR CC participants with idiopathic PVT who contributed age, sex, age at PVT, smoking status, plasma HC and natural anticoagulant concentrations. Results: Age at PVT was lower in MTHFR TT than MTHFR TC and CC (31±8 vs. 46±16 vs. 52±14 years, respectively, p=0.001); median (IQR) plasma HC was higher in MTHFR TT than in the other groups [17 (12.6,25.0) vs. 10 (7.4,13.1) vs. 11.9 (8.1,5.8) μmol/l, respectively, p=0.002)]. MTHFR TT and protein C independently predicted age at PVT (pConclusions: MTHFR TT anticipates age at PVT by an average of 20 years compared to other MTHFR genotypes. PT GA had no age lowering effect combined with MTHFR TT. Smoking predicts cavernoma formation, suggesting that smoking may associate with recurrent subclinical PVT before the symptomatic presentation.

The proprotein convertase subtilisin/kexin type 9 (PCSK9) is emerging as a novel cardiovascular risk factor. Levels of PCSK9 in thrombotic primary antiphospholipid syndrome (PAPS) have never been investigated.Cross sectional comparison of baseline characteristics of 91 PAPS patients enrolled in the multicenter prospective ATHERO-APS cohort study. PCSK9 levels were categorized into tertiles and the association with arterial and recurrent thrombosis were assessed by univariable and multivariable logistic regression analysis.Median age was 51 years and 71.4% (n = 65) were women. Overall, 33% (n = 30) experienced an arterial event while 31% (n = 28) had recurrent thrombotic events. Median PCSK9 levels were 1243 (1100-1650) pg/ml. Patients in the third PCSK9 tertile (1458 pg/ml) showed a higher prevalence of dyslipidemia, lupus anticoagulant positivity and a history of previous arterial and recurrent thrombosis than patients in the first and second tertile. PCSK9 levels were higher in arterial than venous thrombosis (1502 vs. 1180 pg/ml, p = 0.002), and in patients with recurrent vs isolated thrombosis (1680 vs. 1150 pg/m, p 0.001). High plasma PCSK9 levels were associated with a 4-fold increase risk for arterial events and with a 10-fold increase risk for recurrent thrombosis after adjustment for confounding factors.These preliminary data suggest that PCSK9 levels are increased in PAPS patients with arterial and recurrent thrombosis. Its role as a possible therapeutic target in PAPS needs further studies.

Ovarian hyperstimulation (OH) is not a contraindication in selected women with antiphospholipid syndrome and/or systemic lupus erythematosus (SLE). A young woman who was persistently positive antiphospholipid antibodies, low protein free S and homozygous for the paraoxonase G192A mutation underwent three courses of OH; after the fourth course she developed severe thromboembolism and clinical features of SLE despite the use of a gonadotropin-releasing hormone agonist alongside aspirin that should have minimised the risk of OH syndrome. We provide a pathogenetic interpretation of these events in the light of the patient’s investigations.

This paper describes an experiment conducted into the use of hypermedia as an integrated information system development tool. The use of hypertext as a software engineering development tool has been extensively explored with notable success. This has led to the derivation and development of the fully integrated environment for layered development (FIELD) system. To supplement the investigations, a complex environmental control organization, the Norfolk and Suffolk Broads Authority was used as a case study. The development of FIELD and its use for the documentation and development of a prototype information system for the Norfolk and Suffolk Broads Authority is described and the overall methodological implications for future information system development is discussed. It is postulated that the development of such a complex information system may be improved by this method of using hypertext. This paper reports on the initial findings of the experiment into the properties of hypermedia as used in this manner, and the appropriateness of these ideas and their implementation for general applications.

How do we consider problems and models in the practice of simulation? It is our possibly contentious observation that simulation model solving seems to be more critical to the mission of simulation modeling than problem solving. Inspired by the theme of this year's Winter Simulation Conference, we ask the question, "Is problem solving, or simulation model solving, mission critical?" To investigate this we look at three perspectives, those of the textbook, the article and the editorial. The textbook perspective is the balance of the "traditional" view of simulation presented by the academic textbook against practical experience. The article perspective is a classification of papers published in four leading simulation journals in the year 2004 (ACM TOMACS, SIMULATION, Simulation Modelling Practice and Theory, and Simulation & Gaming). The editorial perspective is a discussion of editorial policy presented by the same journals. Our findings show that our observation is not contradicted.

Business Process (BP) literature promotes the value of business processes as essential gearwheels that help organizations to reach their goals. Similarly, many process design approaches claim that Information Technology (IT) is a major enabler of business process, a view also shared by the Information Systems (IS) community. Despite this, BP and IS approaches do not provide clear guidance on how to assess the benefits that a given IS design may bring to the BP prior the IS implementation. Nor is clear indication of which modeling techniques could be used to assess such relationship. This paper uses the insights gained during a UK funded research project, namely ASSESS-IT, that aimed to depict the dynamic relationships between BP and IT to propose an alternative framework to develop BP simulation models that depict the dynamic behavior of the relationships between BP and IS.