tbuckley/cabot-search huggingface dataset:
Abstract 124: Patient Condition Adaptive Path System of Kawasaki Disease
Authors: Masako Fujiwara, Tohru Kobayashi, Satoko Tsuru, Hiroyuki Ida
Journal: Circulation (2015)

Background: In Japan, 2 guidelines are published, the clinical guidelines for medical treatment of acute stage Kawasaki disease(KD)(2012) and guidelines for diagnosis and management of cardiovascular sequelae in KD(2013). Patient Condition Adaptive Path System (PCAPS) is a technique to structure clinical knowledge. It places “patient condition” as a core, to which multiple “target conditions” are linked. On the other hand, patients of KD ware focused the severity of the disease and therapeutic strategy influences the improvement. Purpose: The purpose is confirming the PCAPS KD contents, which complied two Japanese Guidelines and to evaluate adaption of the contents. Methods: PCAPS content is composed of Clinical Process Chart (CPC) and Unit Sheet (US).CPC is an overhead view of clinical path consisting of a chain of units. CPC was made according the guidelines, and coronary evaluation, CHF, cardiac catheterization and ACS unit can activate on time. CPC stratify the patient’s severity. US are composed of specific healthcare tasks in a unit. Results: We confirm PCAPS KD contents on the base of 2 guidelines. We can evaluate diagnostic process and severity of KD by route analysis using CPC (figure). We can visualize relationship between treatments and severity by US. US are effective to support the decision on treatment and examinations. From the analysis, there are no lack of the unit and route, and confirm the advice to decision making. Conclusions: PCAPS can easily analyze the severity and clinical process from CPC route analysis because PCAPS is electrical path which can automatically store the data of each hospital. From US data, there are possibilities to find new severity score.
Event Rates and Risk Factors for the Development of Diabetic Ketoacidosis in Adult Patients With Type 1 Diabetes: Analysis From the DPV Registry Based on 46,966 Patients
Authors: Hannes Kalscheuer, Jochen Seufert, Stefanie Lanzinger, Joachim Rosenbauer, Wolfram Karges, Dominik Bergis, Julia K. Mader, Stefan Zimny, Sebastian M. Schmid, Michael Hummel, Wolfgang Kerner, Reinhard W. Holl
Journal: Diabetes Care (2019)

Diabetic ketoacidosis (DKA) is a life-threatening complication of type 1 diabetes mellitus (T1DM) that results from absolute insulin deficiency and is marked by acidosis, ketosis, and hyperglycemia (1). Therefore, prevention of DKA is one goal in T1DM care, but recent data indicate increased incidence (2).

For adult patients, only limited data are available on rates and risk factors for development of DKA, and this complication remains epidemiologically poorly characterized. The Diabetes Prospective Follow-up Registry (DPV) has followed patients with diabetes from 1995. Data for this study were collected from 2000 to 2016. Inclusion criteria were diagnosis of T1DM, age at diabetes onset ≥6 months, patient age at follow-up ≥18 years, and diabetes duration ≥1 year to exclude DKA at manifestation. DKA was defined as serum pH <7.3, and rates of DKA were analyzed based on a Poisson regression model accounting for overdisperson stratified by sex, age, diabetes duration, HbA1c, treatment regimen, size of diabetes center, and migration background, i.e., whether the patient or one or both parents were born outside the countries of the registry. A diabetes center that treated ≥50 adult patients with T1DM in the year 2016 was considered large.

In total, 46,966 patients were included in this study (average age 38.5 years [median 21.2], …
Prevention is the role of governments, not health systems
Authors: Fiona Godlee
Journal: BMJ (2019)

We are told that prevention is at the heart of the long term plan for the NHS, published last week (doi:10.1136/bmj.l80; doi:10.1136/bmj.l85). This sounds like good news; prevention is, after all, better than cure. But we should be wary of suggestions that it’s the role of the NHS to prevent disease. There are many good elements of secondary prevention in the plan, as Rachel Chapman and John Middleton point out this week (doi:10.1136/bmj.l218), but there are also many other calls on the NHS’s limited …
OP0079-PARE ‘awareness week for rheumatic diseases- 8–14 may 2017’
Authors: B. Boteva, H.L. Bankova
Journal: Annals of the Rheumatic Diseases (2018)

Don't delay, connect today! <h3>Background</h3> Among the main priorities that Bulgarian Organisation for people with rheumatic diseases follow is the dissemination of knowledge and information about RMD's. An important initiative is the annual awareness week about RMD's which is organised since 2015. The awareness week is organised in May and the idea occurred after EULAR's Knowledge transfer program in which BOPRD took part in 2013. <h3>Objectives</h3> In 2017 the focus was on two of the rheumatic diseases – SLE and fibromyalgia. SLE and fibromyalgia are not well known in our country that is why we decided to spread awareness among general public and policymakers. The main goal of the 2017 awareness week for RMD's was to: inform the society and policymakers about the social significance of SLE and fibromyalgia Spread awareness about the most common symptoms of SLE and fibromyalgia Inform people with SLE, fibromyalgia and other RMDs how they can manage symptoms as anxiety, depression, and tension. <h3>Methods</h3> The 2017 awareness week about RMDs was organised in the period <b>8–14 May 2017.</b> One of the most important accents was a seminar, organised on 8 of May. We have invited as lecturer Gergana Radovich who is one of the international T-Tapp trainers. She holds a Master degree in Cognitive science, as well as certificates for Psych-K Facilitator and EFT Practitioner. T-Tapp is a series of movements designed to put the body in proper functional alignment, restore metabolic function and stands on its own as a complete workout. Such a seminar for people with RMDs was not organised before. The participants had the chance to learn more about EFT and T-Tapp and how these techniques can benefit their condition and help them fight fear, tension, and anxiety. At 10-th of May – World lupus day a discussion about the problems that people with SLE face was organised. In the evening the National Palace of Culture (a famous building in the capital of Bulgaria) was lit in purple light to support people with SLE. <h3>Results</h3> The 2017 awareness week for RMDs was very successful. All publications and media interviews that were published at the organisation's website and in other websites reached more than 4000 people in total. 88 people liked the organisation's Facebook page in the period 8–14 May 2017. We have managed to continue to offer online EFT and T-Tapp lessons to the members of the organisation <h3>Acknowledgements</h3> Miglena Ivanova; Snezhana Bozhinova; Janeta Cherpokova; PRCare;Bozhidar Ivkov;Hristina Bankova <h3>Disclosure of Interest</h3> None declared
Differential Impact of Interferon Alpha on JAK2V617F and Calr Mutated Hematopoietic Stem and Progenitor Cells in Classical MPN
Authors: Matthieu Mosca, Lamia Lamrani, Christophe Marzac, Amandine Tisserand, Valérie Edmond, Tracy Dagher, Mira El-Khoury, Caroline Marty, Hugo Campario, Nicole Casadevall, Eric Solary, Hana Raslova, Bruno Cassinat, Stefan N Constantinescu, Jean-Jacques Kiladjian, Francois Girodon, Jean-Luc Villeval, William Vainchenker, Isabelle Plo
Journal: Blood (2018)
Determining the reasons for poorly controlled asthma in an adolescent
Authors: Gary James Connett, Lucy Anne Connett, Mike Thomas
Journal: BMJ (2019)

### What you need to know

A 15 year old girl with a history of mild asthma visits her GP. She has been waking at night coughing and has missed several days of school because of wheeze and chest tightness. Her first GCSE exam is three weeks away. Two years ago, at her last asthma review, her symptoms were well controlled with inhaled beclomethasone dipropionate 200 μg twice daily. 

Periods of worsening asthma are common in young people,1 and non-specialists may be the first port of call. Poor symptom control is a risk factor for acute asthma attacks and is associated with increased mortality.2 Assessing asthma can be more challenging than it might at first appear. Identifying psychosocial stressors and how they might affect adherence to treatment including following a self management plan, the perception of symptoms, as well as direct effects on bronchial hyperreactivity, can be key to helping the young person.3 This article offers an approach to a consultation with an adolescent with asthma symptoms related to psychosocial factors and dysfunctional breathing.

Spend time with the young person to build rapport. This may be key to identifying their needs, priorities, and the underlying causes of their symptoms.

Assess severity —A …
LEFT VENTRICULAR GLOBAL LONGITUDINAL STRAIN AND LONG-TERM PROGNOSIS IN PATIENTS WITH CHRONIC OBSTRUCTIVE PULMONARY DISEASE AFTER ACUTE MYOCARDIAL INFARCTION
Authors: Laurien Goedemans, Rachid Abou, Georgette Hoogslag, Nina Marsan, Victoria Delgado, Jeroen J. Bax
Journal: Journal of the American College of Cardiology (2018)
THU0624 Colchicine: an effective treatment option for unclassified autoinflammatory diseases in children
Authors: J.B. Kuemmerle-Deschner, A.-L. Schock, S. Hansmann, S.M. Benseler
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Children and adults with clinically and genetically defined autoinflammatory diseases (AID) including CAPS, TRAPS and HIDS can receive expensive Interleukin-1 (IL-1) inhibitors in many countries around the world. However, patients suffering from unclassified autoinflammatory conditions characterised by recurrent fevers and organ dysfunction and the absence of a known pathogenic mutation commonly have no access to these treatment options. <h3>Objectives</h3> The aim of this study was to explore the efficacy and safety of colchicine treatment in children and adults with autoinflammatory diseases without pathogenic mutations. <h3>Methods</h3> Consecutive children and adults with autoinflammatory diseases without pathogenic mutations treated with colchicine were included in this single centre study and observed for a median of 12,94 months (range 1,25–66,73). Clinical features, autoinflammatory disease activity indices (AIDAI), inflammatory markers ESR, CRP, SAA and S100, frequency and duration of flares and physician global assessment of disease activity (VAS) were recorded serially and compared at baseline and while receiving Colchicine therapy. <h3>Results</h3> A total of 39 patients were included in the study. These were 16 girls and 23 boys, median age at start of colchicine therapy was 4 years (range 1–54). The diagnoses included PFAPA in 15, mutation-negative FMF in 11, autoinflammation with low-penetrance variants in nine (all <i>NLRP3)</i> and other unclassified AID in four patients. Recurrent fever was the leading symptom, mostly associated with arthralgia and myalgia. The mean disease activity decreased from 4.4 at baseline to 2.2 on colchicine. Mean SAA-levels decreased from 159 to 63.3 mg/L, CRP levels from 6.4 to 2.3 mg/dl. Flare frequency was reduced in 72% and remained unchanged in 28% of patients. Flare duration was reduced in 82%, unchanged in 14% and increased in only 4% of patients. Most common adverse events were abdominal pain and nausea in 50% of patients and appeared to be dose dependent. <h3>Conclusions</h3> Children and adults with unclassified autoinflammatory diseases may benefit significantly for colchicine therapy. Control of clinical disease activity and improved inflammatory markers were documented in 59% of patients. Colchicine should be considered in patients with active inflammatory disease with no access to IL-1 inhibitors. Controlled trials are needed to further explore this approach. <h3>Disclosure of Interest</h3> None declared
AB1427-HPR The role of parents’ awareness in physical activity in children with juvenile idiopathic arthritis
Authors: B. Monek, D Vitár, R. Chrobot, I. Orbán, E. Kiss, G. Poór
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Whilst Juvenile Idiopathic Arthritis (JIA) is one of the most common chronic diseases amongst children, the impact of physical therapy on affected children's life-quality and the importance of an appropriate and regular physical activity get less attention than they ought to. In addition, there are no such studies about the role of parents' awareness in connexion with the regular physical activity for children with JIA available yet. <h3>Objectives</h3> The purpose of the survey was to evaluate the awareness of parents in connexion with the Juvenile Idiopathic Arthritis' diagnose, its treatment options, the importance of regular physical activity and how it affects children's life-quality. Everyday experiences say that children with JIA take part in less physical activities than their healthy mates do. Our aim was to detect how important regular physical activity is for parents. <h3>Methods</h3> This is a descriptive analysis of our self-compiled questionnaire which has 41 questions. It is being filled in both online and in paper forms since the February of 2017 in the National Institute of Rheumatology and Physiotherapy on the Department of Clinical Immunology, Adults' and Children's Rheumatology. Participants of the study are parents whose child has the diagnose of Juvenile Idiopathic Arthritis and ages between 2–18. Parents whose child has not differentiated diagnose or is under 2 years are excluded. <h3>Results</h3> 48 answers met the criteria, 6 answer sheets were excluded. In case the children's condition get worse, 20,8% of parents marked wrong parental measures. All parents acknowledge the importance of physical activity, but only 63% of children do regular physiotherapy at home. Parents could not choose from or rank the appropriate and useful ways of physical activities. Beside the medical team (doctor, physiotherapist, nurse) parents get information from media and internet. They would like to get further information personally in words, in written forms or pamphlets. Parents of children with JIA miss psychic support, alternative treatment options and customised, complex information from the general treatment. <h3>Conclusions</h3> The findings of this study support the fact that parents of children with Juvenile Idiopathic Arthritis are well informed about the JIA's inflammatory nature and its symptoms, but they have few and wrong information in connexion with regular physical activity. They have a lack of knowledge about the different kinds of physical activities and sports' effects on the disease, due to which they choose ergonomically wrong kinds of activities in schools or pre-schools. Based on our results, we would like to develop a complex educational program including physical therapy as well. <h3>References</h3> [1] K Barut, A Adrovic, S Şahin, Ö Kasapçopur. Juvenile Idiopathic Arthritis. Balkan Med J. 2017Mar; 34(2): 90–101. <h3>Disclosure of Interest</h3> None declared
THU0249 More than 60% patients with spondyloarthritis report peripheral manifestations: an ancillary analysis of the asas-comospa study
Authors: C. López-Medina, A. Moltó, M. Dougados
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Peripheral manifestations (arthritis, enthesitis and dactylitis) can be observed in patients with Spondyloarthritis (SpA)<sup>1</sup>, but the factors associated with their presence are not well known. Studies are needed in order to thoroughly evaluate these symptoms. <h3>Objectives</h3> a) To describe the prevalence of peripheral manifestations in patients with SpA in a world-wide population; and b) to determine the factors associated with the presence of these manifestations. <h3>Methods</h3> Data from the ASAS-COMOSPA study were analysed. The prevalence of each peripheral manifestation was evaluated with regard to the criteria fulfilled by the patient (ASAS axial, ASAS peripheral, CASPAR) and with regard to the time of occurrence of axial symptoms (before/concomitant/after). Factors associated with the presence of these peripheral manifestations were also explored by univariate and multivariate logistic regression. <h3>Results</h3> Out of the 3984 patients included in ASAS-COMOSPA, 2562 (64.3%) reported, at least, one peripheral manifestation. Among these, 2051 patients (51.5% from the total database) had current or past history of peripheral arthritis, being more frequent among patients who met CASPAR and Peripheral ASAS criteria (see figure 1). Involvement was more frequently oligoarticular (40.2%) and appearing after axial symptom onset (48.9%). Multivariate analysis showed that patients from South America [OR 2.45, (95%CI 1.93–3.11)], the presence of enthesitis [OR 2.46, (95%CI 2.11–2.86)], dactylitis [OR 6.46, (95%CI 4.78–8.71)], skin psoriasis [OR 2.46, (95%CI 1.79–3.37)], HLAB27+ [OR 0.83, (95%CI 0.72–0.97)] and inflammatory back pain (IBP) [OR 0.37, (95%CI 0.29–0.47)] were associated with peripheral arthritis. A total of 1506 (37.8%) and 618 (15.6%) patients reported enthesitis and dactylitis, respectively. Both occurred after axial symptoms onset in 58.3% and 60.8% of the patients, respectively. Similar results than peripheral arthritis were obtained in the multivariate analysis regarding these two peripheral manifestations, with exception of IBP and HLAB27+, which were not associated with enthesitis. <h3>Conclusions</h3> Peripheral manifestations appear in 64% of patients with SpA and in more than 50% after axial symptoms onset. Peripheral arthritis, were more frequently mono- or oligo- rather than poly-articular, and the presence of psoriasis or any of the three peripheral manifestations acts as risk factor for the development of other peripheral symptoms. <h3>Reference</h3> [1] Dougados M, d'Agostino MA, Banessiano J, et al. Joint Bone Spine2011Dec;78(6):589–603. doi:10.1016/j.jbspin.2011.01.013 <h3>Disclosure of Interest</h3> None declared
The Presence of Defective Epstein-Barr Virus (EBV) Infection in Patients with EBV-Associated Hematological Malignancy
Authors: Yusuke Okuno, Takayuki Murata, Yoshitaka Sato, Hideki Muramatsu, Yoshinori Ito, Takahiro Watanabe, Tatsuya Okuno, Norihiro Murakami, Kenichi Yoshida, Akihisa Sawada, Masami Inoue, Keisei Kawa, Masao Seto, Koichi Ohshima, Yuichi Shiraishi, Kenichi Chiba, Hiroko Tanaka, Satoru Miyano, Yohei Narita, Masahiro Yoshida, Fumi Goshima, Junichi Kawada, Tetsuya Nishida, Hitoshi Kiyoi, Seiichi Kato, Shigeo Nakamura, Satoko Morishima, Shigeyoshi Fujiwara, Norio Shimizu, Yasushi Isobe, Masaaki Noguchi, Atsushi Kikuta, Keiji Iwatsuki, Yoshiyuki Takahashi, Seiji Kojima, Seishi Ogawa, Hiroshi Kimura
Journal: Blood (2018)
AB0962 Irisin levels are associated with exercise, pain and function in patients with knee osteoarthritis
Authors: C. Orellana, J. Calvet, N. Navarro, C. Galisteo, J. Gratacós, M. Larrosa
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Muscle strengthening and aerobic exercise have been shown to improve joint pain and function in patients with knee osteoarthritis (OA). Irisin is a hormone-like myokine synthetized by skeletal muscle and its secretion appears to be related to physical activity in healthy people. <h3>Objectives</h3> To determine the levels of irisin synovial fluid and plasma and evaluate their association with exercise level and pain and function in patients with symptomatic knee OA. <h3>Methods</h3> Cross-sectional study with systematic inclusion of 108 symptomatic primary knee osteoarthritis patients (VAS&gt;4) with ultrasound-confirmed joint effusion. Age, physical exercise, knee osteoarthritis symptoms duration and different anthropometric measurements were collected. Radiographic severity was evaluated according to Kellgren-Lawrence scale. Physical exercise was categorised as never, occasional (less than 150 min per week) or regular (more than 150 min per week. Pain and disability were assessed by the Lequesne algo-functional questionnaire. Irisin was measured by ELISA in synovial fluid and plasma. Summary of clinical data and laboratory parameters and their association with Lequesne scale were performed using non-parametric methods. Medians and Spearman correlation (r) were used for continuous measures, and Mann-Whitney test was applied to categorical variables. This study was approved by the local ethics committee. <h3>Results</h3> Plasma and synovial irisin levels were strongly related (r=0.7). Plasma and joint irisin levels showed an inversely significant association with the level of exercise in patients with symptomatic knee OA: no exercise 763.3 ng/mL, occasional exercise 631.8 ng/mL and regular exercise 523 ng/mL (p&lt;0.01). Patients with severe pain and disability (Lequesne algofunctional score &gt;11) had higher plasma (791.5 vs 680.4 ng/mL, p&lt;0.05) and synovial (711.4 vs 468.7 ng/mL, p&lt;0.05) irisin levels compared to patients with less disability and pain. No relationship was found between irisin concentration in plasma or synovial fluid and radiographic severity. <h3>Conclusions</h3> Irisin levels were associated with pain and function in patients with knee osteoarthritis. Irisin levels were negatively associated with exercise level in this population. <h3>Disclosure of Interest</h3> None declared
Transition from metabolically benign to metabolically unhealthy obesity and 10-year cardiovascular disease incidence: The ATTICA cohort study
Authors: Matina Kouvari, Demosthenes B. Panagiotakos, Mary Yannakoulia, Ekavi Georgousopoulou, Elena Critselis, Christina Chrysohoou, Dimitrios Tousoulis, Christos Pitsavos
Journal: Metabolism (2019)
A Rationally Designed Novel Polymer for Safe and Synergistic Delivery of High Dose Bortezomib, Pomalidomide/Lenalidomide, and Dexamethasone for Multiple Myeloma
Authors: Alexandre Detappe, Hung Nguyen, Clelia Mathieu, Yivan Jiang, Michael Agius, Oksana Zavidij, Brianna Berrios, Yujia Shen, Jeremiah A Johnson, Paiman Peter Ghoroghchian, Irene M. Ghobrial
Journal: Blood (2018)