tbuckley/cabot-search huggingface dataset:
The SARS epidemic: emergence of a new respiratory virus?
Authors: T Seemungal
Journal: Thorax (2003)

▴ Lee N, Hui D, Wu A, et al . A major outbreak of severe acute respiratory syndrome in Hong Kong. N Engl J Med2003;348 (epub ahead of print) This clinical study of 138 patients from a Hong Kong hospital suggests that the severity of the severe acute respiratory syndrome (SARS) (intensive care and/or death) was associated with advanced age, high peak lactate dehydrogenase level (LDH), and an absolute neutrophil count that exceeded the normal range at presentation. 78% of patients had consolidation on the chest radiograph at initial consultation. ▴ Ksiazek TG, Erdman D, Goldsmith CS, et al . A novel coronavirus associated with severe acute respiratory syndrome. N Engl J Med2003;348:1947–58 [OpenUrl][1][CrossRef][2][PubMed][3][Web of Science][4] Nineteen inpatients (6 in Hong Kong, 1 in Canada, 10 in Vietnam, 1 in Taiwan, 1 not stated) with SARS were identified as being infected with the SARS coronavirus determined by … [1]: {openurl}?query=rft.jtitle%253DNew%2BEngland%2BJournal%2Bof%2BMedicine%26rft.stitle%253DNEJM%26rft.issn%253D0028-4793%26rft.aulast%253DWenzel%26rft.auinit1%253DR.%2BP.%26rft.volume%253D348%26rft.issue%253D20%26rft.spage%253D1947%26rft.epage%253D1948%26rft.atitle%253DManaging%2BSARS%2Bamidst%2BUncertainty%26rft_id%253Dinfo%253Adoi%252F10.1056%252FNEJMp030072%26rft_id%253Dinfo%253Apmid%252F12748313%26rft.genre%253Darticle%26rft_val_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Ajournal%26ctx_ver%253DZ39.88-2004%26url_ver%253DZ39.88-2004%26url_ctx_fmt%253Dinfo%253Aofi%252Ffmt%253Akev%253Amtx%253Actx [2]: /lookup/external-ref?access_num=10.1056/NEJMp030072&link_type=DOI [3]: /lookup/external-ref?access_num=12748313&link_type=MED&atom=%2Fthoraxjnl%2F58%2F7%2F604.atom [4]: /lookup/external-ref?access_num=000182823400001&link_type=ISI
Misdiagnosis of Fabry disease: importance of biochemical confirmation of clinical or pathological suspicion
Authors: G.E. Linthorst, M.A. De Rie, K.H. Tjiam, J.M.f.G. Aerts, K.P. Dingemans, C.E.M. Hollak
Journal: British Journal of Dermatology (2004)

Generalized angiokeratoma are associated with three lysosomal storage disorders, one of which is Fabry disease (α‐galactosidase A deficiency). Treatment for Fabry disease with supplementation of recombinant enzyme is available in the European Union and subsequently physicians' awareness may rise. A patient who was erroneously diagnosed with Fabry disease is presented.
CREATININE CLEARANCE IN OSTEOMALACIA
Authors: A.J. Williams, J.N. Barnes, V. Fonseca, D.P. Mikhailidis, P. Dandona, G. Decaux, F. Delwiche, P.A. Lucas, J.H. Meadows, G.A. Coles, R.C. Brown
Journal: The Lancet (1984)
Inhaler devices for asthma: a call for action in a neglected field
Authors: A. Papi, J. Haughney, J.C. Virchow, N. Roche, S. Palkonen, D. Price
Journal: European Respiratory Journal (2011)

The Brussels Declaration, published in the European Respiratory Journal in 2008 1, recognises the high prevalence of patients with poorly controlled asthma and calls for changes in asthma management across Europe. Prescribing an appropriate inhaler device for asthma, a device that the patient accepts and can handle correctly, is one key element in this process. Inhaler mishandling is very common in real-world clinical practice and can contribute to poor asthma control 2–5.

The International Primary Care Respiratory Group (IPCRG) is committed to identifying reasons for poor asthma control and to promoting interventions to help patients achieve asthma control 6–9. An international panel of healthcare providers (HCPs), academics and a patient representative was convened under the auspices of IPCRG to discuss and challenge the science behind inhaler therapy, and to propose practical solutions to real-life problems related to inhaler choice and mishandling. The focus was on the problems confronting clinicians in prescribing a suitable inhaler for each individual and those confronting patients in using their inhalers.

Until recently, inhaler therapy and devices have been marginal topics of clinical investigation and research in the field of asthma, mainly confined to a limited circle of experts, and lacking evidence for practical application. Thus, we propose this call for action, given: 1) the importance of inhaler technique for effective inhaled therapy; 2) the critical gaps in knowledge that need still to be addressed; and 3) the lack of solid evidence supporting HCPs in making clinical decisions regarding inhalers for asthma treatment.

Correct inhaler technique is fundamental for effective inhaled therapy. Errors in inhaler technique are very common 10–13 and can impact drug delivery to the lungs, thereby compromising bronchodilation in the short term and asthma control in the long term, depending on the drug considered …
Excellent Agreement Between Dermatology and Pediatric Researchers In Severity Scoring Of Atopic Dermatitis (SCORAD) Index In Children
Authors: Rodrigo Hoyos Bachiloglu, Cristian Navarrete, Cristian Vera, Sergio Silva, Lorena Cifuentes, Carolina Iturriaga, Francisca Cristi, Arturo Borzutzky
Journal: Journal of Allergy and Clinical Immunology (2014)
Accuracy of reporting of family history of colorectal cancer
Authors: R J Mitchell, D Brewster, H Campbell, M E M Porteous, A H Wyllie, C C Bird, M G Dunlop
Journal: Gut (2004)

<b>Background and aims:</b> Family history is used extensively to estimate the risk of colorectal cancer but there is considerable potential for recall bias and inaccuracy. Hence we systematically assessed the accuracy of family history reported at interview compared with actual cancer experience in relatives. <b>Methods:</b> Using face to face interviews, we recorded family history from 199 colorectal cancer cases and 133 community controls, totalling 5637 first and second degree relatives (FDRs/SDRs). We linked computerised cancer registry data to interview information to determine the accuracy of family history reporting. <b>Results:</b> Cases substantially underreported colorectal cancer arising both in FDRs (sensitivity 0.566 (95% confidence interval (CI) 0.433, 0.690); specificity 0.990 (95% CI 0.983, 0.994)) and SDRs (sensitivity 0.271 (95% CI 0.166, 0.410); specificity 0.996 (95% CI 0.992, 0.998)). There was no observable difference in accuracy of reporting family history between case and control interviewees. Control subjects similarly underreported colorectal cancer in FDRs (sensitivity 0.529 (95% CI 0.310, 0.738); specificity 0.995 (95% CI 0.989, 0.998)) and SDRs (sensitivity 0.333 (95% CI 0.192, 0.512); specificity 0.995 (95% CI 0.991, 0.995)). To determine practical implications of inaccurate family history, we applied family history criteria before and after record linkage. Only two of five families reported at interview to meet surveillance criteria did so after validation, whereas only two of six families that actually merited surveillance were identified by interview. <b>Conclusions:</b> This study has quantified the inaccuracy of interview in identifying people at risk of colorectal cancer due to a family history. Colorectal cancer was substantially underreported and so family history information should be interpreted with caution. These findings have considerable relevance to identifying patients who merit surveillance colonoscopy and to epidemiological studies.
Human-specific epigenetic variation in the immunological Leukotriene B4 Receptor (LTB4R/BLT1) implicated in common inflammatory diseases
Authors: Gareth A Wilson, Lee M Butcher, Holly R Foster, Andrew Feber, Christian Roos, Lutz Walter, Grzegorz Woszczek, Stephan Beck, Christopher G Bell
Journal: Genome Medicine (2014)

Common human diseases are caused by the complex interplay of genetic susceptibility as well as environmental factors. Due to the environment's influence on the epigenome, and therefore genome function, as well as conversely the genome's facilitative effect on the epigenome, analysis of this level of regulation may increase our knowledge of disease pathogenesis.In order to identify human-specific epigenetic influences, we have performed a novel genome-wide DNA methylation analysis comparing human, chimpanzee and rhesus macaque.We have identified that the immunological Leukotriene B4 receptor (LTB4R, BLT1 receptor) is the most epigenetically divergent human gene in peripheral blood in comparison with other primates. This difference is due to the co-ordinated active state of human-specific hypomethylation in the promoter and human-specific increased gene body methylation. This gene is significant in innate immunity and the LTB4/LTB4R pathway is involved in the pathogenesis of the spectrum of human inflammatory diseases. This finding was confirmed by additional neutrophil-only DNA methylome and lymphoblastoid H3K4me3 chromatin comparative data. Additionally we show through functional analysis that this receptor has increased expression and a higher response to the LTB4 ligand in human versus rhesus macaque peripheral blood mononuclear cells. Genome-wide we also find human species-specific differentially methylated regions (human s-DMRs) are more prevalent in CpG island shores than within the islands themselves, and within the latter are associated with the CTCF motif.This result further emphasises the exclusive nature of the human immunological system, its divergent adaptation even from very closely related primates, and the power of comparative epigenomics to identify and understand human uniqueness.
Granulocytic Sarcoma in a Patient With Blast Crisis Mimicking a Chronic Subdural Hematoma
Authors: Caitlin E. O'Brien, Amanda M. Saratsis, Jean-Marc Voyadzis
Journal: Journal of Clinical Oncology (2011)

Case ReportA 34-year-old black man was admitted to the neurosurgical intensive care unit with a 1-day history of progressive weakness and decreased mental status.He was known to the neurosurgical service, having presented 1 week prior for evaluation of persistent headaches for 1 month.At that time, he was diagnosed with bilateral chronic subdural hematomas and observed in the neurosurgical unit for 1 week, after which he left against medical advice.He had no history of head trauma, anticoagulation, or thrombocytopenia.His medical history was notable for chronic myelogenous leukemia (CML) diagnosed at our institution 10 months prior, for which he was receiving imatinib 600 mg daily.The patient had a known history of poor adherence to his leukemia management.On admission, the patient was intubated.He opened his eyes to voice.The right pupil was 4 mm and minimally reactive, and the left pupil was 3 mm and sluggishly reactive.He followed simple commands, moved all extremities purposefully against gravity, and had no additional focal neurologic deficits.The remainder of the physical examination was notable only for splenomegaly.A noncontrast head computed tomography (CT) scan revealed a hypodense right frontotemporal subdural collection measuring 1 cm, a small hypodense left subdural collection measuring 5 mm, and 7 mm of right-to-left midline shift (Fig 1A).Both lesions were interpreted as chronic subdural hematomas.Complete blood count revealed leukocytosis (247.9K/CCM) consisting of 30% metamyelocytes, 25% myelocytes, 16% blastocytes, and 3% promyelocytes; anemia (hemoglobin of 10.3 g/dL); and normal platelets (138 K/CMM).The patient's international
PNEUMO-ENCEPHALOGRAPHY IN EPILEPSY
Authors: WILLARD W. DICKERSON
Journal: American Journal of Psychiatry (1941)

We have presented a partial review of the literature dealing with encephalographic studies of groups of epileptics, together with a summary of our experience with encephalography in 286 institutionalized epileptic patients. From our studies we believe that we may draw the following conclusions: 1. Encephalography is a valuable and relatively safe procedure in the study of epileptic patients. 2. The value of this procedure has been considered from the standpoint of diagnosis, prognosis, and treatment. A. There is no lesion characteristic of epilepsy as revealed by encephalography. The structural changes revealed by this method cannot be differentiated with regard to etiologic agent, nor from similar changes in non-epileptic individuals. These changes in themselves are not an essential part of the convulsive disorder, but may be regarded as concomitant or as a result of the same process giving rise to the epileptic manifestations. Encephalography has revealed the presence of lesions in some patients which were not indicated from the history or by the physical and neurological examinations. The relatively frequent occurrence of incomplete drainage and failure of the ventricles to fill with air in the symptomatic groups may indicate that this phenomenon may be of more significance as evidence of abnormality than was formerly believed. B. The most valuable aspect of encephalography is that of prognosis. The presence of irreversible changes in structure is convincing evidence that often little can be done for the patient other than to render his existence as comfortable as possible. C. Encephalography is of little value as a therapeutic measure in epilepsy. Only 2 of 122 patients showed complete remission from seizures for a 6-months' period. A relatively small number were improved, a slightly larger number made worse, and the greatest number unchanged. We know of no way by which patients may be selected for the therapeutic effect of this procedure.
The art versus the science of medicine. Are clinical practice guidelines the answer?
Authors: M. E SUAREZ-ALMAZOR, A. S RUSSELL
Journal: Annals of the Rheumatic Diseases (1998)

Clinical practice guidelines (CPGs) are systematically created recommendations to assist physicians in the decision making processes related to the choice of health interventions.1 2 There has been increasing interest in the development of CPGs within all medical fields. In 1993 there were 374 publications indexed by MEDLINE as pertaining to CPGs; by 1996, the number had increased to over 980. The American Medical Association has listed in their “Directory of Medical Parameters” over 1600 sets of guidelines.3 The enthusiasm and frenzy are such that “Guidelines for the development of guidelines” have been proposed.4 The American College of Rheumatology has also developed several sets of guidelines covering areas such as osteoarthritis, rheumatoid arthritis, and steroid induced osteoporosis.5-11 

Variation in practice has been reported for most fields in medicine, including musculoskeletal diseases, often driven by physician preferences or practice characteristics. For instance, prescriptions of second line drugs for patients with rheumatoid arthritis vary among rheumatologists.12 13 Demographic characteristics of the prescribing physician seem to have more impact in the selection of specific second line agents than the clinical characteristics of the patient.13 Similar variation has been seen at the primary care level and other specialties for osteoarthritis and low back pain.14-18 In one study where outcomes were reported patients with low back pain treated by “high prescriber” family physicians had the same outcomes as patients treated by “low prescribers”, but much higher medical costs.17 In this age of shrinking budgets and limited resources practice variation may be considered inappropriate not only when resulting in differences in outcomes (benefits or risks) but also when substantial economic costs arise from interventions with unclear advantages.

Where there is no clear evidence favouring the use of one drug over another, practice variation may be readily understandable. Physicians frequently …
IDH1 mutations are present in the majority of common adult gliomas but rare in primary glioblastomas
Authors: Koichi Ichimura, Danita M. Pearson, Sylvia Kocialkowski, L. Magnus Bäcklund, Raymond Chan, David T.W. Jones, V. Peter Collins
Journal: Neuro-Oncology (2009)

We screened exon 4 of the gene isocitrate dehydrogenase 1 (NADP+), soluble (IDH1) for mutations in 596 primary intracranial tumors of all major types. Codon 132 mutation was seen in 54% of astrocytomas and 65% of oligodendroglial tumors but in only 6% of glioblastomas (3% of primary and 50% of secondary glioblastomas). There were no mutations in any other type of tumor studied. While mutations in the tumor protein p53 gene (TP53) and total 1p/19q deletions were mutually exclusive, IDH1 mutations were strongly correlated with these genetic abnormalities. All four types of mutant IDH1 proteins showed decreased enzymatic activity. The data indicate that IDH1 mutation combined with either TP53 mutation or total 1p/19q loss is a frequent and early change in the majority of oligodendroglial tumors, diffuse astrocytomas, anaplastic astrocytomas, and secondary glioblastomas but not in primary glioblastomas.
Association between vitamin D receptor, CCR5, TNF-α and TNF-β gene polymorphisms and HBV infection and severity of liver disease
Authors: Pothakamuri Venkata Suneetha, Shiv Kumar Sarin, Ankur Goyal, Gollapudi Tharun Kumar, Deepak Kumar Shukla, Syed Hissar
Journal: Journal of Hepatology (2006)
Effects of Intracellular H+ on the Electrical Properties of Excitable Cells
Authors: W Moody
Journal: Annual Review of Neuroscience (1984)

The brain's default mode network consists of discrete, bilateral and symmetrical cortical areas, in the medial and lateral parietal, medial prefrontal, and medial and lateral temporal cortices of the human, nonhuman primate, cat, and rodent brains. Its ...Read More
Effects of adrenaline on a compartment of slowly-exchangeable calcium in the perfused rat heart
Authors: S. V LAMONT, G. J BARRITT
Journal: Cardiovascular Research (1983)

The effects of adrenaline on kinetically-distinct compartments of exchangeable calcium in the spontaneously-beating perfused rat heart were investigated under steady-state conditions using a 45Ca2+ outflow technique together with a nonlinear least-squares curve fitting procedure and 51Cr-EDTA to monitor the loss of freely-diffusible Ca2+ from the extracellular space. In addition to Ca2+ distributed in the vascular (compartment 1) and interstitial (compartment 2) spaces, the minimum number of kinetically-distinct compartments of cellular exchangeable Ca2+ (which include Ca2+ bound to extracellular sites on the sarcolemma) required to fit the data (compartments 3,4 and 5) was three. A system in which cellular compartment 3 is linked to the vascular space and compartments 4 and 5 are linked to the interstitial space was consistent with the data. For hearts perfused under control conditions, the fractional transfer rates for Ca2+ outflow from cellular compartments 3, 4 and 5 were 0.70, 0.11 and 0.017 min−1, respectively. Adrenaline increased the quantity of exchangeable Ca2+ in compartment 5 and decreased that present in the compartment 4. Evidence that compartment 5 includes mitochondrial exchangeable Ca2+ is discussed. It is concluded that one of the actions of adrenaline on the heart is to increase the quantity of exchangeable Ca2+ in the mitochondria.
A disease-related rheumatoid factor autoantibody is not tolerized in a normal mouse: implications for the origins of autoantibodies in autoimmune disease.
Authors: L G Hannum, D Ni, A M Haberman, M G Weigert, M J Shlomchik
Journal: The Journal of Experimental Medicine (1996)

We have analyzed B cell tolerance in a rheumatoid factor (RF) transgenic mouse model. The model is based on AM14, a hybridoma, originally isolated from an autoimmune MRL/lpr mouse that has an affinity and specificity typical of disease-related RFs from this strain. AM14 binds to immunoglobulin (Ig)G2a of the "a" allotype (IgG2aa) and not to IgG2ab. Thus, by crossing the transgenes onto an IgHa (BALB/c) background or to a congenic IgHb (CB.17) background, we could study the RF-expressing B cells when they were self-specific (IgHa) or when they were not self-specific (IgHb). These features make the AM14 model unique in focusing on a true autoantibody specificity while at the same time allowing comparison of autoreactive and nonautoreactive transgenic B cells, as was possible in model autoantibody systems such as anti-hen egg lysozyme. Studies showed that AM14 RF B cells can make primary immune responses and do not downregulate sIgM, indicating that the presence of self-antigen does not induce anergy of these cells. In fact, IgHa AM14 transgenic mice have higher serum levels of transgene-encoded RF than their IgHb counterparts, suggesting that self-antigen-specific activation occurs even in the normal mouse background. Since AM14 B cells made primary responses, we had the opportunity to test for potential blocks to self-reactive cells entering the memory compartment. We did not find evidence of this, as AM14 B cells made secondary immune responses as well. These data demonstrate that a precursor of a disease-specific autoantibody can be present in the preimmune repertoire and functional even to the point of memory cell development of normal mice. Therefore, immunoregulatory mechanisms that normally prevent autoantibody production must exert their effects later in B cell development or through T cell tolerance. Conversely, the data suggest that it is not necessary to break central tolerance, even in an autoimmune mouse, to generate pathologic, disease-associated autoantibodies.
Factors influencing the normalization of CD4+ T-cell count, percentage and CD4+/CD8+ T-cell ratio in HIV-infected patients on long-term suppressive antiretroviral therapy
Authors: C. Torti, M. Prosperi, D. Motta, S. Digiambenedetto, F. Maggiolo, G. Paraninfo, D. Ripamonti, G. Cologni, M. Fabbiani, S.L. Caputo, L. Sighinolfi, N. Ladisa, I. El-Hamad, E. Quiros-Roldan, I. Frank
Journal: Clinical Microbiology and Infection (2011)
HRCT lung abnormalities are not a surrogate for exercise limitation in bronchiectasis
Authors: E.A. Edwards, I. Narang, A. Li, D.M. Hansell, M. Rosenthal, A. Bush
Journal: European Respiratory Journal (2004)

In paediatric bronchiectasis, there has been limited work on the relationship between disease severity as assessed by exercise limitation and high resolution computed tomography (HRCT). An observational study was performed on 36 children who completed a questionnaire, physical examination, spirometry and sputum analysis, followed by a low dose of radiation protocol chest computed tomography (CT) scan and cycle ergometry incremental exercise test. A modified Bhalla score was used to score the HRCT abnormalities. The exercise variables used to assess functional status were heart rate, oxygen consumption, oxygen saturations and time of exercise. The results were compared with established normal paediatric values. The median (range) age of the children was 13 yrs (10.6–17.1). Age, sex, height, weight or pubertal status were equally distributed between the children with cystic fibrosis (CF) or non-CF bronchiectasis. The children with non-CF bronchiectasis had a lower median forced expiratory volume in one second % predicted than the children with CF (69% versus 76%, respectively). The distribution of lung disease differed between the two groups. The children with CF bronchiectasis had predominantly right upper lobe disease, and scored higher for the presence and extent of bronchiectasis. Otherwise, there was no statistical difference in the overall CT score or the individual CT parameters between the groups. There was evidence of exercise limitation in both CF and non-CF bronchiectasis compared to normal children. No consistent relationships were identified between the lung function parameters, HRCT findings or exercise parameters in either disease group. In this study, high resolution computed tomography features were not found to be markers of exercise capacity. As spirometry, high resolution computed tomography features and exercise testing give different information in children with cystic fibrosis and non-cystic fibrosis bronchiectasis, the current authors suggest all three may be required for the comprehensive assessment of respiratory status.