tbuckley/cabot-search huggingface dataset:
Ampicillin and Gentamicin in Infants With Suspected Sepsis
Authors: Scott J. Weissman, Barbara Stoll
Journal: JAMA Pediatrics (2020)

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Abstract 16380: Disease Specific Induced Pluripotent Stem Cell Derived Cardiomyocytes Represent Pathophysiological Phenotype in Hypertrophic Cardiomyopathy
Authors: Maki Takeda, Shigeru Miyagawa, Takuji Kawamura, Emiko Ito, Akima Harada, Noriko Mochizuki-oda, Yoshiki Sawa
Journal: Circulation (2020)

Although hypertrophic cardiomyopathy (HCM) is a major hereditary heart disease, unknown underlying basic pathology make it difficult for physicians to heal this disease. We hypothesized that the disease specific iPS derived cardiomyocytes from HCM with the myosin binding protein C (MYBPC3) mutation represent the pathophysiological phenotype, and editing the mutation would cancel the diseased phenotype to check the role in the mutation gene. We established iPS cells with heterozygous frameshift mutation (WT/MT-iPS) from patient and performed genome editing to generate cells with repair of mutation (WT/WT-iPS) and homozygous mutation (MT/MT-iPS) using CRISPER-Cas9. Droplet digital PCR revealed that the transcript of the mutant allele was about 30% in WT/MT-iPS derived cardiomyocytes (CMs) compared to the WT allele and also WT/WT-iPSCMs and MT/MT-iPSCMs expressed only the transcript of WT allele and MT allele, respectively. The RNA expression of MYBPC3 was restored in WT/WT-iPSCMs and decreased in MT/MT-iPSCMs assessed by quantitative real-time PCR. Sarcomeric disarray was observed in WT/MT-iPSCMs, and was more prominently expressed in MT/MT-iPSCMs assessed by immunofluorescence staining. Cell motion analyzer revealed that relaxation velocity was significantly decreased and relaxation duration was significantly increased in WT/MT-iPSCMs and MT/MT-iPSCMs compared to WT/WT-iPSCMs. Moreover significant increase in contraction end velocity was observed in MT/MT-iPSCMs compared to WT/MT and WT/WT-iPSCMs. Established disease specific iPS-CMs with MYBPC3 mutation reproduces pathophysiology in HCM, providing important clues that elucidate true nature in patient’s hereditary diseases.
Abstract 272: Differences in Out-of-hospital Cardiac Arrest Survival Between Inner-city and Suburban Areas in New York
Authors: Mohanapriya Sayeen Nagarajan, Casey Owens, Ghania Haddad-Essaihi, Timmy Li, Lance B Becker, Daniel Rolston
Journal: Circulation (2020)

Introduction: Previous studies have shown that survival from out-of-hospital cardiac arrest (OHCA) is higher in urban settings than in rural settings. However, few studies have assessed survival differences in inner-city areas compared to suburban areas. We assessed differences in survival to admission and survival to discharge between OHCA patients treated at inner-city hospitals and those treated at suburban hospitals. Method: We conducted a retrospective analysis of adult atraumatic OHCA patients presenting to 12 hospitals in 2018 and 2019 within a healthcare system in the downstate New York area. Hospitals were classified as either inner-city or suburban using the urban-rural classification system of the National Center for Health Statistics, according to hospital addresses. We modified the LACE score to create a measure of the Charlson Comorbidity Index and number of ED visits in the past 6 months (termed “CE score”) to represent comorbidities and overall health. Two multivariable logistic regression models were developed to assess the association between location (inner city vs suburban) and survival to admission and discharge, after controlling for relevant covariates. Results: A total of 3,035 patients were included in this analysis; 1,350 patients (44%) presented to inner-city hospitals and 1,685 (56%) presented to suburban hospitals. Overall median age was 75 years, 59% were male, and 63% were White. Controlling for race, age, and CE score, inner-city hospital patients had higher odds of survival to admission (adjusted OR: 1.84; 95% CI: 1.57, 2.15) and higher odds of survival to discharge (adjusted OR: 1.35; 95% CI: 1.13, 1.60) compared with suburban hospital patients. Conclusion: Patients treated at inner-city hospitals had significantly higher odds of both survival to admission and survival to discharge compared with those treated at suburban hospitals. Further studies are needed to determine the relevant factors and inform public health interventions to increase survival from OHCA in suburban areas.
Home Oxygen Therapy for Adults with Chronic Lung Disease. An Official American Thoracic Society Clinical Practice Guideline
Authors: Susan S. Jacobs, Jerry A. Krishnan, David J. Lederer, Marya Ghazipura, Tanzib Hossain, Ai-Yui M. Tan, Brian Carlin, M. Bradley Drummond, Magnus Ekström, Chris Garvey, Bridget A. Graney, Beverly Jackson, Thomas Kallstrom, Shandra L. Knight, Kathleen Lindell, Valentin Prieto-Centurion, Elisabetta A. Renzoni, Christopher J. Ryerson, Ann Schneidman, Jeffrey Swigris, Dona Upson, Anne E. Holland
Journal: American Journal of Respiratory and Critical Care Medicine (2020)

Evidence-based guidelines are needed for effective delivery of home oxygen therapy to appropriate patients with chronic obstructive pulmonary disease (COPD) and interstitial lung disease (ILD).
Structure of the platelet glycoprotein Ib receptor in complex with a novel antithrombotic agent
Authors: Jing Wang, Yongxiang Gao, Lan Chen, Yugang Guo, Siyi Hu, Liansheng Cheng, Weihua Xiao, Jiyuan Ke, Zhongliang Zhu, Liwen Niu
Journal: Blood (2020)
Novel endoscopic ultrasound-guided hepaticoduodenostomy using a forward-viewing echoendoscope for altered anatomy
Authors: Yuya Hisada, Susumu Hijioka, Akihiro Ohba, Yoshikuni Nagashio, Yuya Kanai, Takuji Okusaka, Yutaka Saito
Journal: Endoscopy (2020)

Endoscopic ultrasound-guided hepaticoduodenostomy (EUS-HDS) is used for drainage of the posterior sectoral duct (PSD) [1] [2] [3] [4] [5]. However, no studies have reported PSD drainage using a forward-viewing echoendoscope for surgically altered anatomy.
Field evaluation of a rapid antigen test (Panbio™ COVID-19 Ag Rapid Test Device) for COVID-19 diagnosis in primary healthcare centres
Authors: Eliseo Albert, Ignacio Torres, Felipe Bueno, Dixie Huntley, Estefanía Molla, Miguel Ángel Fernández-Fuentes, Mireia Martínez, Sandrine Poujois, Lorena Forqué, Arantxa Valdivia, Carlos Solano de la Asunción, Josep Ferrer, Javier Colomina, David Navarro
Journal: Clinical Microbiology and Infection (2020)
Abstract 14639: An Integrated Heart Center Model Improves Outcomes After Congenital Heart Surgery
Authors: Carlos M Mery, Andrew M Well, Ziv Beckerman, Erin Gottlieb, Byron Holt, Daniel Stromberg, Kimberly Krauklis, Laura Morton, Blake Denison, Elizabeth McCullum, Charles D Fraser
Journal: Circulation (2020)

Introduction: Contemporary experience suggests that congenital heart surgery (CHS) outcomes are optimized with an integrated, focused, and multidisciplinary program. In July 2018, our institution transitioned to an integrated Heart Center (IHC) model with new dedicated surgical, cardiac intensive care, anesthesiology, perfusion, and nursing teams. Multidisciplinary protocols and quality processes were implemented. This transition provides an unusual opportunity to evaluate the impact of an IHC model on outcomes after CHS. Methods: Patients who underwent index CHS 10/16-3/20 were included. The cohort was divided into 2 groups: historical (first 21 mo) and IHC (second 21 mo). Cases were evaluated using STAT category, mortality, and other outcomes as defined by Society of Thoracic Surgeons criteria. Descriptive statistics, univariate analyses, and multivariable regression models were used. Results: The cohort included 627 patients (historical: 230, IHC: 396, 72% increase). Numbers of STAT 4 and 5 cases increased 174% (31 to 85) and 900% (1 to 10), respectively. Neonatal cases increased 100%, infants 138%, and adults 622%. Despite an increase in complexity, perioperative mortality decreased from 3.5% to 0.5% (p=0.007) (Table). Median postoperative length of stay (LOS) increased from 5[4-10] to 7 [4-18] days (p&lt;0.001). 30-day readmission rates increased from 8.6% to 14% (p=0.048). After adjusting for patient characteristics and STAT category, perioperative mortality remained significantly lower (OR 3.4, 95% CI 1.1-5.6, p=0.004), postoperative LOS was longer (RR 6.9, 95% CI 2.6-11.1, p=0.002), and 30-day readmission was not different (p=0.7). Conclusions: Implementation of an IHC model improves perioperative survival after CHS, coupled with an increase in volumes and complexity. CHS care should be delivered as part of a dedicated and multidisciplinary IHC model.
Changing spectrum of suspected drugs of epidermal necrolysis: A World Health Organization pharmacovigilance database analysis from 1997-2020
Authors: Thomas Bettuzzi, Saskia Ingen-Housz-Oro, Carmen Chinchilla Purtillo, Laurence Le Cleach, Patrick Maison, Nicolas de Prost, Pierre Wolkenstein, Bénédicte Lebrun-Vignes, Emilie Sbidian
Journal: Journal of the American Academy of Dermatology (2020)
Increased Incidence of Philadelphia Negative B-Cell Acute Lymphoblastic Leukemia Among Adults of Hispanic Ethnicity: A Population Based Study
Authors: Ashley M. Perry, Evan C. Chen, Marla M. Jalbut, Lillian Werner, Gabriela S. Hobbs, Philip C. Amrein, Amir T. Fathi, Andrew M. Brunner
Journal: Blood (2017)
Abstract 13715: Pathophysiological Role of Dexmedetomidine for Monocrotaline-induced Pulmonary Arterial Hypertension in Rats
Authors: Yohei Yamaguchi, Susumu Hosokawa, Yusuke Kajikawa, Yasuhiro Maejima, Mitsuaki Isobe, Tetsushi Furukawa, Shozaburo Doi
Journal: Circulation (2020)

Introduction: The abnormal proliferation of pulmonary arterial smooth muscle cells (PASMCs) is one of pathological characteristics of pulmonary arterial hypertension (PAH). Inflammation is thought to play an important role for PASMCs proliferation in PAH. Dexmedetomidine (DEX) is a selective α2-adrenergic receptor agonist that is used as a sedative in clinical settings. It has been reported that DEX regulates certain inflammatory responses. Hypothesis: DEX ameliorates Monocrotaline (MCT)-induced PAH in rats through its anti-inflammatory effect. Methods: In vivo, 6-week-old male Sprague-Dawley rats were injected 60 mg/kg of MCT subcutaneously. At day 14 after MCT injection, continuous infusions of DEX (2μg/kg/hour) by osmotic pumps were started in one group (MCT + DEX group), and not in another group (MCT group). We performed cardiac catheterization to measure right ventricular systolic pressure (RVSP) and prepared rats paraffin embedded lung tissues for Immunohistochemistry at day 23. In vitro, we analyzed the effect of DEX on human PASMCs (hPASMCs) proliferation stimulated with fibroblast growth factor 2 (FGF2). In addition, we examined mRNA levels of proinflammatory cytokines in hPASMCs by FGF2 stimuation with/without DEX. Results: Both RVSP and survival rate markedly improved in MCT + DEX group compared with those in MCT group (RVSP; 34mmHg ± 4mmHg vs. 70mmHg ± 10mmHg, survival rate 42% vs. 0% at day 29). In histological analysis, medial hypertrophy of pulmonary arterioles and phosphorylated-p65 positive PASMCs significantly reduced in MCT + DEX group. DEX also inhibited hPASMCs proliferation dose dependently. Furthermore, IL-6 mRNA expression was suppressed by DEX in hPASMCs stimulated with FGF2. Conclusions: DEX improved MCT-induced PAH in rats by suppressing PASMCs proliferation through the anti-inflammatory effect, which may result from inhibition of NF-κB activation induced by IL-6. DEX can be a new therapeutic tool for PAH.
Abstract 13599: A Truncated Mutation in Filamin C Reveals Non-myocyte Contributions to Hypertrophic Cardiomyopathy in Ipsc-derived Cardiac Fibroblasts
Authors: Mariana A Argenziano, jiajia yang, Mariana Burgos Angulo, Alexander Bertalovitz, Thomas V McDonald
Journal: Circulation (2020)

Introduction: Hypertrophic cardiomyopathy (HCM) is a cardiac genetic disorder that affects 1:500 individuals and represents one of the main causes of sudden cardiac death in young adults. Several mutations have been associated with HCM, including MYH7 and FLNC . The discovery of induced pluripotent stem cells (IPSC), has provided a unique patient- and disease-specific model to study cardiac disease. Although iPSC derived cardiomyocytes (IPSC-CMs) can provide a genetically diverse human model to study mechanisms of disease, the heart relies in a three-dimensional network of cells that actively participate in the development of hypertrophy. Hypothesis: We hypothesize that mutations causing HCM affect not only CMs, but other non-myocyte cell types such as cardiac fibroblasts contributing to the phenotype of the disease. Methods: IPSCs were reprogrammed from an HCM patient harboring a pathogenic mutation on FLNC (p. Ile616Aspfs*4) using Sendai virus. Cells were differentiated to cardiomyocytes (IPSC-CMs) and cardiac fibroblasts (IPSC-CFs) and cultured for 30 days before analysis. Gene expression, morphology and wound healing (WH) were analyzed in IPSC-CMs and IPSC-CFs. Results: We observed that IPSC-CFs show higher expression of FLNC than IPSC-CMs (CT CM: 1.04±0.19 vs CT CF: 4.05±0.97, **p&lt;0.01). Besides, FLNC mutated (MT) IPSC-CFs showed lower expression of FLNC when compared to controls (CT), with no changes in expression in IPSC-CMs. Expression of the fibrotic markers POSTN , SMA and THY1 was higher in IPSC-CFs from MT. Immunostaining revealed changes in morphology in IPSC-CFs of MT with accumulation of FLNC in the nucleus. Treatment with TGF-b showed upregulation of CCL2 , SMA , POSTN , COL1A1 and FLNC in MT IPSC-CFs. Wound healing assay revealed no changes in cell migration between CT and MT, although TGF-b treatment increased WH % in MT (MT: 22.5±1.5% vs MT TGF: 59.4±6.5% at 8hs, **p&lt;0.01). Conclusions: Based on our results, we conclude that CFs play an important role on the development of HCM; with higher changes in gene expression, cell migration and morphology than those observed in CMs. Further functional studies will provide evidence of the effects of these changes on the phenotype of the disease.
Abstract 14266: Management of Spontaneous Coronary Artery Dissection: Trends Over Time
Authors: Elliot Fitzsimmons, Elizabeth Thompson, Robert L Wilensky, Clare McCarthy, Jennifer Lewey
Journal: Circulation (2020)

Introduction: Spontaneous coronary artery dissection (SCAD) is a non-atherosclerotic cause of ACS that preferentially impacts young women. Increasing evidence suggests that patients with SCAD do well with a conservative approach, as intramural hematoma frequently resolves with medical management and complications from PCI are common. Our objective is to describe changes in management of SCAD over time at the University of Pennsylvania Health System (UPHS). Methods: We conducted a retrospective cohort study among patients treated within UPHS from 2005-2019. We identified all patients in the electronic health record with SCAD using ICD-9 (414.12) and ICD-10 (I25.42) codes. Charts were manually reviewed and indexed by date of SCAD event. Time categories were calculated by tertiles of patients. Since the first tertile spanned 21 years, this category was subdivided. The most recent time period represents practice after the AHA Scientific Statement on SCAD was published. Cochran-Armitage trend tests compared trends over time. Results: We identified 140 patients with SCAD. In this cohort, 43% of patients treated from 1993-2005 were managed conservatively, compared to 89% of patients treated in 2018-2019, while 57% of patients treated from 1993-2005 were managed invasively, compared to 11% of patients treated in 2018-2019, p&lt;0.001 for trend over time (Figure). Between 1993-2005 and 2018-2019, the use of DAPT increased from 40% to 80% (p&lt;0.001 for trend) and use of statins increased from 60% to 85% (p=0.021 for trend). Beta blocker use remained stable (80% vs. 89%, p=0.401) and ACEi/ARB use varied considerably without a clear trend over time (p=0.553). Conclusions: The proportion of patients with SCAD managed conservatively has steadily increased since 2015. This trend corresponds to the increased understanding of clinical outcomes of this patient population. Medical treatment has changed over time and most patients are treated with DAPT, statins, and beta blockers.
Abstract 15142: Epigenetic Biomarkers for Assessment of Post-Stroke Recovery
Authors: Shaoling Huang, Wei Guo, Xing Yin, Manoj Pandurang Dandekar, Tao Peng, Melvin E Klegerman, Melanie R Moody, Hyunggun Kim, Jaroslaw A Aronowski, David D McPherson, Keith Booher
Journal: Circulation (2019)

Background: Epigenetic markers represent a useful and reliable biomarker of pathogenesis of diseases. Stroke is a multifactorial disease causing aberrant DNA methylation profiles. We have found tha...
Abstract 13344: Do Heart Failure Patients’ Personal Priorities Influence Their Self-care Maintenance Behaviors?
Authors: Foster Osei Baah, Barbara J Riegel
Journal: Circulation (2020)

Introduction: The personal priorities of community dwelling patients with HF may influence their self-care behavior. However, clinicians are unsure of the personal priorities that influence self-care choices in patients with HF. Objective: To explore personal priorities ranked highest among HF patients with poor or excellent self-care behaviors. Methods: We conducted a cross sectional convergent mixed methods study of community dwelling patients with HF in the US, excluding those with significant cognitive impairment (MoCA score ≤22). All completed the Self-Care of HF Index v7.2 (scores standardized 0-100). Extreme case sampling was used to select people with poor self-care maintenance scores (≤60) or excellent self-care maintenance scores (≥80). In interviews, participants placed their personal and healthcare needs into the same imaginary bucket and then rank-order them from highest to lowest priority. Interview data was audio-recorded, transcribed verbatim and analysed using qualitative inductive analysis. Results: We enrolled 33 patients with HF (aged 42-82 years, 57% male, 45.5% black, 45.5% married, 54.6% unemployed). Sixteen patients were poor in self-care maintenance and 17 were excellent. Two themes representing HF patients’ personal priorities emerged: a) Healthcare (60.6%, N=20), and b) Family and Housing (36.4%, N=12). Patients who chose healthcare identified medications, insurance and living a longer life as important. As stated by one patient, “without your health you have nothing.” Patients who chose family and housing identified; 1) rent, mortgage, or real estate taxes and 2) food for their children and a roof over their head as most important. They explained by saying “ I have to have some place to live and the utilities ” and “ I can be in a card box, but I just wanna make sure they are fed .” Of those who chose healthcare as their priority, 12 (60%) had excellent self-care maintenance. Only 4 patients (33.3%) who chose family and housing had excellent self-care. Conclusion: HF patients who identify healthcare needs as their highest priority may be better at self-care maintenance. Identifying the priorities of patients with HF will help clinicians tailor discharge instructions.
Abstract 14979: Endostatin as a Predictor of Severity and Survival in Pediatric Congenital Heart Disease Associated Pulmonary Hypertension
Authors: Caroline Daly, Megan Griffiths, Jun Yang, Melanie Nies, Rachel L Damico, Dunbar D IVY, Eric D Austin, Michael Pauciulo, Bill Nichols, Allen D Everett
Journal: Circulation (2020)

Introduction: Pediatric pulmonary arterial hypertension (PAH) is a multifactorial pulmonary vascular disease with high mortality. Endostatin (ES), an angiogenic inhibitor, is associated with disease severity and mortality in adults with PAH and poor lung growth in children. Hypothesis: Increased ES is associated with worse disease severity and outcomes in pediatric PAH. Methods: Serum ES levels were measured in two cohorts of pediatric PAH (IPAH, FPAH and APAH) patients; the cross-sectional CCHMC PAH Biobank (PAHB, N=175) and a longitudinal cohort from Children’s Hospital of Colorado (CHC, N=61). Outcomes included medical therapy, functional and hemodynamic measures, and survival (death, transplant, palliative shunt). Adjusted logistic and linear regressions and Kaplan-Meier analysis were used to assess the relationship between ES and clinical outcomes. Results: In both cohorts, ES was significantly higher in PAH associated with congenital heart disease (APAH-CHD, PAHB n=70, p=0.002; CHC n=29, p=0.007) and was highest in those with a ventricular shunt (n=24, p=0.001). In APAH-CHD, ES was associated with a decreased 6MWD (-108m, -187- -30, p=0.01) and increased mean right atrial pressure (mRAP 1.8mmHg, 0.3-3.3, p=0.02). Longitudinally, in the APAH-CHD subgroup when adjusted for age, sex, and multiple time points, higher ES was associated with increased PVRi and mPAP (PVRi 2.5WU*m 2 , 0.7-4.3, p=0.007; mPAP 10mmHg, 4.5-15, p&lt;0.001). Kaplan-Meier analysis (Figure 1) demonstrated that ES was associated with worse outcomes in both cohorts, with an adjusted Cox proportional hazard ratio of 4.3 (1.13-16, p=0.03) in the PAHB. Conclusions: ES was associated with worse functional measures, pulmonary vascular hemodynamics, and survival in pediatric APAH-CHD. These observations suggest that serum ES could act as a pulmonary vascular specific biomarker to identify those who are at increased risk of developing PAH and predict poor outcomes in APAH-CHD.