tbuckley/cabot-search huggingface dataset:
HIGH-SENSITIVITY TROPONIN-I LEVELS PREDICT LONG-TERM MORTALITY INDEPENDENT OF CORONARY ARTERY DISEASE SEVERITY
Authors: Salim Hayek, Yi-An Ko, Mosaab Awad, Hina Ahmed, Brandon Gray, Keyur Patel, Iraj Hesaroieh, Joy Hartsfield, Ravila Bhimani, Neal Patel, Hiroshi Aida, Arianna Sidoti, Agim Beshiri, Jonathan Kim, Peter Wilson, Leslee Shaw, Stephen Epstein, Arshed Quyyumi
Journal: Journal of the American College of Cardiology (2016)
Variant-Sequence Transthyretin (Isoleucine 122) in Late-Onset Cardiac Amyloidosis in Black Americans
Authors: Daniel R. Jacobson, Raymond D. Pastore, Robert Yaghoubian, Immaculata Kane, Gloria Gallo, Francis S. Buck, Joel N. Buxbaum
Journal: New England Journal of Medicine (1997)

After the age of 60, isolated cardiac amyloidosis is four times more common among blacks than whites in the United States; 3.9 percent of blacks are heterozygous for an amyloidogenic allele of the normal serum carrier protein transthyretin in which isoleucine is substituted for valine at position 122 (Ile 122). We hypothesized that the high prevalence of transthyretin Ile 122 is at least partially responsible for the increased frequency of senile cardiac amyloidosis among blacks.
Abstract A37: A dominantly acting allele of murine Mcm4 causes chromosome instability and promotes leukemiogenesis
Authors: Bruce N. Bagley, Vilena Maklakova, Christina Kendziorski, David J. Adams, Lara S. Collier, Jonathon G. Marshall, Rachael A. Lester, Michelle M. Cancel, Laura G. Bendzick, Thomas Keane, Scott C. Kogan, Ruth Sullivan, Robert Cormier
Journal: Cancer Research (2011)

Abstract Mouse models have been invaluable tools for studying human cancer. Given the contribution of mouse models to understanding tumorigenesis, when a spontaneous mouse mutant that developed heritable T cell lymphoblastic leukemia/lymphoma (T-ALL) arose in our colony, we pursued studies to both characterize the disease in these mice and to identify the mutant gene responsible for the phenotype. Because the mutation was spontaneous and the phenotype was dominant, we named the mutant Spontaneous dominant leukemia (Sdl). Sdl heterozygous mice develop disease with short latency and high penetrance; while mice homozygous for the mutation die early during embryonic development. We have discovered a spontaneously acquired mutation in Mcm4 in Sdl mice by whole exome sequencing. MCM4 is part of the heterohexameric complex of MCM2–7 that is important for licensing of DNA origins prior to S phase and also serves as the core of the replicative helicase that unwinds DNA at the replication fork. Previous studies in murine models have discovered that reduced levels of MCM complexes resulting from hypomorphic Mcm alleles promote tumor formation by causing genomic instability. However, Sdl mice possess normal levels of MCMs and there is no evidence for loss-of-heterozygosity at the Mcm4 locus in Sdl leukemias; indicating that the Sdl Mcm4 allele is not a hypomorphic or loss-of-function allele. Sdl mice harbor increased numbers of spontaneous micronuclei and Sdl T-ALLs are characterized by intragenic deletions at the Notch1 locus. Therefore, our studies indicate that dominantly acting alleles of MCMs can be compatible with viability but have dramatic oncogenic consequences caused by chromosome instability. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the Second AACR International Conference on Frontiers in Basic Cancer Research; 2011 Sep 14-18; San Francisco, CA. Philadelphia (PA): AACR; Cancer Res 2011;71(18 Suppl):Abstract nr A37.
SAT0227 Urinary heparanase activity is elevated in patients with lupus nephritis and correlate with protein excretion
Authors: K.-J. Kim, J.-Y. Kim, S.-J. Park, I.-W. Baek, C.-H. Yoon, W.-U. Kim, C.-S. Cho
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> The heparin sulphate proteoglycans (HSPGs) in the glomerular basement membrane (GBM) play an important role in the charge-selective permeability of the glomerular filter. The β-D-endoglycosidase heparanase has been proposed to be important in the pathogenesis of proteinuria by selectively degrading the negatively charged side chains of HSPGs within the GBM in various forms of glomerulonephritis. <h3>Objectives</h3> To evaluate plasma and urinary activity of heparanase and to determine association between its levels and proteinuria in patients with lupus nephritis <h3>Methods</h3> Plasma from 50 patients with systemic lupus erythematosus and 10 normal healthy subjects were collected. The clinical and laboratory data of the patients were obtained at the time of sampling. Thirty three patients had a history of lupus nephritis and their urine was also collected. Proteinuria was defined as more than 0.5 g/day. Heparanase activity of plasma and urine was determined by a commercially available kit. <h3>Results</h3> Plasma heparanase activity was significantly elevated in SLE patients compared to controls (745.2±112.5 <i>vs</i>. 203.6±87.1 mIU/ml, <i>p</i> =0.039). Twenty five of patients with lupus nephritis showed significant proteinuria and their heparanase activity was not different from those without proteinuria (607.0±123.1 <i>vs</i>. 700.8±224.3 mIU/ml, <i>p</i> =0.725). On the other hand, urinary heparanase activity of patients with proteinuria was significantly elevated compared with those of patients without proteinuria (1092.1±334.2 <i>vs</i>. 93.3±42.8 mIU/ml, <i>p</i> =0.008). Moreover, urinary heparanase activity was positively correlated with 24 hours protein excretion (γ =0.546, <i>p</i> =0.016). Urinary heparanase activity showed an inverse correlation with complement haemolytic activity (CH50) (γ = -0.454, <i>p</i> =0.030) and had a tendency to associate negatively with C3 and C4 complement levels (γ = -0.331, <i>p</i> =0.123 and γ = -0.299, <i>p</i> =0.166, respectively). <h3>Conclusions</h3> Urinary heparanase activity was elevated in patients with lupus nephritis and reflect the urinary protein excretion, suggesting a potential role in the pathogenesis of proteinuria in lupus nephritis. <h3>Disclosure of Interest</h3> None Declared
Abstract 1735: Obatoclax enhances CDDP and trail-induced apoptosis in epithelial ovarian adenocarcinoma cells.
Authors: Melisa A. Martinez-Paniagua, Luis A. Romero-Gonzalez, Yu Mei Anguiano-Hernandez, Carolina Gonzalez-Torres, Mario I. Vega
Journal: Cancer Research (2013)

Abstract Epithelial ovarian cancer comprises the majority of malignant ovarian tumors in adult women. Ovarian cancer is the fifth cause of death in women and annually 13,850 deaths are reported in United States. Effective screening strategies are lacking and the majority of cases are diagnosed with advanced stage disease. Chemo is the conventional therapy after surgery but the initial response is between 60 to 80%, however, some patients that initially responded to the therapy suffer of recurrent disease or become resistant to the treatment. As a consequence, the overall 5 year survival is only about 30%. It is important to identify novel therapies for ovarian cancer to treat or sensitize tumor cells to the existence therapy. It has been suggested that the tumor cells become resistance due to an increased expression of antiapoptotic proteins especially from the Bcl2 family (Bcl-2, Bcl-XL, Mcl-1). Obatoclax is a small molecule that mimics the BH3 domain of the BH3 only family members and inhibits the effect of antiapoptotic Bcl-2 members. The objective of this study was to assess the susceptibility of ovarian cancer cell lines to chemo or immuno therapy mediated apoptosis by obatoclax and also to elucidate the mechanism. Four different epithelial ovarian adenocarcinomas cell lines (SKOV3, NIH-OVCAR-3, TOV112D and TOV21G) were treated with different concentrations of Obatoclax (nM-μM), CDDP (5-50μg/ml) or TRAIL (50-1200ng/ml) alone or with a combination of treatment to assess the viability, measured by the XTT kit (Roche). Basal and cell treated total protein expressions were assessed by western. Induction of apoptosis was measured by TUNEL. As a single agent, Obatoclax or CDDP inhibited the viability of the cells in a dose response form; TRAIL did not show an evident response. The combination of the treatment after selecting the IC50 and IC20 response showed an increased inhibition of the viability. TUNEL positive cells were measured and the induction of apoptosis was mediated by a caspase dependent manner and PARP cleavage. Our data suggest that obatoclax in combination with CDDP or TRAIL can enhance the induction of apoptosis in resistance cell lines, suggesting to be an optional treatment of patients with ovarian cancer. Citation Format: Melisa A. Martinez-Paniagua, Luis A. Romero-Gonzalez, Yu Mei Anguiano-Hernandez, Carolina Gonzalez-Torres, Mario I. Vega. Obatoclax enhances CDDP and trail-induced apoptosis in epithelial ovarian adenocarcinoma cells. [abstract]. In: Proceedings of the 104th Annual Meeting of the American Association for Cancer Research; 2013 Apr 6-10; Washington, DC. Philadelphia (PA): AACR; Cancer Res 2013;73(8 Suppl):Abstract nr 1735. doi:10.1158/1538-7445.AM2013-1735
Route of Administration Determines Induction of T-Cell-Independent Humoral Responses to Adeno-Associated Virus Vectors
Authors: W Xiao, N Chirmule, M A Schnell, J Tazelaar, J V Hughes, J M Wilson
Journal: Molecular Therapy (2000)

Vectors based on adeno-associated viruses (AAV) type 2 show promise for treating chronic diseases because transgene expression appears to be stable. This study evaluated the impact of humoral immunity to the capsid proteins on vector uptake by hepatocytes following an intravascular approach. Route of vector administration in mice had a qualitative effect on antivector B cell responses. Administration of vector into the tail vein resulted in T-cell-dependent (TD) B cell responses that were completely inhibited with depleting CD4 antibody. Delivery of vector into the portal circulation via the spleen yielded B cell response that were partially T cell independent (TI) rendering strategies based on T cell inhibition ineffective in allowing vector readministration. The TI B cell response was short lived in comparison to the TD response. Rhesus monkeys produced a B cell memory response to intraportal vector which appeared to be T cell dependent based on Ig isotypes. Gene therapy strategies that require AAV vector readministration should consider vector biodistribution and its impact on B cell activation.
Abstract 2745: Genetic determinants of telomere length – a genome wide association study
Authors: Meng Chen, Jian Gu, Yuanqing Ye, Jie Lin, David Chang, Xifeng Wu
Journal: Cancer Research (2011)

Abstract Telomere plays a critical role in maintaining genome integrity. Telomere shortening is associated with the risk of many age-related diseases. Classical twin studies have shown that genetic components may contribute up to 80% of the heritability of telomere length. Previous association studies have reported several genetic loci in regulating telomere length, but the results have been inconsistent. In this study, we adopted a three-stage genome-wide association study (GWAS) design to identify the genetic determinants of telomere length. The mean telomere length was measured by quantitative real-time PCR. Genotyping were done by illumin's whole-genome SNP array in stage I and II, and by TaqMan assay in stage III. Multivariate linear regression analysis was applied to assess the association between genetic variants and telomere length adjusting for age and gender. In stage I, we analyzed 300K SNPs in 459 healthy controls and found 15,351 SNPs that were associated with telomere length at P&amp;lt;0.05. In phase II, we analyzed these 15,351 SNPs in two independent populations with GWAS data with 952 control subjects and 272 control subjects, respectively, and identified 18 SNPs, each with a combined P value &amp;lt; 10−4. Among them, two loci located at chromosome 20q11.22 and 14q21 were associated with telomere length with a significant P value &amp;lt; 10−7. We are in the process of genotyping these 18 SNPs and measuring telomere lengths in approximately 1,200 independent control subjects for stage III validation. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 102nd Annual Meeting of the American Association for Cancer Research; 2011 Apr 2-6; Orlando, FL. Philadelphia (PA): AACR; Cancer Res 2011;71(8 Suppl):Abstract nr 2745. doi:10.1158/1538-7445.AM2011-2745
Reduced Radioactivity in the Periphery of the Liver in a Patient With Idiopathic Portal Hypertension
Authors: SHIRO AKAKI, AKIHITO MITSUMORI, SUSUMU KANAZAWA, YOSHIHIRO TAKEDA, IKUO JOJA, YOSHIO HIRAKI, KOSAKU SAKAGUCHI
Journal: Clinical Nuclear Medicine (1997)

The authors report a case of idiopathic portal hypertension in which radioaccumulation in the peripheral region of the liver decreased markedly. On dynamic CT, peripheral regional enhancement of the liver was seen in the arterial phase. The region was hypointense on T1-weighted MR images and hyperintense on T2-weighted images. On portograms via the superior mesenteric artery, markedly decreased portal venous perfusion was seen in the peripheral region of the liver. Tc-99m galactosyl human serum albumin (GSA) liver scintigrams showed decreased accumulation in the peripheral region and unchanged accumulation in the central region of the liver. Tc-99m GSA liver scintigraphy clearly showed localized liver dysfunction in the peripheral region.
Polymyositis and Dermatomyositis
Authors: Anthony Bohan, James B. Peter
Journal: New England Journal of Medicine (1975)

POLYMYOSITIS is an inflammatory myopathy of unknown cause to which the term dermatomyositis is applied in the presence of the characteristic skin rash. These disorders share common features with the connective-tissue diseases and are usually classified together. It is said that there is an increased association with neoplasia.The Problem of DiagnosisThere are at present no generally accepted criteria for the diagnosis of polymyositis-dermatomyositis akin to the Jones criteria for rheumatic fever or to the American Rheumatism Association criteria for rheumatoid arthritis. Consequently, the basis for the diagnosis has varied widely in retrospective studies, which, . . .
A continuing debate on 6-minute withdrawal time as a quality indicator during colonoscopy
Authors: U. Saritas, Y. Ustundag
Journal: Endoscopy (2012)

We read with interest the article by Moritz et al. [1], which concluded that a 6-minute withdrawal time should not be used as an indicator of quality or as a surrogate end point for adenoma detection rate in colonoscopy. This conclusion was drawn because no difference was found in the adjusted odds ratios for the detection of polyps (≥ 5 mm) for endoscopists with a median visual withdrawal time (VWT) of ≥ 6 minutes compared with endoscopists with a median VWT of < 6 minutes.
THU0356 Benefits of a multidisciplinary treatment in women with fibromyalgia and obesity
Authors: S. Castro, R. Fontova, M.J. Poveda, A. Castel, R. Qanneta, S. Montull, R. Periñan, I. Miralles, N. Aragonés, I. Salvat, R. Cascόn, S. Monterde, A. Padrol, C. Añez, M. Rull
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> Fibromyalgia (FM) is a musculoskeletal chronic pain condition with low response to pharmacological conventional treatment (CPhT). Multidisciplinary treatment (MT) has showed efficacy in improving FM symptoms. Nevertheless, MT has not been studied in patients with FM and obesity. <h3>Objectives</h3> To compare the benefits of the CPhT versus the MT (with pharmacological, cognitive-behavioural, and physiotherapy treatments) in a sample of women with FM and obesity (Body Mass Index ≥30). <h3>Methods</h3> 44 women with obesity and FM according to the American College of Rheumatology criteria. Mean age 49.9 years (S.D. 6.4). 21 patients attended CPhT and 23 MT. Patients were assessed before treatment, after treatment, and at 3-6-12 month follow-up. Outcomes were: pain intensity (Numeric Rating Scale), functionality (FIQ), psychological distress (HADS), quality of life (COOP-WONCA), and sleep problems (Medical Outcome Study: MOS). <h3>Results</h3> The interaction group x time with mixed linear model analyses demonstrated that patients of the MT group improved more than patients of the CPhT group in pain intensity (p&lt;.001), functionality (p&lt;.0001), quality of life (p&lt;.05), and sleep index problems (p&lt;.001). <h3>Conclusions</h3> MT consisting of pharmacological treatment, cognitive-behavioural psychological treatment and physiotherapy improves FM symptoms in women with FM and obesity. Improvements were maintained at follow-up. Supported by the Foundation Maratό Grant Number 070910 <h3>Disclosure of Interest</h3> None Declared
Esophageal Scarring Causing False-Positive Uptake on I-131 Whole-Body Imaging
Authors: DAVID M. SCHUSTER, NAOMI ALAZRAKI
Journal: Clinical Nuclear Medicine (1998)

Swallowed esophageal activity can simulate uptake in the neck on I-131 imaging. The authors present a case of a 49-year-old man, 6 years after resection of simultaneous papillary thyroid cancer and mediastinal thymoma, with subsequent external beam radiotherapy and 150 mCi I-131 ablation. No abnormality was present on I-131 imaging 1 year later. The follow-up scan demonstrates uptake in the surgical bed region, which disappeared after the patient drank water. A CT scan illustrates scarring and dilatation of the esophagus due to post-surgery and radiation sequelae at this level, causing hold-up of swallowed radiotracer even at 72 hours.
OC-001 The Impact of the Introduction of Formalised Polypectomy Assessment on Training in the United Kingdom
Authors: K Patel, O Faiz, M D Rutter, P Dunckley, S Thomas-Gibson
Journal: Gut (2013)

<h3>Introduction</h3> Polypectomy is regarded as the most hazardous part of colonoscopy, accounting for the majority of procedure-associated morbidity and yet is a necessary skill for all colonoscopists. Training in polypectomy has, to date, been variable and poorly structured. Anecdotal evidence suggested poor exposure to polypectomy during training. A novel assessment tool, the Directly Observed Polypectomy Skills (DoPYS), was introduced nationally in the United Kingdom in October 2011 with the intention of both improving training and facilitating documentation of competency. <h3>Methods</h3> The aim was to assess the impact of the mandatory introduction of the DOPyS as part of the formal colonoscopy certification process. Applications for certification in the year prior to the introduction of DOPyS were analysed retrospectively and compared with data collected prospectively for those in the following year. Data were collected on the total lifetime number of colonoscopies performed, the number of assessments for both colonoscopy and polypectomy and whether applicants had any evidence of performing polypectomy before certification of competence in colonoscopy. <h3>Results</h3> There were 175 applicants for certification in the first year. The median number of procedures per candidate was 287. Thirty two per cent of candidates had evidence of any observed polypectomy with 7 per cent of candidates referring to training in endoscopic mucosal resection (EMR). The median number of formative colonoscopy assessments was 3 (range 0–16). In the year since DOPyS was introduced there were 150 applications for certification. The median number of procedures per candidate was 206. All of these candidates had evidence of polypectomy assessment with a median number of DOPyS of 7 (range 3–27). 89 per cent of applicants had evidence of assessed EMR. The median number of formative colonoscopy assessments in this cohort was 32 (range 9–199). There was a significant increase in the number of logged polypectomy assessments (p &lt; 0.001), experience of EMR (p &lt; 0.001) and formative colonoscopy assessments (p &lt; 0.001). There was no significant difference in the total number of colonoscopy procedures performed. <h3>Conclusion</h3> These data – the largest in the literature to date – show that structured polypectomy assessment improves trainees' documented exposure to therapeutic endoscopy as well as providing formal evidence of skills acquisition. As polypectomy plays an increasing role globally in colorectal cancer prevention, the DOPyS provides an effective means of assessing and certifying polypectomy in order to minimise the well-recognised risks associated with this technique. <h3>Disclosure of Interest</h3> None Declared.
Endoscopic ultrasound-guided transgastric biliary metal stent placement after dilation with tapered-tip Teflon catheters and marking with a clip
Authors: V. Prachayakul, P. Aswakul, S. Leelakusovong
Journal: Endoscopy (2012)

We report the case of a 74-year-old man with known type 2 diabetes mellitus, hypertension, and retinal detachment who was referred for endoscopic retrograde cholangiopancreatography (ERCP) because of a fever and severe upper abdominal pain for a few days. An ultrasound of his upper abdomen showed a 4 × 5-cm heterogeneous hypoechoic area in the gallbladder fossa with intrahepatic bile duct dilatation, which it was suspected was due to gallbladder cancer. He therefore proceeded to ERCP for biliary drainage.
Radionuclide Imaging in Primary Amyloidosis with Liver Involvement
Authors: SHY-DEN CHEN, CHIA-HUNG KAO, SEK-KWONG POON
Journal: Clinical Nuclear Medicine (1998)

A 74-year-old man with hepatomegaly, hyperglycemia, and proteinuria was diagnosed with primary amyloidosis with liver involvement, proven by biopsy. Abnormal distribution of tracer in the liver on Tc-99m phytate liver-spleen imaging and abnormal tracer uptake by the liver on Tc-99m pyrophosphate whole body imaging were observed. Scintigraphic imaging studies may be used noninvasively to evaluate the involvement of organs in patients with primary amyloidosis, reducing the risk of bleeding caused by biopsy.
SAT0266 Behcet's Disease in Morocco: Report of 1484 Cases
Authors: F.-Z. Alaoui, M. Moudatir, K. Echchilali, S. Benamour, H. El Kabli
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> Behçet9s disease is a complex multi-system disorder of unknown aetiology characteristically presenting with recurrent oral ulcers. It is presumed to be a vasculitis and includes involvement of the ocular, cardiovascular, renal, gastrointestinal, pulmonary,vascular, musculoskeletal, and central nervous systems. <h3>Objectives</h3> It is a study of 1484 cases of Behcet9s disease (BD) diagnosed between 1981 and 2013 in an internal medicine department. Even in countries where BD is frequent, some unusual features were unknown, raising diagnostic problems. <h3>Methods</h3> It is a retrospective study of 1484 cases of Behcet9s disease (BD). All patients fulfilled the diagnostic criteria of the International Study Group for BD and the Davatchi criteria (2011). <h3>Results</h3> Patients aged between 20 and 40 years were mostly affected (73.8%) with male preponderance (70%). All patients had mucocutaneous lesions including oral ulceration (100%), genital ulcers (84%) and positive pathergy test (52%). Eye lesions were frequent (64.5%) and led to blindness in 30% of cases. Joint involvement was seen in 85% of cases with polyarthritis in 104 cases. Deep venous thrombbosis were observed in 17% of cases complicated by pulmonary embolism in 13 cases. Superior vena cava thrombosis was found in 32 cases. Neurological involvement was present in 19% of cases. 3 patients had intracardiac aneurysms with myocardiopathy in one case. <h3>Conclusions</h3> In this study, unusual features are observed. Polyarthritis and vena cava thrombosis are not rare. Cardiac involvement can be seen in BD. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.6022