tbuckley/cabot-search huggingface dataset:
AB0628 ANALYSIS OF CLINICAL FEATURES OF 15 CASES OF IGG4-RELATED DISEASES
Authors: Ge Yang, Yazhen Su, Gailian Zhang, Liyun Zhang, Ke Xu
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background:</h3> IgG4-related disease is an autoimmune disease of unknown cause. <h3>Objectives:</h3> Summarizing the clinical characteristics of IgG4-related diseases to improve the understanding of clinicians and achieve early diagnosis and reasonable treatment. <h3>Methods:</h3> The clinical data of 15 patients with IgG4-related diseases diagnosed in our hospital from 2014 to 2018 were retrospectively analyzed. <h3>Results:</h3> The ratio of male to female was 2:1 in 15 patients. The average age of onset was 56.8 years (34 to 84 years of age). From the first symptom to the diagnosis of 8 days to 4.75 years, the most common first symptom in 15 patients was Submandibular gland swelling, pain, anorexia, cough, the most common affected organs are the pancreas, lungs, submandibular glands. There were 11 cases of histopathological examination by surgery or needle biopsy. The histopathology showed interstitial fibrous tissue hyperplasia with more lymphocyte and plasma cell infiltration. Immunohistochemistry showed that the number of IgG4-positive cells was &gt;50/HPF, and IgG4/IgG was &gt;40%.Two of the 15 patients underwent surgical resection of the affected organ occupying lesions before definitive diagnosis. One patient was treated with mycophenolate mofetil and one patient was given a medium dose of hormone combined with tamoxifen. The remaining 13 patients were treated with simple hormones or combination immunosuppressive agents, including hydroxychloroquine, mycophenolate mofetil, azathioprine, and cyclophosphamide. <h3>Conclusion:</h3> The organs involved in IgG4-related diseases are diverse, the clinical manifestations are not specific, and the hormone combined immunosuppressive therapy is effective. <h3>References</h3> [1] Umehara H, Okazaki K, Masaki Y, et al. Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011[J]. Japanese Journal of Rheumatology, 2012, 22(1):21-30. [2] Okazaki K, Kawa S, Kamisawa T, et al. Japanese clinical guidelines for autoimmune pancreatitis[J]. Pancreas, 2009, 38(8):849-866. <h3>Disclosure of interests:</h3> None declared
uPARAP/Endo180-dependent intracellular collagen degradation - a major pathway of pathophysiological collagen turnover
Authors: Rebecca A. Wagenaar-Miller, Alejandro C. Curino, Lars H. Engelholm, Boye S. Nielsen, ller Lars Kjã, Alfredo A. Molinolo, Kenn Holmbeck, Niels Behrendt, Thomas H. Bugge
Journal: Cancer Research (2005)

4341  The urokinase plasminogen activator receptor-associated protein (uPARAP)/Endo180 is a member of the macrophage mannose receptor family of recycling endocytic transmembrane glycoproteins. uPARAP/Endo180 is a 180 kDa multidomain glycoprotein that consists of an N-terminal cysteine-rich domain, a collagen binding fibronectin type II domain, eight C-type carbohydrate recognition domains, a transmembrane domain, and a short C-terminal cytoplasmic tail. In situ hybridization and immunohistochemical studies reveal that uPARAP/Endo180 is expressed at low levels in homeostatic tissues. However, uPARAP/Endo180 is abundantly expressed by mesenchymal cells located at sites of active tissue remodeling and rapid extracellular matrix turnover. This includes both physiological tissue remodeling (odontogenesis, endochondral and intramembranous ossification) and pathophysiological tissue remodeling (tumor invasion), where uPARAP/Endo180 is found on chondrocytes, fibroblasts, macrophages, myofibroblasts, odontoblasts, osteoblasts, and osteocytes. Surprisingly, genetic ablation of uPARAP/Endo180 leads to a specific and near complete abrogation of the ability of mesenchymal cells to internalize and degrade a range of collagen species. This defect is apparent in both cultured uPARAP/Endo180-deficient fibroblasts and in fibroblast-like cells within primary explants of uPARAP/Endo180-deficient Polyoma virus middle T-induced mammary tumors. This novel uPARAP/Endo180-dependent intracellular collagen degradation pathway is critical to collagen turnover during tumor progression, as evidenced by accumulation of interstitial collagen and impaired growth of Polyoma virus middle T-induced mammary tumors in uPARAP/Endo180-deficient mice. uPARAP/Endo180 expression is conspicuously similar to the major fibrillar collagenase, MT1-MMP. Indeed, preliminary studies suggest that combined deficits in uPARAP/Endo180-dependent intracellular and MT1-MMP-dependent extracellular collagen degradation pathways are incompatible with prolonged postnatal survival of mice. Taken together, these studies identify uPARAP/Endo180-mediated intracellular degradation of collagen as a major pathway for the turnover of this abundant extracellular matrix component within both pathological and physiological contexts.
Inflammation, infection and coagulation disorders
Authors: Per Morten Sandset
Journal: HemaSphere (2019)

Learning goals Understand the most important interactions between inflammation and coagulation. Understand the mechanism(s) and role of NETs formation. Understand the pathophysiology of DIC. Understanding and knowledge of correctional treatment of different coagulopathies. Introduction There is now growing evidence for a crosstalk between inflammation and coagulation. In this session, Konstantin Stark report on the current evidence for a crosstalk between platelets and neutrophils. These cells are among the first responders to pathogens and perturbation of vascular integrity and the interplay triggers neutrophil extracellular trap (NET) formation. NETs have many functions including providing a scaffold for platelet adhesion and enhanced platelet activation. Cheng-Hock Toh provides an update on the pathophysiology of disseminated intravascular coagulation (DIC), a condition characterized by severe and uncontrolled activation of coagulation that may lead to multiorgan failure secondary to thrombosis in the microvasculature and in medium sized vessels and also due to consumptive coagulopathy leading to bleeding. Novel aspects of pathophysiology include mechanisms of thrombin generation, cellular dysfunction in the microcirculation and the contribution of innate immunity and inflammation with NETs formation. Finally, Riitta Lassila discusses management of coagulopathies in a selection of severe inflammatory disorders.
Patients with chronic urticaria have a higher risk of psychiatric disorders: a population‐based study
Authors: C.Y. Chu, Y.T. Cho, J.H. Jiang, C.C. Chang, S.C. Liao, C.H. Tang
Journal: British Journal of Dermatology (2019)

Background The prevalence of psychiatric comorbidities in patients with chronic urticaria (CU) in a national population is largely unknown. Objectives To investigate the prevalence of psychiatric disorders and psychiatric medication use in patients with CU in Taiwan. Methods Data were sourced from Taiwan's National Health Insurance Research Database for 2011. Patients who had a primary/secondary International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis code of 708·1, 708·8 or 708·9 during 2011 with at least two outpatient visits and an antihistamine prescription were identified as CU cases. Patients with CU were classified into three disease severity groups according to their medication types. Psychiatric disorders were identified by patients having three outpatient visits with a primary or secondary diagnosis of a given psychiatric disease. Psychiatric medication use was defined by having at least four outpatient visits with prescriptions for anxiolytics, antidepressants or sleeping pills in 2010 or 2011. Results Of the 167 132 patients with CU, 82·5% had mild CU, 17·0% had moderate CU and 0·4% had severe CU. Patients with CU had a higher prevalence of psychiatric disorders and psychiatric medication prescription than control groups. The relative risk (RR) of psychiatric disorders was 1·43 for patients with mild, 1·50 for patients with moderate and 2·32 for patients with severe CU vs. the controls (P < 0·001). For psychiatric medication prescription, the RRs were 1·95, 2·70 and 2·09, respectively, vs. controls (P < 0·001). Conclusions Patients with CU had a higher prevalence and risk of psychiatric disorders and psychiatric medication prescription than control groups. What's already known about this topic? Previous studies have shown a high prevalence of psychiatric comorbidities in patients with chronic urticaria (CU), with rates ranging from 35% to 60%. Anxiety, depression and somatoform disorders have been reported as the most prevalent mental disorders in patients with CU. What does this study add? Patients with CU had a higher prevalence of psychiatric disorders and psychiatric medication use than control groups in the general population. The relative risk (RR) of psychiatric disorders was 1·43 for those with mild CU, 1·50 for those with moderate CU and 2·32 for those with severe CU vs. controls. The RR for psychiatric medication use was 1·95 for those with mild CU, 2·70 for those with moderate CU and 2·09 for those with severe CU vs. controls. Mental health evaluations and management are important elements in CU management.
THU0657 ASSOCIATION OF DIET AND SPICES WITH TREATMENT OUTCOME IN ASIAN INDIAN PATIENTS WITH RHEUMATOID ARTHRITIS – A CROSS SECTIONAL STUDY
Authors: Harshini Alur Shivakumar, Ramya Aithala, L Jeyaseelan, Debashish Danda
Journal: Annals of the Rheumatic Diseases (2019)

<h3>Background</h3> Influence of diet on inflammation, specially foods like fish oil, spices like turmeric, capsaicin, garlic etc. are reported in published literature. However, a well-designed study on this subject amongst Asian Indian patients is lacking. <h3>Objectives</h3> To analyze whether the type and quantity of intake of various food constituents, with particular reference to Indian spices, makes an impact on the control of disease activity in patients with Rheumatoid arthritis(RA) <h3>Methods</h3> Patients diagnosed as RA by the ACR 2010 criteria and receiving standard triple drug therapy in our clinic between June 2017 and June 2018, for at least one year were enrolled. Disease activity was assessed during the routine OPD visit. They were administered a food frequency questionnaire <sup>[1]</sup> pertaining to the quality as well as quantity of food and spice intake. Analysis was done using multivariate logistic regression <h3>Results</h3> A total of 400 patients were included with 86.75% females. 67.75% patients were in disease remission, 10% had mild disease activity and 22.25% moderate to high disease activity; only 18.09% were vegetarians and the rest consumed non-vegetarian food. Median age was 47.99years(SD 10.67),median duration of illness prior to presentation to our clinic was 7years(IQR 4,10), median ESR was 37mm/hr(IQR 23,52),median CRP was5.34mg/L(IQR 2.04,12.4), and median DAS28CRP was 2.07(IQR 1.64,2.97). Patients with DAS28CRP of &lt;2.6 were compared with those &gt;3.2. Statistically higher consumption of ginger, garlic, turmeric and coriander were noted amongst patients in remission. Similar results were obtained when patients with DAS28CRP of &lt;1.4 were compared with DAS28CRP &gt;5.1. Nonsignificant numerical differences were noted for intake of food constituents like wheat, total pulse, vegetables, fruit, milk and fish <h3>Conclusion</h3> Higher consumption of Indian spices like ginger, garlic, turmeric and coriander were found to be associated with better control of disease activity and hence the inflammation, as evidenced by DAS28CRP in patients with Rheumatoid arthritis, receiving standard triple therapy <h3>References</h3> [1] Shim JS, Oh K, Kim HC. Dietary assessment methods in epidemiologic studies. Epidemiol Health. 2014 <h3>Disclosure of Interests</h3> None declared
Abstract P260: Can Metabolic Syndrome Predict the Incidence of Silent Myocardial Infarction? An Analysis From the Atherosclerosis Risk in Communities (ARIC) Study
Authors: Yong Shen, Stephanie Filipp, Matthew J Gurka, Mark D DeBoer, Thomas A Pearson
Journal: Circulation (2018)

Introduction: Diabetes patients are at risk for clinical Myocardial Infarction (MI) and have a larger proportion being silent Myocardial Infarction. However, less is known about the impact of Metabolic Syndrome (MetS, also known as prediabetes) on the incidence of silent MI. Here, we studied whether the degree of MetS severity can be predictive for future risk of silent MI. Methods: 12,527 ARIC study participants who are free of coronary heart diseases (CHD) and diabetes at baseline (1987-1989) were included for the analysis. Silent MI was determined by ECG serial changes of MI without prior clinical history of MI. A continuous MetS severity score was formulated from the integration of MetS components to assess its prediction for future silent MI and clinical MI. Results and Conclusions: 458 participants (3.7%, 458 of 12,527) developed clinical MI and 87 (0.7%, 87 of 12,527) were diagnosed with silent MI until ARIC visit 4 (1996-1998). Within the 10 years follow-up period, gender, smoking status, MetS components (waist circumference, blood pressure, HDL cholesterol) and the integrated MetS severity score were identified as significant risk factors for the incidence of both silent MI and clinical MI. Participants with MetS had a significant adjusted HR for incident silent MI (HR = 1.98, 95% CI: [1.30, 3.02], p=0.0015) as compared to clinical MI (HR = 1.67, 95% CI: [1.39, 2.00], p&lt;0.0001). The 10-year risk scoring equations of silent MI and clinical MI were constructed as a multivariate predictive tool based on MetS severity score. In conclusion, higher MetS severity score is associated with further risk of both clinical and silent MI, identifying the potential clinical application of MetS severity score in MI prevention.
Abstract 17885: A Novel Porcine Model of Acute Myocardial Ischemia Guided by FFR for Accuracy of Dynamic CT Myocardial Perfusion Imaging Study
Authors: Xiaorong Zhou, Noah Rosenthal, Sahil A Parikh, Isidore Okere, Prabhakar Rajiah, David Wilson, Hiram G Bezerra
Journal: Circulation (2013)

Purpose: Determine the degree of coronary stenosis in a porcine model of balloon-induced myocardial ischemia guided by Fractional Flow Reserve ( FFR). Optimize imaging methods and processing for my...
Abstract 203: Long-term Outcome Of Kawasaki Disease With Giant Coronary Aneurysms Which Waste Cardiac Follow-up; A Case Report
Authors: Masanori Mizuno, Masahide Chikada, Yoshimitsu Tsuzuki, Hirokuni Ono, Masaki Arima, Kenjiro Goto, Kentaro Aso
Journal: Circulation (2015)

We report 2 cases of Kawasaki disease (KD) which required surgical interventions long after initial diagnosis. Case 1: Twenty-one years-old male. He was diagnosed as KD at the age of 1. Despite he received intravenous gamma-globulin therapy, bilateral giant coronary aneurysms were formed. In addition, multiple aneurysms include abdominal, iliac and axillary arteries were formed simultaneously. Coronary angiography (CAG) was thought to be dangerous because of iliac artery aneurysm formation. He received medical follow-up with aspirin administration only. He referred to our department at the age of 13. Ischemic changes were confirmed on both treadmill exercise test and cardiac scintigraphy. CAG showed 75% right coronary artery (RCA) stenosis and 99% left main trunk stenosis. Coronary artery bypass grafting (CABG) was performed and his postoperative course has been uneventful. Case 2: Thirty-seven years-old male. He was diagnosed as KD at the age of 1. Left giant coronary aneurysms were formed and subsequently aneurysm was occluded. Aspirin was administrated, but he interrupted medication and follow-up for himself. He referred to our institution due to cardiopulmonary arrest during exercise. After successful resuscitation, CAG revealed total occlusion of RCA and 99% left coronary artery stenosis. Emergent CABG was performed and no postoperative sequel has occurred. Both cases didn’t receive sufficient assessment during the follow-up period. As a result, fatal cardiac event was occurred in case 2. Coronary aneurysm tends to change into combine aneurysmal and stenotic lesion. Long time follow up is necessary even if patients have no symptom. In addition, to decrease lost follow up patients, we should explain the risk of sudden cardiac death repeatedly and emphasis necessity of long term follow-up and medication.
PF733 COMBINED ORAL ANALGESIA AND ANXIOLYSIS FOR PAIN ASSOCIATED WITH BONE MARROW ASPIRATION AND BIOPSY
Authors: C. Cerchione, G. Martinelli, L. Catalano, N. Pugliese, R. Della Pepa, A. Gravetti, D. Nappi, F. Pane, V. Martinelli
Journal: HemaSphere (2019)

Background: Bone marrow aspiration and biopsy (BMAB) is a painful procedure, and the commonly adopted local infiltration anesthesia (LIA) with lidocaine is unable to relieve the pain during the most uncomfortable phases, or the anticipatory anxiety related to pain recalling thereafter. As there are no formal guidelines for adding a sedoanalgesic premedication before beginning the BMAB, many combinations have been adopted by several authors. Aims: Our randomized and patient blinded trial aimed to evaluate, as primary end point, the efficacy and safety of opioid and benzodiazepine agent combination plus LIA in patients who underwent BMAB for hematological malignancies. Two secondary end points were: 1) to define if patients who already underwent to BMAB without LIA prefer sedoanalgesia; 2) to demonstrate if sedoanalgesia can influence the quality of the biological specimen harvested. Methods: Patients were randomly assigned into two arms for receiving either placebo plus LIA (standard group, 48,6%) or oral fentanyl citrate 200 mcg + oral midazolam 5 mg in addition to LIA (combo‐group, 51,4%) during BMAB. Pre‐procedural anxiety and procedural pain were assessed according to the Numered Rating Scale (NRS: 0–10), dividing the time of the procedure into five intervals (T0, T1, T2a, T2b, and T3) and evaluating discomfort grade during each moment of procedure in both groups. Cognitive function was measured before and 30 minutes after the procedure. Possible side effects were recorded, as well as the adequacy of tissue samples harvested. A total number 116 patients were enrolled in the study. 9 patients did not meet inclusion criteria and were excluded. 52 patients were randomized and assigned to standard group and 55 to combo group. Results: At T2b and T3 (corresponding to the biopsy time and time after the biopsy, respectively) there was a significantly lower ( p &lt; 0.05) perception of pain in the patients who received sedoanalgesia (combo‐group) compared to those who did not (standard group). Moreover, 100 % of the patients in combo group who had previously undergone this procedure without premedication reported that they would prefer sedoanalgesia for the subsequent procedures. Summary/Conclusion: Administration of oral analgesia and anxiolysis is a safe and feasible option to be used in outpatient setting; sedoanalgesia is very effective in reducing pain during the biopsy and it diminishes the anticipatory anxiety related to a painful procedure. Patients should have the possibility to choose between local anesthesia alone or sedoanalgesia plus local anesthesia.
Defining Genetic Variation in Widely Used Congenic and Backcrossed Mouse Models Reveals Varied Regulation of Genes Important for Immune Responses
Authors: Danielle A. Chisolm, Wayne Cheng, Shelby A. Colburn, Aaron Silva-Sanchez, Selene Meza-Perez, Troy D. Randall, Amy S. Weinmann
Journal: Immunity (2019)
Abstract 19023: Left Ventricle Diastolic Dysfunction With Preserved Ejection Fraction, Who is at Higher Risk?
Authors: Arnav Kumar, Roozbeh Sharif, Adnan Khan, Mohammad Morsy, Wissam Khalife
Journal: Circulation (2013)

Background: Prevalence of left ventricular diastolic dysfunction (LVDD) in a multi ethnic population and the associated risk factors have not been well studied. To risk stratify patients with LVDD, demographics should be further analyzed in a population representative of the nation’s diversity. We aimed to investigate how race and age correlate with the presence and severity of LVDD in a multi-ethnic population. Methods: In a cross-sectional study, we reviewed the medical records of 2702 randomly selected patients who presented to the echocardiographic lab in a tertiary care academic center between 2008 and 2011. Diastolic function was evaluated based on echocardiographic characteristics. Demographic information (including self-reported race) and co-morbid clinical conditions were compared between patients with normal diastolic function and those with LVDD. Mixed races were excluded from the analysis. Using logistic regression, we conducted a multivariate analysis to examine the association of demographic variables with presence and severity of LVDD, after adjusting for co-morbid clinical conditions. Results: The study population included 2702 patients: average (SD) age of 60.3 (16.2), 1853 (68.6%) with LVDD, 1301 (48.4%) females and 848 (31.4%) African-Americans (AA). AAs constituted 213 (25.1%) of patients with normal diastolic function, compared to 660 (35.6%) patients with LVDD (OR: 1.6, 95%CI: 1.2, 2.1, p&lt;0.0001). Multivariable analysis indicated that older age (p&lt;0.0001) and AA race (p=0.0031) are independent correlates of LVDD, after adjusting for potential clinical confounders such as body mass index, high blood pressure, chronic kidney disease, coronary artery disease, diabetes mellitus, right ventricular systolic pressure and other co morbid conditions. AA patients were also found to have a higher likelihood of more severe stages of diastolic dysfunction when compared to other ethnic groups, after adjusting for age and co-morbid conditions. However, patient gender was not associated with presence or severity of LVDD. Conclusion: Older age and African-American ethnicity increases the risk and severity of LVDD, regardless of clinical co-morbid conditions.
PB1865 SINUS BRADYCARDIA AS A SIGN OF CENTRAL NERVOUS SYSTEM INVOLVEMENT IN A CHILD WITH GRISCELLI SYNDROME TYPE 2
Authors: O. Kilic, D. Ay Tuncel, B.S. Gorkem, A. Ozcan, S. Akyol, O.O. Yuregir, O. Pamukcu, E. Unal, M. Karakukcu, T. Patıroglu
Journal: HemaSphere (2019)

Background: Hemophagocytic syndrome is often fatal in Griscelli syndrome type 2, and the only cure is hematopoietic stem cell transplantation. Aims: We aimed to emphasize the importance of sinus Bradycardia as a sign of Central Nervous System Involvement in a Child with Griscelli Syndrome Type 2 Methods: A 6‐year‐old female Syrian refugee with gray hair was referred to Erciyes University for hematopoietic stem cell transplantation (HSCT) for Griscelli syndrome type 2. Her parents were consanguineous, and one of her siblings had the similar hair color and died when she was 9 months old. Blood count analyses revealed a hemoglobin of 5 gr/dl, leukocyte count of 810x10 9 /L and platelet count of 2x10 9 /L. Bone marrow aspiration and biopsy showed hemophagocytosis with normal cellularity. Sinus bradycardia was detected on the first day of her admission on ECG (50/min). Because of sinus bradycardia, a cranial magnetic resonance imaging (MRI) was done. A homozygous pathogenic c.514_518delCAAGC (p.Gln172Asnfs∗2) (pQ172Nfs∗2) variant was detected in the molecular analysis of RAB27A gene. Results: After the diagnosis of Griscelli syndrome type 2, she was treated according to the HLH 2004 protocol with additional intrathecal chemotherapy. The bradycardia recovered in three days. Six weeks after this treatment the patient showed complete improvement but her parents refused further treatments. Summary/Conclusion: Different types of arrhythmia can be seen in different types of diseases that may affect the CNS. According to best of our knowledge, this is the first reported case of a child with Griscelli syndrome type 2 who presented sinus bradycardia as a sign of CNS involvement. It should be considered that sinus bradycardia in a child with partial oculocutaneous albinism may be an important predictor for CNS involvement of GS‐2.