tbuckley/cabot-search huggingface dataset:
865. An Ex Vivo Gene Therapy Approach for Metachromatic Leukodystrophy Using Neural Progenitor Cells
Authors: Ken Kawabata, Makoto Migita, Hideki Mochizuki, Yoshitaka Fukunaga, Takashi Shimada
Journal: Molecular Therapy (2004)

Metachromatic leukodystrophy(MLD) is an autosomal recessive inherited disease caused by mutations of the gene encoding the lysozomal enzyme, arylsulfatase A(ASA). Accumulation of the substrate, sulfated glycoprotein, in both the central and peripheral nervous system results in progressive motor and mental deterioration. No effective therapy has been developed yet. Neural progenitor cells are thought to be useful for cell replacement therapy and also cell mediated gene therapy of neurodegenerative diseases. In the present study, we examined the feasibility of ex vivo gene therapy for MLD using neural progenitor cells. Neural progenitor cells (neurospheres) were prepared from the striatum of E14 embryo MLD knockout mice or GFP transgenic mice and transduced with the VSV pseudotyped HIV vector carrying the ASA gene (HIV-ASA). HIV vector transduced progenitor cells retained the potential for differentiation into neurons, astrocytes, and oligodendrocytes in vitro. Expression of ASA in neurospheres transduced with HIV-ASA was confirmed by the spectrophotometric enzyme assay and Western blotting. When neurospheres from GFP mice were transduced with HIV-ASA and inoculated into the brain parenchyma of adult MLD mice, GFP positive cells were detectable one month after injection. Those cells include GFAP positive astrocytes and undifferentiated cells. Immunohistochemistry using anti-ASA antibody demonstrated that ASA was localized in both GFP positive and negative cells, suggesting that ASA was secreted from the transduced cells and taken up by the surrounding cells. These results hold promise for the use of genetically engineered neural stem cells for ex vivo therapy of MLD.
For What Purpose?
Authors: D Servan-Schreiber
Journal: JAMA (2000)

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
Plant MITEs: Useful Tools for Plant Genetics and Genomics
Authors: Ying Feng
Journal: Genomics Proteomics & Bioinformatics (2003)

Abstract MITEs (Miniature inverted-repeat transposable elements) are reminiscence of non-autonomous DNA (class II) elements, which are distinguished from other transposable elements by their small size, short terminal inverted repeats (TIRs), high copy numbers, genic preference, and DNA sequence identity among family members. Although MITEs were first discovered in plants and still actively reshaping genomes, they have been isolated from a wide range of eukaryotic organisms. MITEs can be divided into Tourist-like, Stowaway-like, and pogo-like groups, according to similarities of their TIRs and TSDs (target site duplications). In despite of several models to explain the origin and amplification of MITEs, their mechanisms of transposition and accumulation in eukaryotic genomes remain poorly understood owing to insufficient experimental data. The unique properties of MITEs have been exploited as useful genetic tools for plant genome analysis. Utilization of MITEs as effective and informative genomic markers and potential application of MITEs in plants systematic, phylogenetic, and genetic studies are discussed.
Booked inpatient admissions and hospital capacity
Authors: H. Rogers
Journal: BMJ (2002)

EDITOR—Gallivan et al report a mathematical model of variability of length of stay in an intensive care unit after cardiac surgery.1 The programmes of the NHS modernisation agency use quality improvement methods adapted to health care in England.2 The cornerstones of the approach are patient centredness; mapping the whole process; analysis of true demand and capacity; and redesign through innovation, experimentation, and incremental implementation. The methods are underpinned by the science of complex adaptive systems.3 The NHS is the epitome of a complex adaptive system.

Data on the variability of length of stay are valuable but reflect activity, not demand. Activity is limited by capacity throughout the process. Gallivan et al assumed that length of stay was an independent variable, but length of stay in cardiac intensive care will be affected by a patient's clinical condition and the availability of beds for care afterwards. The dependency of other patients may affect the care given and influence length of stay. Gallivan et al assumed …
Quantitative assessment of retroviral transfer of the human multidrug resistance 1 gene to human mobilized peripheral blood progenitor cells engrafted in nonobese diabetic/severe combined immunodeficient mice
Authors: B. Schiedlmeier, K. Kühlcke, H. G. Eckert, C. Baum, W. J. Zeller, S. Fruehauf
Journal: Blood (2000)
Letter to a Dying Man
Authors: Daniel Thomas Moran
Journal: JAMA (2005)

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
Making Clinical Governance Work for You
Authors: Lindsay F P Smith, Jenny Shepperd
Journal: BMJ (2001)

Ruth Chambers, Gill Wakley 

Radcliffe Medical Press, £19.95, pp 280 ISBN 1 85775 413 1

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Clinical governance is everywhere. Everything that individuals and practices used to do in the name of quality has now been rebadged as clinical governance. Almost anything can apparently be included under the clinical governance umbrella; its range seems unlimited, much like that of general practice itself. All trusts and primary care groups (PCGs) have a statutory …

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Activation of HIF-1α and LL-37 by commensal bacteria inhibits Candida albicans colonization
Authors: Di Fan, Laura A Coughlin, Megan M Neubauer, Jiwoong Kim, Min Soo Kim, Xiaowei Zhan, Tiffany R Simms-Waldrip, Yang Xie, Lora V Hooper, Andrew Y Koh
Journal: Nature Medicine (2015)
Long-term haemodynamic effects of a 4-week regimen of nipradilol, a new β-blocker with nitrovasodilating properties, in patients with portal hypertension due to cirrhosis
Authors: Takumi Aramaki, Tatsuya Sekiyama, Yasumi Katsuta, Hiroshi Kurokawa, Hirokazu Komeichi, Hitoshi Tsutsui, Hideto Terada, Masaru Ohsuga, Katsuaki Satomura, Hidemasa Okumura
Journal: Journal of Hepatology (1992)
Renal sympathetic denervation in patients with treatment-resistant hypertension (The Symplicity HTN-2 Trial): a randomised controlled trial
Authors: Murray D. Esler, Henry Krum, Paul A. Sobotka, Markus P. Schlaich, Roland E. Schmieder, Michael Böhm, Felix Mahfoud, Horst Sievert, Nina Wunderlich, Lars Christian Rump, Oliver Vonend, Michael Uder, Mel Lobo, Mark Caulfield, Andrejs Erglis, Michel Azizi, Marc Sapoval, Suku Thambar, Alexandre Persu, Jean Renkin, Heribert Schunkert, Joachim Weil, Uta C. Hoppe, Tony Walton, Dierk Scheinert, Thomas Binder, Andrzej Januszewicz, Adam Witkowski, Luis M. Ruilope, Robert Whitbourn, Heike Bruck, Mark Downes, Thomas F. Lüscher, Alan G. Jardine, Mark W. Webster, Thomas Zeller, Jerzy Sadowski, Krzysztof Bartus, Craig A. Straley, Neil C. Barman, David P. Lee, Ronald M. Witteles, Vivek Bhalla, Joseph M. Massaro
Journal: The Lancet (2010)
Statistics Notes: Comparing several groups using analysis of variance
Authors: D. G Altman, J M. Bland
Journal: BMJ (1996)

<h3>Background</h3> Despite the widespread use of accreditation in many countries, and prevailing beliefs that accreditation is associated with variables contributing to clinical care and organisational outcomes, little systematic research has been conducted to examine its validity as a predictor of healthcare performance. <h3>Objective</h3> To determine whether accreditation performance is associated with self-reported clinical performance and independent ratings of four aspects of organisational performance. <h3>Design</h3> Independent blinded assessment of these variables in a random, stratified sample of health service organisations. <h3>Settings</h3> Acute care: large, medium and small health-service organisations in Australia. <h3>Study participants</h3> Nineteen health service organisations employing 16 448 staff treating 321 289 inpatients and 1 971 087 non-inpatient services annually, representing approximately 5% of the Australian acute care health system. <h3>Main measures</h3> Correlations of accreditation performance with organisational culture, organisational climate, consumer involvement, leadership and clinical performance. <h3>Results</h3> Accreditation performance was significantly positively correlated with organisational culture (rho=0.618, p=0.005) and leadership (rho=0.616, p=0.005). There was a trend between accreditation and clinical performance (rho=0.450, p=0.080). Accreditation was unrelated to organisational climate (rho=0.378, p=0.110) and consumer involvement (rho=0.215, p=0.377). <h3>Conclusions</h3> Accreditation results predict leadership behaviours and cultural characteristics of healthcare organisations but not organisational climate or consumer participation, and a positive trend between accreditation and clinical performance is noted.
The effects of experimental skin damage in patients with lichen planus
Authors: L. STANKLER, S.W.B. EWEN
Journal: British Journal of Dermatology (2006)

Experimental scratching induced phloxinophilic eosinophilic bodies in unaffected skin of ten patients with active lichen planus. This reaction was inhibited by concomitant local injection of methylprednisolone acetate and could not be induced in patients with inactive lichen planus, in patients with other dermatoses, nor in healthy subjects. The findings are consistent with a viral aetiology for lichen planus.
Abstract 19062: Outcomes of Patients with Acute Type B (DeBakey III) Aortic Dissection: a 13 Year Single Center Experience
Authors: Rana O Afifi, Harleen K Sandhu, Samuel S Leake, Varsha Kumar, Ali Azizzadeh, Kristofer M Charlton-Ouw, Tom C Nguyen, Charles C Miller, Hazim J Safi, Anthony L Estrera
Journal: Circulation (2014)

Introduction: Thoracic endovascular aortic repair (TEVAR) has emerged as a treatment option for patients with acute type B aortic dissection (ATBAD). The aim of this study is to evaluate the long-term outcomes of patients with ATBAD. Methods: We reviewed patients with ATBAD between 2001 and 2014, analyzing patient variables, in-hospital complications and long-term outcomes based on status (complicated (c) vs uncomplicated (u)) and treatment modalities. Long term outcomes were analyzed using Kaplan-Meier and log-rank statistics. Results: We treated 442 patients with ATBAD. Mean age was 60.5±14.1 and 165 (37.3%) females. Of these 292 (66.1%) had uATBAD and were treated medically, while 150 (33.9%) had cATBAD. Of the cATBAD patients 56 (37.3%) were treated medically, 52 (34.7%) with open surgery, 29(19.3%) with TEVAR, and 13 (8.7%) with other open vascular procedures. Hospital mortality and morbidity were significantly related to treatment group (table 1). Median follow-up time was 4.4 years (range 0-13.4). ...
Activation of the COX2-PGE2 axis by immune effector cells results in the self-limiting nature of type-1 immunity in cancer tissues
Authors: Jeffrey L Wong, Natasa Obermajer, Ravikumar Muthuswamy, Robert P Edwards, Kunle Odunsi, David L Bartlett, Pawel Kalinski
Journal: Journal for ImmunoTherapy of Cancer (2014)

Meeting abstracts Type-1 immune responses, mediated by IFNγ and TNFα-producing CTLs, Th1, and NK cells, are essential for effective anti-tumor immunity. Despite recent advances in the induction and stabilization of these responses by cancer immunotherapies, the clinical success of these
Overexpression of Pim-1 and Myc accelerates genomic instability
Authors: Meejeon Roh, Chisu Song, Bernard Gary, Sarki A. Abdulkadir
Journal: Cancer Research (2005)

2807   Pim-1 is an oncogenic kinase that cooperates with c-Myc in oncogenesis. We have previously shown that Pim-1 overexpression can induce genomic instability in prostate epithelial cells. c-Myc expression also has been shown to induce genomic instability when p53 function is lost in 32D cells, which is a diploid murine myeloid cell line. To determine whether Pim-1 and c-Myc can cooperate to induce genomic instability, we estabilished stable prostate epithelial cell lines that overexpress both Pim-1 and c-Myc. There were no significant differences in cell proliferation or apoptosis in any of these cell lines even with stimulus such as TRAIL. However cell cycle profile analysis by FACS showed dramatic cooperation in induction of polyploidy between Pim-1 and c-Myc. Either Pim-1 or Myc alone induced modest increase of polyploidy cells (25.24%, 34.91% of >4N cells respectively) compared to control cell line (18.72%). However when Pim-1 and Myc were overexpressed simultaneously, the percentage of polyploidy cells was increased to 68.66%. We found that cyclin E expression level is significantly increased, and Cdc25C expression level is decreased in Myc overexpressing cells. We also found out that expression level of cyclin B1 is significantly increased in Pim-1 overexpressing cells. This increase is observed in early passage cells, suggesting that it is involved in genomic instability. Together our data suggest that Pim-1 and Myc can accelerate genomic instability by affecting different stage of cell cycles; Pim-1 affect G2/M phase, and Myc affect G1 /S phase
THE TECHNIC AND DIAGNOSTIC VALUE OF ASPIRATION OF BONE MARROW FROM THE ILIAC CREST
Authors: M A RUBINSTEIN
Journal: Annals of Internal Medicine (1950)

Article1 June 1950THE TECHNIC AND DIAGNOSTIC VALUE OF ASPIRATION OF BONE MARROW FROM THE ILIAC CRESTMICHAEL A. RUBINSTEIN, M.D.MICHAEL A. RUBINSTEIN, M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-32-6-1095 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptIn the spring of 1943, I saw a patient with roentgenologic evidence of a single metastatic lesion in the iliac bone, in whom the primary site of malignancy could not be established. In order to recover the neoplastic cells and possibly to identify them, I attempted aspiration of the iliac lesion. The aspiration proved easy to perform. However, in this particular case no neoplastic cells were recovered, but instead normal bone marrow was seen. Since that time my associates and I have performed iliac aspirations systematically on almost every patient of hematological interest admitted to the Montefiore Hospital.The aim... This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: New York, N. Y.*Presented at the Thirtieth Annual Session, American College of Physicians, March 29, 1949, New York, N. Y.From the Medical Division, Montefiore Hospital, New York, N. Y. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThe history of bone marrow in orthopaedic surgery (part I trauma): trepanning, bone marrow injection in damage control resuscitation, and bone marrow aspiration to heal fracturesSafe and successful bone marrow biopsy: An anatomical and CT-based cadaver studyTrepanning or trephines: a history of bone marrow biopsyBibliography Articles Cited in CommentariesNew sternal puncture needle.A comparison of bone-marrow aspiration and skeletal roentgenograms in the diagnosis of metastatic carcinoma 1 June 1950Volume 32, Issue 6Page: 1095-1098KeywordsBoneBone marrowCellsHematology and oncologyLesions Issue Published: 1 June 1950 PDF downloadLoading ...
Abstract 19722: Pericardial Mass in a Patient With Rheumatoid Arthritis: A Case Report
Authors: Mohammad A Al-Ani, Michelle H Weber, Alexandra R Lucas, Matthew D Kosboth
Journal: Circulation (2014)

Introduction: Rheumatoid arthritis (RA) can affect the heart via either inflammatory or ischemic processes. Pericardial disease is common in RA patients (30-50%) but is usually subclinical and not hemodynamically significant unless accompanied by infiltrative cardiomyopathy which carries a poor prognosis. Case report: A 65 year-old Caucasian male presented with long standing RA, severe fatigue and mild arthritis of MCP joints. Physical examination indicated S3, II/VI decrescendo diastolic murmur, and 2+ LL edema. ESR was normal but anti-CCP antibodies were &gt; 250 units. On echocardiogram, a large pericardial mass was detected without atrial nor ventricular collapse and mild-moderate aortic regurgitation. Cardiac MRI defined the mass as a heterogeneous entity attached to the right, anterior, and inferior borders of the heart with compression of the right atrium, left and right ventricles, and the tricuspid valve. CT guided biopsy demonstrated fibrinous material without granulomas nor infection. Fatigue was initially attributed to uncontrolled RA and low dose prednisone and Leflunamide were started. 3 months later, he developed frank heart failure with NYHA class IIIb functional impairment. Cardiac Tamponade was confirmed by heart catheterization demonstrating typical 4-chamber pressure equalization. The mass was surgically excised with partial pericardiectomy. The patient had a dramatic improvement and 4 years later, he remains asymptomatic. Discussion: Our patient presented with uncontrolled RA and a pericardial mass that was not hemodynamically significant per the initial echocardiogram. Despite intensifying immunosuppression, his fatigue progressed with signs of congestive heart failure. Tamponade physiology was diagnosed invasively and the mass was resected with excellent recovery. Microscopically, the lesion consisted of a fibrous and exudative material. The patient likely had multiple asymptomatic episodes of pericarditis, gradually leading to this condition. We demonstrate with this case that RA pericardial disease can cause hemodynamic compromise while sparing myocardium and valves and that surgery is associated with very good prognosis. Physicians should be vigilant about a cardiac cause of fatigue in RA patients.
Transgrediens pachyonychia congenita (PC): case series of a nonclassical PC presentation
Authors: K. Harris, P.R. Hull, C.D. Hansen, F.J.D. Smith, W.H.I. McLean, J.L. Arbiser, S.A. Leachman
Journal: British Journal of Dermatology (2011)

Pachyonychia congenita (PC) is a rare keratin disorder that typically presents with nail dystrophy and focal plantar keratoderma. We present seven cases of PC with transgrediens involvement of the dorsal feet.To document the extension of their disease to the dorsum of the feet in patients with mutation-confirmed PC, to report the natural history of PC with such transgrediens involvement, to generate hypotheses regarding aetiology, and to suggest prevention and treatment modalities.Genetically confirmed cases of PC with transgrediens foot involvement were verified through the International Pachyonychia Congenita Research Registry (IPCRR) and characterized via telephone survey and photography.Seven patients with PC in the IPCRR were confirmed to have transgrediens lesions on the dorsal feet (six KRT6A mutations; one KRT16 mutation). Six cases had pre-existing nontransgrediens keratoderma and all cases reported standing, wearing shoes, foot moisture, and/or infection as exacerbating or predisposing factors. Improvement, reported in six cases, was attributed to use of antibiotics or gentian violet, or improved footwear.Transgrediens involvement of the dorsal feet is a rare manifestation of mutation-confirmed PC and may be more common in patients who carry a KRT6A mutation. Trauma, friction, infection and wound healing may exacerbate or predispose toward transgrediens lesions. It remains to be proven whether transgrediens-associated infection is causal or represents a primary or secondary process. Patients with PC who develop transgrediens lesions may benefit from fungal and bacterial cultures, followed by appropriate antimicrobial treatments. Efforts to decrease skin friction and moisture may also improve and/or prevent transgrediens spread.
Medicine for the Practicing Physician.
Authors: J. Willis Hurst
Journal: Annals of Internal Medicine (1984)

The Purpose of this Book and How to Use It. Behavioural Problems. Neoplastic Diseases. Nutritional Disorders. Immunologic and Allergic Disorders. Joint and Muscle Disorders. Connective Tissue Disease and The Vasculitides. Infectious Disease Problems. Endocrine and Metabolic Disorders. Common Gynaecologic Disorders. Dermatologic Problems. Haematologic Disorders. Pulmonary Disorders. Hypertension. Cardiovascular Problems. Renal Disorders. Abnormalities of Inorganic Metabolism. Disorders of the Genitourinary System. Disorders of the Gastrointestinal System and the Liver. Neurological Disorders. Disorders of the Ears, Nose and Throat. Common Eye Disorders. Environmental Emergencies. Drug Abuse and Poisons.
A patient who changed my life twice: What medicine's all about
Authors: J. S Tobias
Journal: BMJ (2000)

It may have been obvious to her at the time, but somehow I did not quite like to admit to Sylvia that she was my very first—patient, that is. I had just finished my preclinical training at Cambridge and had come down to London to walk the wards at Bart's.

Sylvia, on the other hand, was much more experienced. She had myasthenia gravis and was barely older than me but was already a veteran of the intensive care unit, and by now a pale, frail, frightened shadow of her former self, with a tracheostomy. Weighing less than 40 kg, just …
A new recurrent translocation, t(5;11)(q35;p15.5), associated with del(5q) in childhood acute myeloid leukemia. The UK Cancer Cytogenetics Group (UKCCG)
Authors: Rina J. Jaju, Oskar A. Haas, Michael Neat, Jochen Harbott, Vaskar Saha, Jacqueline Boultwood, Jill M. Brown, Hendrati Pirc-Danoewinata, Bernd W. Krings, Ulrich Müller, Stephan W. Morris, James S. Wainscoat, Lyndal Kearney
Journal: Blood (1999)