tbuckley/cabot-search huggingface dataset:
AB1136 Clinical features of adult chinese patients with periodic fever, aphthous stomatitis, pharyngitis, and adenitis: report from an single centre
Authors: D. Wu, M. Shen
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome is a polygenic or multifactorial autoinflammatory disease (AUIDs) with unknown etiology. It mainly affects children, though recently it has been also recognised in adulthood. PFAPA syndrome has not been reported in the Chinese population up till now. <h3>Objectives</h3> We aimed to investigate the clinical features, treatment and prognosis of the first cohort of adult Chinese patients with PFAPA syndrome. <h3>Methods</h3> We prospectively evaluated clinical data of adult patients (≥14 years) with recurrent fever of unknown origin who were suspected monogenic AUIDs in the period April 2015 to December 2017, at the adult AUIDs centre, Department of Rheumatology, Peking Union Medical College Hospital. Gene tests of AUIDs associated pathogenic genes were examined to exclude monogenic AUIDs. The clinical features were evaluated for patients with PFAPA syndrome, and were compared with a newly reported large Italian cohort of adult patients with PFAPA syndrome.<sup>1</sup> <h3>Results</h3> During the study period, a total of 7adult patients with PFAPA syndrome were diagnosed (table 1), and the male to female ratio was 6:1. All these patients had a disease onset in adulthood, and the mean age of onset was 27±10 years, and the mean time of delay of diagnosis was 4.9±4.0 years. Clockwork periodicity fever was the main feature of PFAPA syndrome, and the median duration of attacks was3.5 days, and the median interval between attacks was 4 weeks. Pharyngitis, adenitis and aphthous stomatitis were present in 85.7%, 57.1% and 42.9%, respectively. Other common symptoms included fatigue (100%), headache (71.4%), and myalgia (71.4%). The presence of arthralgia/arthritis was less frequent compared to the Italian cohort (p=0.012) (table 2). Corticosteroids given during attacks were effective in 3 patients. NSAIDS were partially effective but tonsillectomy was of no effect. Colchicine was rarely used compared to the Italian cohort (p=0.046). <h3>Conclusions</h3> PFAPA syndrome is one of the main causes of adults Chinese patients with recurrent fever of unknown origin and might be misdiagnosed. Clinicians, not only paediatricians, should take into account this clinical entity, especially for patients with pharyngitis, cervical adenopathy and aphthous stomatitis. <h3>Reference</h3> [1] A comprehensive comparison between pediatric and adult patients with periodic fever, aphthous stomatitis, pharyngitis, and cervical adenopathy (PFAPA) syndrome. Clin Rheumatol (2017) 36:463–468 <h3>Disclosure of Interest</h3> None declared
Decreased Expression of Serine/Arginine‐Rich Splicing Factor 1 in T Cells From Patients With Active Systemic Lupus Erythematosus Accounts for Reduced Expression of RasGRP1 and DNA Methyltransferase 1
Authors: Michihiro Kono, Takashi Kurita, Shinsuke Yasuda, Michihito Kono, Yuichiro Fujieda, Toshiyuki Bohgaki, Takayuki Katsuyama, George C. Tsokos, Vaishali R. Moulton, Tatsuya Atsumi
Journal: Arthritis & Rheumatology (2018)

Objective T cells from systemic lupus erythematosus (SLE) patients have reduced protein levels of RasGRP1, a guanine nucleotide exchange factor for Ras, and increased transcript of alternatively spliced (AS) forms lacking exon 11. Serine/arginine‐rich splicing factor 1 (SRSF1) binds pre–messenger RNA (pre‐mRNA) to regulate AS forms of several genes, including CD3ζ in SLE T cells. This study was undertaken to assess whether SRSF1 controls the expression of RasGRP1 in T cells from patients with SLE. Methods We studied T cells from 45 SLE patients and 18 healthy subjects. Expression levels of SRSF1, wild‐type (WT) RasGRP1, and DNA methyltransferase 1 (DNMT1) were assessed by quantitative polymerase chain reaction. Direct binding of SRSF1 to exon 11 of RasGRP1 mRNA was evaluated with an oligonucleotide–protein pulldown assay. Healthy T cells and SLE T cells were treated with SRSF1‐specific small interfering RNA or SRSF1 expression vector, respectively, and then evaluated for mRNA/protein expression. Results SRSF1 expression levels were significantly lower in T cells from SLE patients compared to those from healthy subjects, and correlated inversely with disease activity and positively with levels of RasGRP1‐WT and DNMT1. SRSF1 bound directly to exon 11 of RasGRP1 mRNA. Silencing of SRSF1 in human T cells led to increased ratios of RasGRP1‐AS to RasGRP1‐WT and decreased levels of RasGRP1 protein, whereas overexpression of SRSF1 in SLE T cells caused recovery of RasGRP1, which in turn induced DNMT1/interleukin‐2 expression. Conclusion SRSF1 controls the alternative splicing of RasGRP1 and subsequent protein expression. Our findings extend evidence that alternative splicing plays a central role in the aberrant T cell function in patients with SLE by controlling the expression of multiple genes.
SAT0465 Clinical manifestations and prognosis of sle with clinically significant antiphospholipid antibodies
Authors: Y. Zhang, X. Feng, L. Sun
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> Antiphospholipid antibodies (aPLs) have been described in 20%&gt;40% of SLE patients, with 50%&gt;70% of patients with SLE and aPL showing the clinical features of APS after 20 years of follow-up<sup>1</sup>. It assumes that aPLs-positive SLE patients would have a more severe clinical phenotype and worse prognosis than those without aPLs. <h3>Objectives</h3> We decided to investigate the clinical manifestations and prognosis of SLE with clinically significant aPLs in a multiple centre SLE cohort. <h3>Methods</h3> A follow-up study to investigate the prognosis of SLE, has been conducted in 26 centres across Jiangsu province as described before<sup>2</sup>. SLE patients fulfilled ACR/SLICC criteria. Patients who had ever recorded first admissions and detected aPLs during the 1999±2009 decade were followed and checked for their survival status in 2015. Clinically significant aPL were defined as: positive LA test, aCL IgG/IgM antibodies&gt;99 th percentile and/or aβ2GPI&gt;99 th percentile on two or more occasions at least 12 weeks apart. <h3>Results</h3> 1) Among 1372 SLE patients, 495 patients was reported the aPLs minutely, and 146 cases was with clinically significant aPLs. Compared with aPLs negative SLE patients, the proportion of men, and the rates of oral ulcer, neuropsychiatric involvement, dsDNA, antinuclear antibody and C3 were significantly higher in aPLs positive SLE. (table 1) 2) There were 20 deaths in aPLs positive SLE group and 52 deaths in aPLs negative SLE group during the average follow up of 7.38±0.56 years and 7.54±0.0.47 years respectively. There was no significant difference in survival curves by Kaplan Meier survival analysis (p=0.776). (Picture 1) 3) Multivariate Cox regression analysis revealed that long time of diagnosis (HR 4.205, p&lt;0.05), SDI&gt;1 in admission (HR 11.982, p&lt;0.01), neuropsychiatric involvement (HR 2.826, p&lt;0.05), and increased serum creatinine (HR 8.403, p&lt;0.01) were independent predictors of mortality. (table 2) <h3>Conclusions</h3> In this study, we observed that around one-third of patients had clinically significant aPLs, and such autoantibody positivity was associated with a different clinical and serological profile. However, the mortality between aPLs positve and negative SLE patients had no significant difference.SLE patients presented with vital organ damages rather than active disease at initial hospitalisation are likely to have a poor outcome, especially neuropsychiatric involvements and renal insufficiency. <h3>References</h3> [1] Levine JS, Branch DW, Rauch J. The antiphospholipid syndrome. The New England journal of medicine2002;346:752–763. [2] Feng X, Pan W, Liu L, et al. Prognosis for Hospitalized Patients with Systemic Lupus Erythematosus in China: 5-Year Update of the Jiangsu Cohort. PloS One2016;11:e0168619. <h3>Disclosure of Interest</h3> None declared
Measles outbreak hotspots are in US states where parents can opt out of vaccinations
Authors: Janice Hopkins Tanne
Journal: BMJ (2018)

States that make it easy for parents to avoid vaccinating their children are at high risk for outbreaks of measles and other infectious diseases, a study published in PLoS Medicine has shown.1

The percentage of US children who are unvaccinated has risen since 2009. The 2015 National Immunization Survey found that only 72.2% of young children aged 19-35 months were fully vaccinated in line with practice guidelines.

“A social movement of public health vaccine opposition has been growing in the United States in recent years; subsequently, measles outbreaks have also increased,” said the authors of the study, from the National School of Tropical Medicine at Baylor College of Medicine in Houston, …
Boron neutron capture therapy for vulvar melanoma and genital extramammary Paget's disease with curative responses
Authors: Junichi Hiratsuka, Nobuhiko Kamitani, Ryo Tanaka, Eisaku Yoden, Ryuji Tokiya, Minoru Suzuki, Rolf F. Barth, Koji Ono
Journal: Cancer Communications (2018)

Although the most commonly recommended treatment for melanoma and extramammary Paget's disease (EMPD) of the genital region is wide surgical excision of the lesion, the procedure is highly invasive and can lead to functional and sexual problems. Alternative treatments have been used for local control when wide local excision was not feasible. Here, we describe four patients with genital malignancies who were treated with boron neutron capture therapy (BNCT).The four patients included one patient with vulvar melanoma (VM) and three with genital EMPD. They underwent BNCT at the Kyoto University Research Reactor between 2005 and 2014 using para-boronophenylalanine as the boron delivery agent. They were irradiated with an epithermal neutron beam between the curative tumor dose and the tolerable skin/mucosal doses.All patients showed similar tumor and normal tissue responses following BNCT and achieved complete responses within 6 months. The most severe normal tissue response was moderate skin erosion during the first 2 months, which diminished gradually thereafter. Dysuria or contact pain persisted for 2 months and resolved completely by 4 months.Treating VM and EMPD with BNCT resulted in complete local tumor control. Based on our clinical experience, we conclude that BNCT is a promising treatment for primary VM and EMPD of the genital region. Trial registration numbers UMIN000005124.
Development of a novel peptide aptamer-based immunoassay to detect Zika virus in serum and urine
Authors: Do Thi Hoang Kim, Duong Tuan Bao, Hyun Park, Nguyen Minh Ngoc, Seon-Ju Yeo
Journal: Theranostics (2018)

Zika virus (ZIKV) has been identified as a cause of adverse outcomes of pregnancy, including microcephaly and other congenital diseases.Most people infected with ZIKV do not show any symptoms.Development of a method to discriminate dengue virus (DENV) and ZIKV infections has been challenging, and efficient assays for patient management are limited, attributable to high levels of cross-reactivity among co-circulating Flaviviruses.Thus, there is an urgent need for a specific high-throughput diagnostic assay to discriminate ZIKV infections from other Flavivirus infections.Methods: A novel epitope peptide of the ZIKV envelope protein was predicted using three immune epitope database analysis tools and then further modified.A molecular docking study was conducted using three-dimensional structures of the ZIKV envelope and peptide.Experimentally, interactions between the selected peptides and virus were assessed via a fluorescence-linked sandwich immunosorbent assay (FLISA), and performance of peptide-linked sandwich FLISA was evaluated in virus-spiked human serum and urine.Results: The Z_10.8 peptide (KRAVVSCAEA) was predicted to be a suitable detector, with a higher binding affinity than other candidates based on four criteria (binding affinity, root mean square deviation, position of amine residue of lysine at the N-terminus, and interactive site) in a docking study.Z_10.8 was significantly more efficient at detecting ZIKV than the other two peptides, as shown in the direct FLISA (P < 0.001).Further, the equilibrium dissociation constant (K d ) for the Z_10.8 peptide was 706.0 ± 177.9 (mean ± SD, nM), with specificity to discriminate ZIKV from DENV.The limit of detection for the sandwich FLISA was calculated as 1×10 4 tissue culture infective dose (TCID) 50 /mL.The presence of serum or urine did not interfere with the performance of the Z_10.8-linkedsandwich FLISA.Conclusion: Four criteria are suggested for the development of an in silico modeled peptide aptamer; this computerized peptide aptamer discriminated ZIKV from DENV via immunoassay.
AB0261 Ongoing good response with biologics. data from the austrian biorg registry
Authors: M. Herold, G. Eichbauer-Sturm, T. Nothnagl, R. Puchner, B. Rintelen, P. Spellitz, B.F. Leeb
Journal: Annals of the Rheumatic Diseases (2018)

<h3>Background</h3> The goal for the treatment of rheumatoid arthritis (RA) should be remission or at last low disease activity. Using data of the Austrian biologic registry we evaluated disease activity in patients with RA and treated with biologics for more than six years. <h3>Objectives</h3> The aim of this evaluation was to elucidate long term disease activity in patients with RA who are treated with a biologic. We checked data at baseline and at control-visits every six months after inclusion in BioReg. <h3>Methods</h3> Data were extracted from the Austrian BioReg registry (http://www.bioreg.at) which was initiated in 2009 to document patients treated with one of the biologics approved in Austria. Patients with ongoing biologic therapy as well as biologic-naive patients starting biologic therapy can be included (baseline, BL). Further documentation is recommended about every 6 months (V2, V2 up to V12). Until September 2017, 2132 patients (1157 RA, 497 SpA, 401 PsA, 77 other diseases) have been documented. Estimation of disease activity was done using DAS-28, RADAI-5, HAQ as well as ESR and CRP. <h3>Results</h3> DAS-28 (median values of BL; V2; V4; V10;V12) of our patients with RA are 3,30; 2,51; 2,33; 2,49; 2,58, the respective RADAI-5 values are 3,20; 2,20; 2,00; 2.00; 2,40 and values of HAQ are 0,75; 0,50; 0,50; 0,50; 0,63. Median values of inflammation’s laboratory markers (ESR in mm/1st hour and CRP in mg/l) were always within or close to the normal range (ESR and CRP in RA 15; 12; 11,5; 13,5; 11 and 2,1; 2,0; 2,0; 2,4; 3,0 <h3>Conclusions</h3> Our data confirm the efficiency of therapy with a biologic. During 6 years of continuous treatment more than half of the patients with RA are continuously in remission or low disease activity with a DAS-28 below 2,6, RADAI-5 equal or below to 2,40 and normal values of ESR and CRP. <h3>Acknowledgements</h3> BIOREG is supported by an unlimited industrial grant <h3>Disclosure of Interest</h3> None declared
Oral Fluconazole in Pregnancy and Risk of Stillbirth and Neonatal Death
Authors: Björn Pasternak, Viktor Wintzell, Kari Furu, Anders Engeland, Martin Neovius, Olof Stephansson
Journal: JAMA (2018)

This pharmacoepidemiology study uses Swedish and Norwegian registry data to investigate associations between fluconazole use during pregnancy and subsequent stillbirth and neonatal death.
Safety and immune response of a live-attenuated herpes zoster vaccine in patients with systemic lupus erythematosus: a randomised placebo-controlled trial
Authors: Chi Chiu Mok, Kwok Hung Chan, Ling Yin Ho, Yim Fong Fung, Wai Fong Fung, Patrick Chiu Yat Woo
Journal: Annals of the Rheumatic Diseases (2019)

Objectives To study the safety and immunogenicity of a live-attenuated herpes zoster (HZ) vaccine in patients with systemic lupus erythematosus (SLE). Methods Adult SLE patients having a SLEDAI &lt;6 and stable immunosuppressive treatment for ≥6 months were recruited. Participants were randomly assigned to receive HZ vaccine (Zostavax) or placebo injection. Anti-varicella zoster virus (VZV) IgG reactivity (baseline and week 6) was measured by an enzyme-linked fluorescence assay. Cell-mediated response was assessed by a VZV-stimulated interferon-gamma (IFN-γ) enzyme-linked ELISPOT assay. Adverse events and immune responses of the two groups were compared. Results 90 SLE patients were recruited (age 45.6±14.1 years; 93% women) and assigned to Zostavax or placebo (in 1:1 ratio). Baseline clinical parameters were similar between the two groups. The change in anti-VZV IgG from week 0 to 6 was +59.8% in the vaccine and −2.1% in the placebo group. Week 6 anti-VZV IgG was significantly higher in vaccinated than placebo-treated patients, after adjustment for baseline (4.16±1.26 vs 3.32±1.01; p&lt;0.001). The number of IFN-γ secreting T-cell spots decreased in the placebo-treated patients (−17%) but increased in vaccinated patients (+42%). The T-cell spots number at week 6 was significantly higher in vaccine—than placebo-treated patients after adjustment for baseline (38.1±78.2 vs 23.1±47.9; p=0.02). Significantly more vaccinated patients reported self-limiting injection site reaction than controls (31% vs 7%; p&lt;0.01). Two vaccinated patients (4.4%) and one (2.2%) placebo-treated patient had mild/moderate SLE flares but no patients developed HZ eruption within 6 weeks postvaccination. Conclusions In patients with stable SLE not receiving intensive immunosuppression, Zostavax was well-tolerated and provoked an immune response. Trial registration number US ClinicalTrials.gov registry ( NCT02477150 ).
Brain MRI fiber-tracking reveals white matter alterations in hypertensive patients without damage at conventional neuroimaging
Authors: Lorenzo Carnevale, Valentina D’Angelosante, Alessandro Landolfi, Giovanni Grillea, Giulio Selvetella, Marianna Storto, Giuseppe Lembo, Daniela Carnevale
Journal: Cardiovascular Research (2018)

Hypertension is one of the main risk factor for dementia. The subtle damage provoked by chronic high blood pressure in the brain is usually evidenced by conventional magnetic resonance imaging (MRI), in terms of white matter (WM) hyperintensities or cerebral atrophy. However, it is clear that by the time brain damage is visible, it may be too late hampering neurodegeneration. Aim of this study was to characterize a signature of early brain damage induced by hypertension, before the neurodegenerative injury manifests. This work was conducted on hypertensive and normotensive subjects with no sign of structural damage at conventional neuroimaging and no diagnosis of dementia revealed by neuropsychological assessment. All individuals underwent cardiological clinical examination in order to define the hypertensive status and the related target organ damage. Additionally, patients were subjected to DTI-MRI scan to identify microstructural damage of WM by probabilistic fiber-tracking. To gain insights in the neurocognitive profile of patients a specific battery of tests was administered. As primary outcome of the study we aimed at finding any specific signature of fiber-tracts alterations in hypertensive patients, associated with an impairment of the related cognitive functions. Hypertensive patients showed significant alterations in three specific WM fiber-tracts: the anterior thalamic radiation, the superior longitudinal fasciculus and the forceps minor. Hypertensive patients also scored significantly worse in the cognitive domains ascribable to brain regions connected through those WM fiber-tracts, showing decreased performances in executive functions, processing speed, memory, and paired associative learning tasks. Overall, WM fiber-tracking on MRI evidenced an early signature of damage in hypertensive patients when otherwise undetectable by conventional neuroimaging. In perspective, this approach could allow identifying those patients that are in initial stages of brain damage and could benefit of therapies aimed at limiting the transition to dementia and neurodegeneration.
Medical Expulsive Therapy for Ureteral Stones—Stone Age Medicine
Authors: Philipp Dahm, John M. Hollingsworth
Journal: JAMA Internal Medicine (2018)

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Matching Dietary Amino Acid Balance to the In Silico-Translated Exome Optimizes Growth and Reproduction without Cost to Lifespan
Authors: Matthew D.W. Piper, George A. Soultoukis, Eric Blanc, Andrea Mesaros, Samantha L. Herbert, Paula Juricic, Xiaoli He, Ilian Atanassov, Hanna Salmonowicz, Mingyao Yang, Stephen J. Simpson, Carlos Ribeiro, Linda Partridge
Journal: Cell Metabolism (2017)

(Cell Metabolism 25, 610–621; March 7, 2017) In the originally published version of this article, there were errors in the amino acid ratio values reported in the Supplemental Information accompanying the manuscript. They were as follows: in Table S2, (1) the values for columns under FLYAA were a replica of MM3AA, and (2) the “ratio” columns for MM3AA and MM4AA were based on the mass values rather than the molar values as intended. These same errors were found in Data S1 (tab “Fly_diet aa ratios”). Finally, the molar ratio values for the in silico-translated exome of the “animal” in Data S1 (tab “aa limitations calculator,” column F) did not contain the values for Drosophila melanogaster as intended. These errors have now been corrected in the article online. The authors apologize for the errors and any inconvenience that may have resulted. Matching Dietary Amino Acid Balance to the In Silico-Translated Exome Optimizes Growth and Reproduction without Cost to LifespanPiper et al.Cell MetabolismMarch 07, 2017In BriefDietary protein is a critical determinant of health, but the empirical determination of optimal amino acid ratios is challenging. Piper et al. show that a consumer’s genome provides a template for optimal dietary amino acid proportions. Low amounts of optimized protein are simultaneously beneficial for appetite, growth, reproduction, and lifespan. Full-Text PDF Open Access