tbuckley/cabot-search huggingface dataset:
Motivational Impairments in Autism May Be Broader Than Previously Thought
Authors: Gabriel S. Dichter
Journal: JAMA Psychiatry (2018)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Psychiatry HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Psychiatry journal
THU0614 Interstitial lung disease in patients with antisynthetase syndrome and anti-ro52 antibodies positive
Authors: C. Aguilera Cros, A. Ruiz Román, L. Méndez Díaz, G. Morote Ibarrola, N. Garrido Puñal, M. Arcila Durán, M.A. Montes Cano, J.A. Rodriguez Portal
Journal: Annals of the Rheumatic Diseases (2018)

One of our patients reported that her psoriatic lesions got worse on anakinra.Forty one patients reported no adverse events during the treatment.Conclusions: Anakinra was effective in controlling the symptoms in colchicineresistant FMF cases.It was also effective in FMF related amyloidosis.The major cause of treatment termination was injection site reactions.Anakinra seems to be an effective alternative in patients who have insufficient response to colchicine.
Flaviviruses Exploit the Lipid Droplet Protein AUP1 to Trigger Lipophagy and Drive Virus Production
Authors: Jingshu Zhang, Yun Lan, Ming Yuan Li, Mart Matthias Lamers, Maxime Fusade-Boyer, Elizabeth Klemm, Christoph Thiele, Joseph Ashour, Sumana Sanyal
Journal: Cell Host & Microbe (2018)
Buried lumen-apposing metal stent (LAMS) following endoscopic ultrasound-guided gallbladder drainage: the LAMS-in-LAMS rescue treatment
Authors: Dario Ligresti, Fabio Cipolletta, Michele Amata, Marta Cimavilla, Mario Traina, Ilaria Tarantino
Journal: Endoscopy (2018)

A 70-year-old woman with jaundice who had been diagnosed with unresectable malignant distal biliary stricture underwent endoscopic ultrasound (EUS)-guided gallbladder drainage following failed endoscopic retrograde cholangiopancreatography (ERCP). EUS-guided bile duct drainage was impractical because of the presence of an intervening vessel, and an EUS-guided cholecystogastrostomy was created with placement of an 8 × 8-mm lumen-apposing metal stent (LAMS) [1].
AB1004 Osteoporosis screening in a tertiary rheumatoid arthritis clinic. who’s screening now?
Authors: L. Kyi, T. Kovitwanichkanont, D. Wang, N. Walpola, S. Raghunath, S. Pignataro, S. Morton, M. Leech
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Osteoporosis is a complication of rheumatoid arthritis (RA) due to inflammatory disease and treatment with glucocorticoids<sup>1</sup>. Screening and management of osteoporosis (OP) is shared amongst General Practitioners (GP) together with subspecialists including rheumatologists<sup>2</sup>. <h3>Objectives</h3> To assess the adequacy of osteoporosis screening in a tertiary RA clinic in Melbourne, and to determine where most screening is occurring. <h3>Methods</h3> A cross-sectional study of patients at a tertiary RA clinic was undertaken. Osteoporosis screening, therapy and related factors were evaluated. This was compared to best practice screening ACR/GIOP guidelines. <h3>Results</h3> 116 RA patients, 66% female (median age 58 years) were Included. OP screening occurred in 61.2% of patients with 40.5% and 20.7% performed by their rheumatologist and by the GPs respectively. The remainder 38.8% of patients recalled no recent screening. 36.2% of patients were taking prednisolone, while 74% reported prior exposure. 58.6% of patients had prednisolone for over 3 months. Calcium or vitamin D supplementation was noted in 62% of the population. 21.6% reported a history of minimal trauma fracture and alarmingly only 10% reported currently taking anti-resorptive therapy. 47% of patients had a DEXA scan performed within the last 3 years. Of the 53% that did not have a recent DEXA scan, three quarters had indications for osteoporosis screening based on the 2010 ACR/GIOP guidelines. 35 patients had indications based on age, 11 patients based on glucocorticoid exposure and 1 patient based on history of minimal trauma fracture. <h3>Conclusions</h3> One third of at-risk patients in this sub-cohort are not screened for osteoporosis. More patients are currently screened for osteoporosis by their rheumatologist than their GPs. Underscreening and treatment of osteoporosis in this clinic could be addressed by clearer GP-rheumatologist shared treatment model. <h3>References</h3> [1] Lane NE, Lukert B. The science and therapy of glucocorticoid induced bone loss. Endocrinol Metab Clin North Am1998;27: 465–83. [2] The Royal Australian College of General Practitioners. Clinical guideline for the prevention and treatment of osteoporosis in postmenopausal women and older men. 1st edn. South Melbourne, Vic: RACGP, 2010. <h3>Disclosure of Interest</h3> None declared
A recessive form of hyper-IgE syndrome by disruption of ZNF341-dependent STAT3 transcription and activity
Authors: Vivien Béziat, Juan Li, Jian-Xin Lin, Cindy S. Ma, Peng Li, Aziz Bousfiha, Isabelle Pellier, Samaneh Zoghi, Safa Baris, Sevgi Keles, Paul Gray, Ning Du, Yi Wang, Yoann Zerbib, Romain Lévy, Thibaut Leclercq, Frédégonde About, Ai Ing Lim, Geetha Rao, Kathryn Payne, Simon J. Pelham, Danielle T. Avery, Elissa K. Deenick, Bethany Pillay, Janet Chou, Romain Guery, Aziz Belkadi, Antoine Guérin, Mélanie Migaud, Vimel Rattina, Fatima Ailal, Ibtihal Benhsaien, Matthieu Bouaziz, Tanwir Habib, Damien Chaussabel, Nico Marr, Jamel El-Benna, Bodo Grimbacher, Orli Wargon, Jacinta Bustamante, Bertrand Boisson, Ingrid Müller-Fleckenstein, Bernhard Fleckenstein, Marie-Olivia Chandesris, Matthias Titeux, Sylvie Fraitag, Marie-Alexandra Alyanakian, Marianne Leruez-Ville, Capucine Picard, Isabelle Meyts, James P. Di Santo, Alain Hovnanian, Ayper Somer, Ahmet Ozen, Nima Rezaei, Talal A. Chatila, Laurent Abel, Warren J. Leonard, Stuart G. Tangye, Anne Puel, Jean-Laurent Casanova
Journal: Science Immunology (2018)

Heterozygosity for human signal transducer and activator of transcription 3 (STAT3) dominant-negative (DN) mutations underlies an autosomal dominant form of hyper-immunoglobulin E syndrome (HIES). We describe patients with an autosomal recessive form of HIES due to loss-of-function mutations of a previously uncharacterized gene, ZNF341 ZNF341 is a transcription factor that resides in the nucleus, where it binds a specific DNA motif present in various genes, including the STAT3 promoter. The patients' cells have low basal levels of STAT3 mRNA and protein. The autoinduction of STAT3 production, activation, and function by STAT3-activating cytokines is strongly impaired. Like patients with STAT3 DN mutations, ZNF341-deficient patients lack T helper 17 (TH17) cells, have an excess of TH2 cells, and have low memory B cells due to the tight dependence of STAT3 activity on ZNF341 in lymphocytes. Their milder extra-hematopoietic manifestations and stronger inflammatory responses reflect the lower ZNF341 dependence of STAT3 activity in other cell types. Human ZNF341 is essential for the STAT3 transcription-dependent autoinduction and sustained activity of STAT3.
The Association of Prenatal Ultrasonography and Autism Spectrum Disorder—Reply
Authors: N. Paul Rosman, Jodi Abbott, Rachel Vassar
Journal: JAMA Pediatrics (2018)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA Pediatrics HomeNew OnlineCurrent IssueFor Authors Podcast Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA Pediatrics journal
CCR2-dependent monocytes/macrophages exacerbate acute brain injury but promote functional recovery after ischemic stroke in mice
Authors: Weirong Fang, Xuan Zhai, Dong Han, Xiaoxing Xiong, Tao Wang, Xun Zeng, Shucheng He, Rui Liu, Masaaki Miyata, Baohui Xu, Heng Zhao
Journal: Theranostics (2018)

Rationale: Peripheral blood monocytes are recruited into the ischemic brain and transform into macrophages after stroke.Nevertheless, the exact role of CCR2-dependent monocytes/ macrophages in brain injury after stroke remains elusive.Methods: We used CCR2 knockout (KO) mice and the CCR2 pharmacological inhibitor, propagermanium (PG), to address the role of CCR2-dependent monocytes/macrophages in the acute stage and neurological functional recovery after middle cerebral artery (MCA) occlusion and reperfusion.Results: CCR2 KO resulted in smaller infarct size and lower mortality than in wild type (WT) mice, when measured 3 days after stroke.However, from 5 to 28 days after stroke, the KO mice had higher mortality and showed no obvious neurological functional recovery.In addition, WT mice treated with PG had similar stroke outcomes compared with CCR2 KO, as measured by T2 weighted MRI.Flow cytometry and real-time PCR analyses suggest that monocyte-derived macrophages (MoDMs) in the stroke brains mainly polarized to pro-inflammatory macrophages at the early stage, but gradually switched to anti-inflammatory macrophages at 7 days after stroke.In addition, adoptive transfer of anti-inflammatory macrophages into CCR2 KO mice at 4 and 6 days after stroke alleviated mortality and promoted neurological recovery.Conclusion: CCR2-dependent monocytes/macrophages are a double-edged sword; they worsen acute brain injury, but are essential for neurological recovery by promoting anti-inflammatory macrophage polarization.
Hypoxia-stressed cardiomyocytes promote early cardiac differentiation of cardiac stem cells through HIF-1α/Jagged1/Notch1 signaling
Authors: Keke Wang, Ranran Ding, Yanping Ha, Yanan Jia, Xiaomin Liao, Sisi Wang, Rujia Li, Zhihua Shen, Hui Xiong, Junli Guo, Wei Jie
Journal: Acta Pharmaceutica Sinica B (2018)

Hypoxia is beneficial for the differentiation of stem cells transplanted for myocardial injury, but mechanisms underlying this benefit remain unsolved. Here, we report the impact of hypoxia-induced Jagged1 expression in cardiomyocytes (CMs) for driving the differentiation of cardiac stem cells (CSCs). Forced hypoxia-inducible factor 1α (HIF-1α) expression and physical hypoxia (5% O2) treatment could induce Jagged1 expression in neonatal rat CMs. Pharmacological inhibition of HIF-1α by YC-1 attenuated hypoxia-promoted Jagged1 expression in CMs. An ERK inhibitor (PD98059), but not inhibitors of JNK (SP600125), Notch (DAPT), NF-κB (PTDC), JAK (AG490), or STAT3 (Stattic) suppressed hypoxia-induced Jagged1 protein expression in CMs. c-Kit+ CSCs isolated from neonatal rat hearts using a magnetic-activated cell sorting method expressed GATA4, SM22α or vWF, but not Nkx2.5 and cTnI. Moreover, 87.3% of freshly isolated CSCs displayed Notch1 receptor expression. Direct co-culture of CMs with BrdU-labeled CSCs enhanced CSCs differentiation, as evidenced by an increased number of BrdU+/Nkx2.5+ cells, while intermittent hypoxia for 21 days promoted co-culture-triggered differentiation of CSCs into CM-like cells. Notably, YC-1 and DAPT attenuated hypoxia-induced differentiation. Our results suggest that hypoxia induces Jagged1 expression in CMs primarily through ERK signaling, and facilitates early cardiac lineage differentiation of CSCs in CM/CSC co-cultures via HIF-1α/Jagged1/Notch signaling.
FRI0043 Trends in all-cause and cardiovascular mortality in patients with rheumatoid arthritis. a 10- to 20-year follow-up study in 3 consecutive incidence cohorts
Authors: S.A. Provan, S. Lillegraven, E.A. Haavardsholm, T.K. Kvien, J. Sexton, T. Uhlig
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The mortality rate in patients with rheumatoid arthritis (RA) has been estimated to be 1.5–1.6 compared to the general public. Moreover, RA patients are still not benefitting fully from the declining mortality rate that the general population has enjoyed and cardiovascular disease (CVD) continues to be a major concern. <h3>Objectives</h3> To examine the all-cause and CVD mortality in 3 consecutive cohorts of patients with incident RA, compared to population controls <h3>Methods</h3> <b>The Oslo RA register (ORAR)</b> was established in 1994. The inclusion criteria were a diagnosis of RA according to the 1987 ACR criteria and residency in Oslo. The register was updated annually until 2009, and validated for completeness.<sup>1</sup> In January 2012, 3328 patients were registered in ORAR. We grouped patients into 3 successive incidence cohorts; 1996(1994–1998), 2001 (1999–2003) and 2006(2004–2008). For each patient we identified 5 historical population controls matched according to age at disease incidence, gender and postal code of residence. Patients and controls were linked to the Norwegian Cause of Death Registry. Counts were compared using chi<sup>2</sup> tests. The hazard ratio (HR) for survival was calculated using stratified cox-regression models adjusted for highest achieved level of education. <h3>Results</h3> 422, 477 and 459 patients in the 1996, 2001 and 2006 cohorts were matched to 2110, 2385 and 2295 controls respectively. 20, 15 and 10 years follow-up for 1996, 2001 and 2006 cohorts were available in 201, 292 and 311 patients respectively. Results are presented in the table 1. <h3>Conclusions</h3> RA patients with disease incidence in the period 1994–2003 had increased all-cause and CVD related mortality after 10–15 years of disease duration, compared to the population of Oslo. The increased mortality was not observed in the 2004–2008 cohort. Improved survival in recent years may be related to more effective therapies and earlier diagnosis, but further analyses are warranted. <h3>Reference</h3> [1] Kvien, Uhlig. J Rheumatol2004. <h3>Disclosure of Interest</h3> None declared
GMC in the spotlight
Authors: Fiona Godlee
Journal: BMJ (2018)

The GMC is under fire. An ongoing inquest into the death of a trainee anaesthetist has heard allegations of failings in the GMC’s handling of his case, involving drug misuse. And a major review, which concluded this week, has exposed the damaging extent of fear and mistrust with which doctors view their regulator.

Clare Dyer reports on the tragic story of Julien Warshafsky (doi:10.1136/bmj.k2564), in which the GMC failed to order sufficient tests to monitor his fentanyl use and appointed to supervise him a psychiatrist who was not a specialist in …
Doctors who miss tribunals risk being struck off, warns judge
Authors: Clare Dyer
Journal: BMJ (2018)

The General Medical Council (GMC) should warn doctors facing regulatory hearings that they are “courting removal” from the medical register if they do not turn up at the tribunal, a High Court judge has urged.1

Mr Justice Kerr made the call in a judgment dismissing Abayomi Sanusi’s appeal from a decision by a medical practitioners tribunal to strike him off the register.

In November 2017 the tribunal found that Sanusi, a surgical registrar, who was not represented and did not appear at the hearing but put in written submissions, had failed to comply with junior doctors’ requests to examine three deteriorating patients.2 It also concluded that he had lied in a job interview.

Kerr said, “This was another case in which a professional, facing serious disciplinary charges including a charge …
SP0141 Improving the study of bone diseases by correlative electron-, ion- and x-ray microscopy including their analytical techniques
Authors: S.H. Christiansen
Journal: Annals of the Rheumatic Diseases (2018)

<h3></h3> In the last decades al lot of knowledge was gathered on the structure and composition of bone. Cutting-edge correlative high-resolution microscopy and spectroscopy permits reaching the next level of understanding: Correlative workflows starting from X-ray microscopy volume analysis with voxel sizes of&lt;1 µm, over large scale scanning electron data acquisition, to dual beam microscope analysis (focused electron- and ion beam) permit the scale bridging investigation of bone architectures and thus merging the “big picture” and the underlying ultrastructure with statistical significance. In combination with additional analytical add-ons, physical properties such as optical, mechanical, compositional, structural etc. deliver a highly detailed correlative dataset of bone. The present paper utilises the comprehensive data acquisition to study bone degradation as it occurs in diseases such as osteoporosis and inflammatory osteoarthritis in mouse models. <h3>Disclosure of Interest</h3> None declared
Insurance correlates with improved access to care and outcome among glioblastoma patients
Authors: Desmond A Brown, Benjamin T Himes, Panagiotis Kerezoudis, Yirengah M Chilinda-Salter, Sanjeet S Grewal, Joshua A Spear, Mohamad Bydon, Terry C Burns, Ian F Parney
Journal: Neuro-Oncology (2018)

The current standard of care for glioblastoma (GBM) constitutes maximal safe surgical resection, followed by fractionated radiation and temozolomide. This treatment regimen is logistically burdensome, and in a health care system in which access to care is variable, there may be patients with worsened outcomes due to inadequate access to optimal treatment. The National Cancer Database was queried for patients with diagnoses of GBM in 2006–2014. Patients were grouped according to insurance status: private insurance, Medicare, Medicaid, or uninsured. Treatments provided (surgery, radiation, and chemotherapy) were compared between groups in univariate and multivariable logistic regression analysis. A total of 61614 patients were analyzed. Compared with private insurance, the odds of surgery for Medicaid and uninsured patients were 0.72 (95% CI: 0.66–0.79) and 0.77 (95% CI: 0.69–0.87), respectively (P < 0.001). The multivariable odds of receiving radiotherapy were 0.91 (95% CI: 0.86–0.96), 0.62 (95% CI: 0.57–0.68), and 0.47 (95% CI: 0.43–0.52) for Medicare, Medicaid, and uninsured patients, respectively (all P < 0.001). In addition, the odds of receiving chemotherapy were 0.94 (95% CI: 0.89–0.99), 0.53 (95% CI: 0.49–0.57), and 0.41 (95% CI: 0.38–0.46) for Medicare, Medicaid, and uninsured patients, respectively (all P < 0.001). Insurance status and type of insurance coverage appear to impact treatments rendered for GBM, independently of other variables. Furthermore, we find that such differential access to care significantly impacts survival. Ensuring adequate access to care for all patients with diagnoses of glioblastoma is critical to optimize survival, especially as therapies continue to advance.
Weekend Effect in Emergency Colon and Rectal Cancer Surgery: A Prospective Study Using Data From the Dutch ColoRectal Audit
Authors: Daniëlle D. Huijts, Julia T. van Groningen, Onno R. Guicherit, Jan Willem T. Dekker, Leti van Bodegom-Vos, Esther Bastiaannet, Johannes A. Govaert, Michel W. Wouters, Perla J. Marang-van de Mheen
Journal: Journal of the National Comprehensive Cancer Network (2018)

Background: It is unclear whether emergency weekend colon and rectal cancer surgery are associated with worse outcomes (ie, weekend effect) because previous studies mostly used administrative data, which may insufficiently adjust for case-mix. Materials and Methods: Prospectively collected data from the 2012–2015 Dutch ColoRectal Audit (n=5,224) was used to examine differences in 30-day mortality and severe complication and failure-to-rescue rates for emergency weekend (Saturday and Sunday) versus Monday surgery, stratified for colon and rectal cancer. Analyses were adjusted for age, sex, body mass index, Charlson comorbidity index, American Society of Anesthesiologists classification score, tumor stage, presence of metastasis, preoperative complication, additional resection for metastasis or locally advanced tumor, location primary colon tumor, type of rectal surgery (lower anterior resection or abdominal perineal resection), and type of neoadjuvant therapy (short-course radiotherapy or chemoradiotherapy). Results: A total of 5,052 patients undergoing colon cancer surgery and 172 undergoing rectal cancer surgery were included. Patients undergoing colon or rectal cancer surgery during weekends had significantly more preoperative tumor complications compared with those undergoing surgery on a weekday. Additionally, differences in year of surgery and location of primary tumor were found for colon cancer surgery. Emergency colon cancer surgery during the weekend was associated with increased 30-day mortality (odds ratio [OR], 1.66; 95% CI, 1.10–2.50) and severe complications (OR, 1.29; 95% CI, 1.03–1.63) compared with surgery on Monday. Estimates for emergency weekend rectal cancer surgery were similar but not statistically significant, likely explained by small numbers. Conclusions: Weekend emergency colon cancer surgery was associated with higher mortality and severe complication rates. More research is needed to understand which factors explain and contribute to these differences.
Comprehensive Safety Analysis of Venetoclax Monotherapy for Patients with Relapsed/Refractory Chronic Lymphocytic Leukemia
Authors: Matthew S. Davids, Michael Hallek, William Wierda, Andrew W. Roberts, Stephan Stilgenbauer, Jeffrey A. Jones, John F. Gerecitano, Su Young Kim, Jalaja Potluri, Todd Busman, Andrea Best, Maria E. Verdugo, Elisa Cerri, Monali Desai, Peter Hillmen, John F. Seymour
Journal: Clinical Cancer Research (2018)

The oral BCL-2 inhibitor venetoclax is an effective therapy for patients with relapsed/refractory (R/R) chronic lymphocytic leukemia (CLL), including disease with high-risk genomic features such as chromosome 17p deletion [del(17p)] or progressive disease following B-cell receptor pathway inhibitors.
Sixty seconds on . . . Hurricane Maria
Authors: Ingrid Torjesen
Journal: BMJ (2018)

Last year’s Hurricane Maria was the worst natural disaster to ever affect Puerto Rico. The tropical cyclone made landfall on 20 September 2017, compounding the destruction caused by Hurricane Irma two weeks earlier.

An estimated $90bn (£67bn; €77bn), making it the third costliest tropical cyclone in the US and its territories since 1900. It damaged roads …