tbuckley/cabot-search huggingface dataset:
Pleomorphic Characteristics of a Germ-Line <b> <i>KIT</i> </b> Mutation in a Large Kindred with Gastrointestinal Stromal Tumors, Hyperpigmentation, and Dysphagia
Authors: Mark E. Robson, Emily Glogowski, Gunhild Sommer, Cristina R. Antonescu, Khedoudja Nafa, Robert G. Maki, Nathan Ellis, Peter Besmer, Murray Brennan, Kenneth Offit
Journal: Clinical Cancer Research (2004)

Abstract Purpose: Somatic mutations that result in the activation of the growth factor receptor KIT are commonly found in gastrointestinal stromal tumors (GISTs). Six families have been reported in which a germ-line mutation in KIT is associated with an autosomal dominant predisposition to the development of GISTs. Hyperpigmentation, urticaria pigmentosa, and dysphagia have been described in some, but not all, families. Preliminary correlations between the site of mutation and the clinical phenotype have been proposed, but the strength of these associations is not defined. Design: A large kindred with multiple GISTs, hyperpigmentation, and dysphagia was identified after the index case presented with multiple GISTs. A germ-line mutation in KIT (W557R) was identified in an affected cousin, after which a large family meeting was held and testing offered. Clinical data were obtained by interview and, whenever possible, medical record documentation. Results: To date, 19 individuals have been tested, and the mutation has been shown to cosegregate with the syndrome. The phenotypic expression, however, is variable. GISTs, often presenting as upper gastrointestinal bleeding, and hyperpigmentation are common, but not diagnosed in all documented or obligate carriers. Dysphagia is a less prevalent complaint. The diagnosis of GISTs appears to be made at a younger age in more recent generations. Metastatic disease is uncommon. Conclusions: A germ-line mutation in KIT resulting in an amino acid substitution in the juxtamembrane region is associated with a syndrome of GIST, hyperpigmentation, and dysphagia, although the prominence of each component varies.
TCT-771 Comparison of post TAVR paravalvular regurgitation between Edwards SAPIEN 3 and Edwards SAPIEN XT; early, single center experience
Authors: Vasileios F. Panoulas, Gennaro Giustino, Azeem Latib, Matteo Montorfano, Alaide Chieffo, Paola Spatuzza, Maurizio Taramasso, Filippo Figini, Eustachio Agricola, Pietro Spagnolo, Ottavio Alfieri, Antonio Colombo
Journal: Journal of the American College of Cardiology (2014)
Relationship between ARIA classification and drug treatment in allergic rhinitis and asthma
Authors: L. Antonicelli, C. Micucci, S. Voltolini, G. E. Senna, P. Di Blasi, G. Visonà, R. De Marco, F. Bonifazi
Journal: Allergy (2007)

Introduction: The Allergic Rhinitis and its Impact on Asthma (ARIA) guidelines provide a stepwise treatment to rhinitis, which classifies the disease according to its duration and severity. Objectives: The aim of the study was to verify whether these variables influence drug prescriptions for rhinitis and asthma. Methods: A multi‐centre cross‐sectional pharmaco‐epidemiological survey was carried out on 1610 allergic rhinitis patients and the relationship between the clinical features of rhinitis and drug therapy for rhinitis and asthma was evaluated. Results: A total of 1321 adult patients were enrolled. Mild intermittent rhinitis was diagnosed in 7.7%of the patients, moderate/severe intermittent in 17.1%, mild persistent in 11.6%, and moderate/severe persistent in 63.6%. A high level of rhinitis‐asthma comorbidity (616/1321 = 46.6%) was found. The majority of patients [1060 (80.24%)] were treated. Significant associations between the severity of rhinitis and the presence of therapy ( P = 0.008), the use of oral antihistamines ( P &lt; 0.001), topical nasal steroids ( P = 0.020) and systemic steroids ( P = 0.005) were found. A weak association was found between the features of rhinitis and the therapy for asthma, and v ice versa the comorbidity with asthma increases the prescription of inhalant ( P &lt; 0.001) and oral steroids ( P = 0.015) to treat rhinitis. Conclusion: The severity of rhinitis influences patient request for rhinitis therapy and the type of medication more than the duration. These features of rhinitis seem to poorly influence asthma therapy. As the ARIA classification is able to reveal a relevant impairment notwithstanding therapy, its role in treated patients merits further study.
Role of teichoic acids in Staphylococcus aureus nasal colonization, a major risk factor in nosocomial infections
Authors: Christopher Weidenmaier, John F Kokai-Kun, Sascha A Kristian, Tanya Chanturiya, Hubert Kalbacher, Matthias Gross, Graeme Nicholson, Birgid Neumeister, James J Mond, Andreas Peschel
Journal: Nature Medicine (2004)
SYNTAX SCORE BY CORONARY CT ANGIOGRAPHY FOR PREDICTION OF COMPLEX PCI
Authors: Aryeh Shalev, Ryo Nakazato, Reza Arsanjani, Rine Nakanishi, Hyung Bok Park, Yuka Otaki, Victor Cheng, Damini Dey, Troy LaBounty, Heidi Gransar, Sean Hayes, Louise Thomson, John Friedman, Daniel Berman, James Min
Journal: Journal of the American College of Cardiology (2013)
Protective Effect of Endotoxin Instillation on Subsequent Bacteria-induced Acute Lung Injury in Rats
Authors: DANIEL JEAN, SAÏDA REZAIGUIA-DELCLAUX, CHRISTOPHE DELACOURT, ROLAND LECLERCQ, CHANTAL LAFUMA, CHRISTIAN BRUN-BUISSON, ALAIN HARF, CHRISTOPHE DELCLAUX
Journal: American Journal of Respiratory and Critical Care Medicine (1998)

The phagocytic capability afforded by neutrophil influx into the lungs is essential to ward off invading bacteria. The objective of this study was to evaluate the effect of prior neutrophil recruitment induced by alveolar instillation of endotoxin (LPS, 200 μ g/kg) 16 h before a pulmonary infection caused by instillation of live Pseudomonas aeruginosa ([PYO]: 1.5 × 108 colony-forming units [cfu]/kg) in rats. A first series of experiments showed that lipopolysaccharide (LPS) instillation induced recruitment of alveolar neutrophils that were capable, ex vivo, of elastase exocytosis, reactive oxygen species secretion, and PYO killing. In a second set of experiments, LPS followed by PYO was compared with PYO alone (n = 11 surviving rats in each group). Parameters were studied 24 h after the bacterial challenge. As compared with PYO alone, pretreatment with LPS followed by PYO was associated with decreased mortality (0% versus 54%, p < 0.05), decreased protein leakage into bronchoalveolar lavage (BAL) fluid (1.8 ± 0.4 versus 13.5 ± 2.2 mg/ml, p < 0.001), and improved bacterial clearance from BAL (4.0 ± 1.4 × 102 versus 1.2 ± 0.5 × 104 cfu/ml, p < 0.05) and from pulmonary parenchyma (8.5 ± 6.4 × 105 versus 1.9 ± 0.8 × 107 cfu/ml, p < 0.05). We conclude that prior alveolar endotoxin instillation induces local recruitment of functionally active neutrophils, and that this is associated with resistance to subsequent experimental pneumonia.
A Randomized Controlled Trial of Acetyl Salicyclic Acid in the Secondary Prevention of Mortality from Myocardial Infarction
Authors: P. C. Elwood, A. L. Cochrane, M. L. Burr, P. M. Sweetnam, G. Williams, E. Welsby, S. J. Hughes, R. Renton
Journal: BMJ (1974)

The results of a randomized controlled trial of a single daily dose of acetyl salicylic acid (aspirin) in the prevention of reinfarction in 1,239 men who had had a recent myocardial infarct were statistically inconclusive. Nevertheless, they showed a reduction in total mortality of 12% at six months and 25% at twelve months after admission to the trial. Further trials are urgently required to establish whether or not this effect is real.
Audit of gastrointestinal bleeding in a district general hospital.
Authors: M. G. Dunnill, S. R. Gould
Journal: BMJ (1992)

design features.Case matching is a more reliable fatality rates in this particular trial) must reflect the dose, defined as the amount needed to produce the means of eliminating errors than comparing the probability that the result was simply due to required effect.study group with a control group of a different size mismatching.The gastric acid outputs or acidities of the as in Hinton's survey.
A Case of Purpura Fulminans Arising from Cryptococcosis
Authors: Ryota Inokuchi, Kensuke Nakamura, Yuta Aoki, Takahiro Hiruma, Masataka Gunshin, Takehiro Matsubara, Takeshi Ishii, Susumu Nakajima, Naoki Yahagi, Hajime Sato, Kazuaki Shinohara
Journal: American Journal of Respiratory and Critical Care Medicine (2012)
Abstract 4285: Visualizing cancer vaccine clearance <i>in vivo</i> using magnetic resonance imaging
Authors: Kim Brewer, Kerry Lake, Nicole Pelot, Drew DeBay, Andrea Penwell, Genevieve Weir, Chris Bowen, Mohan Karkada, Marc Mansour
Journal: Cancer Research (2012)

Abstract Magnetic Resonance Imaging (MRI) is a powerful tool in the evaluation of immunotherapies, since it permits non-invasive longitudinal assessment of tumor growth, and as our group has previously demonstrated, it allows monitoring of immune response via volumetric changes in lymph nodes (LNs) adjacent to (inguinal LNs) or distant from (popliteal LNs) the site of vaccination. MRI also offers potential for visualizing the uptake behavior of vaccines such as DepoVaxTM, a novel liposome-in-oil-based vaccine platform developed by Immunovaccine Inc. In the context of cancer immunotherapy, this platform uses tumor-associated antigens (TAA), adjuvant and stimulator of T helper cells encapsulated in liposomes and suspended in oil. The oil substrate of the DepoVaxTM immunotherapy vaccine platform acts as an adjuvant that greatly increases the potency of the vaccines, eliciting a strong cytotoxic T-cell response, and also permits longitudinal visualization of the vaccine site with MRI. However, this does not reveal any information about the clearance time of the individual vaccine components, particularly the TAAs. By attaching superparamagnetic iron oxide (SPIO) to the TAA and then encapsulating it in liposomes, it is possible to visualize the biodistribution of the TAA over time using MRI and evaluate whether there is truly a slow clearance of the antigen from the depot site, facilitating a prolonged immune response. In this study, tumor-bearing mice challenged with C3 cells (HPV16 tumor model) were evaluated for the longitudinal clearance of the DepoVaxTM vaccine components using SPIO conjugated to the TAA or associated with the lipid component of the vaccine. The SPIO-conjugated antigen was also tested in a Montanide® oil emulsion vaccine as a control. Over the course of this study, we observed the vaccine being distributed to the surrounding tissue from the injection site, and the antigen was actively transported from the vaccine depot site. The iron-labeled antigen cleared slowly but consistently, decreasing by approximately 40% over 35 days. This study provides direct in vivo evidence that DepoVaxTM resides at the vaccine injection site for a prolonged period of time, increasing exposure to antigen in a controlled fashion, which is likely a key factor in the enhanced immune response elicited by this novel depot vaccine. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 103rd Annual Meeting of the American Association for Cancer Research; 2012 Mar 31-Apr 4; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2012;72(8 Suppl):Abstract nr 4285. doi:1538-7445.AM2012-4285
Isolation of a human epidermal cDNA corresponding to the 180-kD autoantigen recognized by bullous pemphigoid and herpes gestationis sera. Immunolocalization of this protein to the hemidesmosome.
Authors: L A Diaz, H Ratrie, W S Saunders, S Futamura, H L Squiquera, G J Anhalt, G J Giudice
Journal: Journal of Clinical Investigation (1990)

Autoantibodies present in the sera of patients with bullous pemphigoid (BP) bind to the basement membrane zone of normal human skin and commonly recognize two epidermal proteins, the BP240 and BP180 antigens. Two BP antigen cDNA clones from a lambda gt11 human keratinocyte library have been identified on the basis of reactivity with a BP serum. The fusion protein (FP) produced by one clone immunoadsorbed autoantibodies, which specifically recognized the BP180 by antigen, showing no cross-reactivity with BP240 by immunoblot analysis. The FP produced by the second clone immunoadsorbed autoantibodies which specifically reacted with the BP240 epidermal antigen. Northern blot analysis demonstrated that the BP180 and BP240 antigens are encoded by distinct RNA transcripts with lengths of 6.0 and 8.5 kb, respectively. Immunoblot analysis of the BP180 lysogen extract identified a 135-kD FP which was recognized by 7 of 16 BP sera and 7 of 8 herpes gestationis sera. A rabbit antiserum prepared against the lysogenic BP180 FP specifically recognized the BP180 antigen from human epidermal extracts by immunoblotting, labeled the BMZ by indirect immunofluorescence, and bound to human epidermal hemidesmosomes by immuno-electron microscopy. These results indicate that the BP180 antigen recognized by BP and herpes gestationis autoantibodies is a unique hemidesmosomal polypeptide, distinguishable from the BP240 antigen.
LITHOPEDION CARRIED TWENTY-SIX YEARS OR MORE.
Authors: H. W. MOREHOUSE
Journal: JAMA (1907)

<h3>History.</h3> —Mrs. B., aged 62 years, commenced to menstruate at the age of fourteen years. Menstruation was always regular and painless. The patient was married at the age of twenty. Her first child was born March 13, 1866; the second was born Sept. 16, 1867; the third, Oct. 9, 1869; the fourth, Aug. 16, 1871. She missed a menstrual period in July, 1880. In August she had a sharp attack of abdominal pain requiring rest in bed and morphin; three days later she had another attack still more severe, necessitating confinement to bed for almost a month. In September there occurred a very severe attack of pain accompanied by symptoms of collapse. She was confined to her bed for three or four weeks thereafter, and was not well enough to return to her home, a short distance in the country, until November. In December a colored uterine discharge appeared, the
FDA Asks Physicians to Stop Prescribing High-Dose Acetaminophen Products
Authors: Mike Mitka
Journal: JAMA (2014)

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 HomeNew OnlineCurrent IssueFor Authors 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) Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More 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 journal
Cardiac Pheochromocytomas Detected by Tc-99m-Hydrazinonicotinyl-Tyr3-Octreotide (HYNIC-TOC) Scintigraphy
Authors: Libo Chen, Fang Li, Hongming Zhuang, Hongli Jing, Yanrong Du, Zhengpei Zeng
Journal: Clinical Nuclear Medicine (2007)

Primary pheochromocytomas of the heart are extremely rare tumors and difficult to diagnose. Iodine labeled metaiodobenzylguanidine (MIBG) is the first choice of the nuclear medicine modality in the evaluation of adrenal pheochromocytoma. However, the sensitivity of MIBG in the diagnosis of extraadrenal pheochromocytoma is less optimal. In this preliminary report, the efficacy of octreotide scintigraphy using Tc-99m labeled hydrazinonicotinyl-Tyr3-octreotide (HYNIC-TOC) in the evaluation of primary cardiac pheochromocytoma was assessed.
Promotion of cycling and health
Authors: N. Mutrie, F. Crawford
Journal: BMJ (2010)

England’s chief medical officer in 2009 called physical activity a potential “wonder drug,”1 and promoting physical activity has been called a “best buy” in public health.2 In our sedentary and technologically advanced societies, population levels of physical activity are low, and knowledge about how to invest in this best buy is urgently needed. Our challenge is to find methods of making this wonder drug more palatable to more people more often
Beta blocker specificity: a building block toward personalized medicine
Authors: Brent R. DeGeorge, Walter J. Koch
Journal: Journal of Clinical Investigation (2007)

Drugs known as beta blockers, which antagonize the beta-adrenergic receptor (beta-AR), are an important component of the treatment regimen for chronic heart failure (HF). However, a significant body of evidence indicates that genetic heterogeneity at the level of the beta(1)-AR may be a factor in explaining the variable responses of HF patients to beta blockade. In this issue of the JCI, Rochais et al. describe how a single amino acid change in beta(1)-AR alters its structural conformation and improves its functional response to carvedilol, a beta blocker currently used in the treatment of HF (see the related article beginning on page 229). This may explain why some HF patients have better responses not only to carvedilol but to certain other beta blockers as well. The data greatly enhance our mechanistic understanding of myocardial adrenergic signaling and support the development of "tailored" or "personalized" medicine, in which specific therapies could be prescribed based on a patient's genotype.
Imatinib mesylate as a cause of acute liver failure
Authors: Timothy J.S. Cross, Catherine Bagot, Bernard Portmann, Julia Wendon, D. Gillett
Journal: American Journal of Hematology (2006)

A 46-year-old patient diagnosed with chronic myeloid leukemia in whom cytogenetic and molecular remission had never been achieved was commenced on Imatinib Mesylate (Gleevec®, Novartis Pharmaceuticals Corp., East Hanover, NJ). After 18 months of treatment, she developed abnormal liver function tests and subsequently acute liver failure, requiring transfer to the regional liver unit. The patient proceeded to liver transplantation but later died. The explanted liver had histological features of severe hepatic necrosis. This is the first case described of fatal hepatic necrosis in a patient who has have been on long-term imatinib therapy. This may have implications for long-term use of the drug and emphasizes the need for regular monitoring of liver function. Am. J. Hematol. 81:189–192, 2006. © 2006 Wiley-Liss, Inc.
Enzyme replacement therapy in a feline model of Maroteaux-Lamy syndrome.
Authors: A C Crawley, D A Brooks, V J Muller, B A Petersen, E L Isaac, J Bielicki, B M King, C D Boulter, A J Moore, N L Fazzalari, D S Anson, S Byers, J J Hopwood
Journal: Journal of Clinical Investigation (1996)

We report studies that suggest enzyme replacement therapy will result in a significant reduction in disease progression and tissue pathology in patients with Maroteaux-Lamy syndrome (Mucopolysaccharidosis type VI, MPS VI). A feline model for MPS VI was used to evaluate tissue distribution and clinical efficacy of three forms of recombinant human N-acetylgalactosamine-4-sulfatase (rh4S, EC 3.1.6.1). Intravenously administered rh4S was rapidly cleared from circulation. The majority of rh4S was distributed to liver, but was also detected in most other tissues. Tissue half-life was approximately 2-4 d. Three MPS VI cats given regular intravenous infusions of rh4S for up to 20 mo showed variable reduction of storage vacuoles in Kupffer cells and connective tissues, however cartilage chondrocytes remained vacuolated. Vertebral bone mineral volume was improved in two MPS VI cats in which therapy was initiated before skeletal maturity, and increased bone volume appeared to correlate with earlier age of onset of therapy. One cat showed greater mobility in response to therapy.
New treatment of some chronic tension states.
Authors: W. Sargant, C. J. Walter, N. Wright
Journal: BMJ (1966)

BRITISHnurses.The rate of transmission was between 6 and 10% to 158 newborns cared for in such a way that organisms from infant carriers could be transmitted to them only via the air- borne route.In contrast, 126 infants who were handled by nurses who also handled the neighbouring carrier babies with little or no handwashing exhibited a 43 % rate of acquisition of the carrier strains.A careful hexachlorophane wash of the hands of the nurses who cared for the carriers decreased the transmission rate to 14 %.The carrier strains were often recovered from settling-plates exposed throughout the special nursery.It is concluded that under ordinary circumstances airborne organisms probably account for only a small proportion of staphylococcal transmission in the nursery, and that efficient handwashing techniques can reduce staphylococcal spread appreciably.
Effect of Estrogen on Nitric Oxide and Endothelin-1
Authors: Jerome I. Brody
Journal: Annals of Internal Medicine (1998)

Letters15 July 1998Effect of Estrogen on Nitric Oxide and Endothelin-1Jerome I. Brody, MDJerome I. Brody, MDAllegheny University Hospitals; Philadelphia, PA 19129Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-129-2-199807150-00023 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail TO THE EDITOR:I read with considerable interest the recent article by Best and colleagues [1]. The observation that estrogen replacement increases nitric oxide levels and, at the same time, reduces concentrations of endothelin-1 is in accord with studies we have done [2] that have used immunocytochemistry to show increased levels of in situ endothelin-1 in diseased coronary artery bypass grafts and coronary arteries. Concurrently, again according to immunocytochemistry, the availability of nitric oxide is reduced in diseased coronary arteries and failed bypass grafts. We have indicated a direct relation between locally increased concentrations of endothelin-1, the consummate vasoconstrictor, and ...References1. Best PJ, Berger PB, Miller VM, Lerman A. The effect of estrogen replacement therapy on plasma nitric oxide and endothelin-1 levels in postmenopausal women. Ann Intern Med. 1998; 128:285-8. Google Scholar2. Brody JI, Fink GB, Capuzzi DM. In situ endothelin in coronary artery disease. Angiology. 1996; 47:1027. Google Scholar3. Brody JI, Capuzzi DM, et al. Nitric oxide synthase in coronary artery disease [Abstract]. Presented at the Biomedicine '97 Meeting, 26 April 1997, Washington, D.C. (American Society for Clinical Investigation). Google Scholar Author, Article, and Disclosure InformationAuthors: Jerome I. Brody, MDAffiliations: Allegheny University Hospitals; Philadelphia, PA 19129 PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics 15 July 1998Volume 129, Issue 2Page: 160KeywordsAttentionCoronary arteriesEndothelial cellsEstrogen replacement therapyEstrogensImmunohistochemistryNitric oxideStable coronary artery diseaseVasodilators ePublished: 15 August 2000 Issue Published: 15 July 1998 Copyright & PermissionsCopyright © 1998 by American College of Physicians. All Rights Reserved.PDF downloadLoading ...