tbuckley/cabot-search huggingface dataset:
Paget's Disease of the Mandible
Authors: Mayank B Patel, Ashok Bhalla
Journal: New England Journal of Medicine (2008)

A 55-year-old man presented with a 2-year history of painful jaw enlargement and progressively ill-fitting dentures. He had no headaches or visual-field defects and did not have hyperhidrosis, oily skin, glucose intolerance, heart failure, or an increase in glove or shoe size. The entire mandible was enlarged bilaterally to the angle of the jaw (Panels A and B), with marked misalignment of the upper and lower teeth. The serum level of insulin-like growth factor I was normal at 15.2 nmol per liter (normal range, 9 to 40), but levels of serum alkaline phosphatase and bone-specific alkaline phosphatase were elevated (154 . . .
The SV2 variant of KLF6 is down-regulated in hepatocellular carcinoma and displays anti-proliferative and pro-apoptotic functions
Authors: Naïma Hanoun, Christophe Bureau, Thoria Diab, Odile Gayet, Nelson Dusetti, Janick Selves, Jean-Pierre Vinel, Louis Buscail, Pierre Cordelier, Jérôme Torrisani
Journal: Journal of Hepatology (2010)
Lymphangioleiomyomatosis presenting in a postmenopausal woman.
Authors: W Sinclair, J L Wright, A Churg
Journal: Thorax (1985)

Lymphangioleiomyomatosis is a rare pulmonary disorder characterised by proliferation of smooth muscle diffusely throughout the lung parenchyma.Most patients present during their reproductive years, and survival is generally less than 10 years.We report a case in a postmenopausal woman that was associated with long survival.Case report
Posttranslational regulation of insulin-like growth factor binding protein-4 in normal and transformed human fibroblasts. Insulin-like growth factor dependence and biological studies.
Authors: C A Conover, M C Kiefer, J Zapf
Journal: Journal of Clinical Investigation (1993)

Insulin-like growth factor binding protein-4 (IGFBP-4) is a 24-26-kD protein expressed by a variety of cell types in vivo and in vitro. Treatment of normal adult human fibroblasts with 10 nM insulin-like growth factor II (IGF-II) for 24 h resulted in an 85% decrease in endogenous IGFBP-4, as assessed by Western ligand blot analysis of the conditioned medium. Incubation of human fibroblast-conditioned medium (HFCM) with IGF-II under cell-free conditions led to a similar loss of IGFBP-4. This posttranslationally regulated decrease in IGFBP-4 appeared to be due to a protease in HFCM: (a) It could be prevented with specific protease inhibitors or incubation at 4 degrees C; (b) proteolysis of recombinant human (rh) IGFBP-4 required HFCM; (c) immunoblotting and radiolabeling confirmed cleavage of IGFBP-4 into 18- and 14-kD IGFBP-4 fragments. The protease was specific for IGFBP-4, and was strictly dependent on IGFs for activation. IGF-II was the most effective of the natural and mutant IGFs tested, inducing complete hydrolysis of rhIGFBP-4 at a molar ratio of 0.25:1 (IGF/IGFBP-4). Simian virus 40-transformed adult human fibroblasts also expressed IGFBP-4 and IGFBP-4 protease, as well as an inhibitor of IGFBP-4 proteolysis. In biological studies, intact rhIGFBP-4 potently inhibited IGF-I-stimulated [3H]aminoisobutyric acid uptake, whereas proteolyzed rhIGFBP-4 had no inhibitory effect. In conclusion, these data provide evidence for a novel IGF-dependent IGFBP-4-specific protease that modifies IGFBP-4 structure and function, and indicate a preferential role for IGF-II in its activation. Posttranslational regulation of IGFBP-4 may provide a means for cooperative control of local cell growth by IGF-I and IGF-II.
A Recessive Form of the Ehlers–Danlos Syndrome Caused by Tenascin-X Deficiency
Authors: Joost Schalkwijk, Manon C. Zweers, Peter M. Steijlen, Willow B. Dean, Glen Taylor, Ivonne M. van Vlijmen, Brigitte van Haren, Walter L. Miller, James Bristow
Journal: New England Journal of Medicine (2001)

The Ehlers–Danlos syndrome is a heritable connective-tissue disorder caused by defects in fibrillar-collagen metabolism. Mutations in the type V collagen genes account for up to 50 percent of cases of classic Ehlers–Danlos syndrome, but many other cases are unexplained. We investigated whether the deficiency of the tenascins, extracellular-matrix proteins that are highly expressed in connective tissues, was associated with the Ehlers–Danlos syndrome.
Response to intramuscular methyl prednisolone in inflammatory hand pain: evidence for a targeted clinical, ultrasonographic and therapeutic approach
Authors: Z. Karim, M. A Quinn, R. J Wakefield, A. K Brown, M. J Green, E. M A Hensor, P. G Conaghan, P. Emery
Journal: Annals of the Rheumatic Diseases (2006)

<b>Background:</b> Hand pain with stiffness is a common clinical presentation to early arthritis clinics, with outcome varying from resolution to the development of rheumatoid arthritis. <b>Objective:</b> To assess the response and predictors of response to intramuscular methylprednisolone (MP) and hydroxychloroquine (HCQ) using a standardised treatment protocol. <b>Methods:</b> Patients with inflammatory hand pain (IHP), defined as predominantly hand pain and morning stiffness of at least 30 min duration, received a standardised assessment prior to receiving intramuscular MP. Response (primary outcome) at 4 weeks was a 50% improvement in symptoms as perceived by the patient; responders who relapsed received repeat intramuscular MP and HCQ. <b>Results:</b> 102 patients were recruited, of which 21% were rheumatoid factor (RF) positive, 23% had clinical synovitis, 25% had raised C-reactive protein level and 55% had ultrasound-detected synovitis. 73% responded, with associated significant reductions in morning stiffness, Health Assessment Questionnaire, painful and tender joint counts, and visual analogue scores (p⩽0.006 for all). Ultrasound-detected synovitis (p&lt;0.001) and RF (p = 0.04), but not clinical synovitis (p = 0.74), were significantly associated with response to intramuscular MP. 86% who remained on HCQ long term reported a benefit. <b>Conclusions:</b> Patients with IHP have significant improvement in symptoms and function following intramuscular MP. Further placebo-controlled trials are required to assess the role of intramuscular MP and ultrasonography in managing this patient group.
Filaggrin null mutations and childhood atopic eczema: A population-based case-control study
Authors: Sara J. Brown, Caroline L. Relton, Haihui Liao, Yiwei Zhao, Aileen Sandilands, Ian J. Wilson, John Burn, Nick J. Reynolds, W. H. Irwin McLean, Heather J. Cordell
Journal: Journal of Allergy and Clinical Immunology (2008)
Half of US doctors want national health insurance programme
Authors: S. Gottlieb
Journal: BMJ (2003)

A substantial proportion of US doctors supports government legislation to establish a national health insurance programme.

Researchers led by Dr Ronald Ackerman of the Indiana University School of Medicine, Indianapolis, Indiana, sampled 3250 doctors from the American Medical Association's physician masterfile, which is recognised as the most complete and accurate list of licensed physicians in the United States ( Annals of Internal Medicine 2003;139:795-801).

All participants were sent the survey and asked to respond to 12 questions, including whether they support or oppose the idea of governmental legislation to establish national health insurance and a …
Dermatology outpatient case-mix survey for all Welsh Trusts, 2007
Authors: G.M. Hill, J.M. Sowden, R.K. Lister, R.A. Logan, A.Y. Finlay
Journal: British Journal of Dermatology (2009)

Background In 2006 a U.K. government White Paper recommended making NHS care in England more accessible by shifting services from secondary care into community settings. There is a shortage of contemporary activity data for U.K. dermatology units to allow benchmarking for service development. This study will not only provide useful comparative data for the future in Wales, but will also serve to highlight the impact of changes made in England. Objective To provide an overview of 1 week's dermatology outpatient activity for the whole of Wales. Methods All dermatology units in Wales collected data for 1 week in early 2007. The case mix, appropriateness of referral, requirement for surgery or second-line therapies and follow-up requirements were all determined. Results A total of 2142 patients were seen. Of new patients, 21% had skin cancer. Seventeen per cent of skin cancers had no diagnosis suggested by the general practitioner (GP) and 10% of basal cell carcinomas, 33% of squamous cell carcinomas and 17% of malignant melanomas were inappropriately diagnosed. In all, 26% of new patients had benign lesions, and this group caused the greatest diagnostic difficulty for GPs. Seventy-one per cent of these patients were diagnosed, reassured and discharged at their first visit without the need for biopsy or surgery. Thirty-seven per cent of new patients required surgery, of which 21% required complex intervention. Twenty-six per cent of follow-up patients were receiving second-line therapies. The new to follow-up ratio varied considerably according to diagnosis, the mean ratio being 1 : 0.21 for benign lesions through to 1 : 5.53 for psoriasis. This highlights the inappropriate nature of a 'one fits all' ratio. The majority of follow-up patients in secondary care required this level of input for monitoring of cancer, complex second-line therapies or surgery. Conclusions This study provides evidence to support logical planning of dermatological services and to assess the impact of proposed changes on different healthcare systems in the U.K.
Lessons From the Third Reich: Ethics and Economics
Authors: Ralph Crawshaw
Journal: JAMA (1997)

To the Editor.  —The timely warning to our profession from Dr Barondess 1 might have been stronger and more to the point if its conclusion,  central lesson... is that medical ethos... can be severely distorted by social and political forces and by perversions in the application of science and technology, had included economic forces as well.  Our profession is currently being ethically subverted by an economic ideology that viciously parallels the political ideology that corrupted German medicine. It was not made clear that our lesson needs forceful translation from a German political example into US economic reality.  The fine article by Barondess is replete with examples of how economic coercion, fear of unemployment, promise of preferential income, and academic status compromised the ethics of front-line German practitioners. It is not intimidation by a totalitarian political force that grips US physicians but, like German physicians in the 1930s, fear of
Identification of those at risk after acute pulmonary embolism
Authors: A. J Fisher, P. A Corris
Journal: Thorax (2009)

It is well recognised by hands-on clinicians that patients who present acutely with haemodynamic compromise and hypotension with a systolic pressure of <90 mm Hg due to acute pulmonary embolism (PE) have a poor prognosis. This is reflected in current British Thoracic Society guidelines in the management of acute PE which recommend aggressive interventions in this population, with thrombolysis as first-line treatment.1

However, stratification of mortality and morbidity risk in normotensive patients who present with acute PE is less clear. Demonstration of right ventricular dysfunction or dilatation on either echocardiography or spiral HRCT scanning of the chest has been used as a potential indicator of those requiring more aggressive treatment even if normotensive. Immediate availability of these modalities is not universal, and a simpler way of identifying those patients at highest risk of adverse events and mortality remains critical as the inpatient mortality rate for normotensive patients with acute PE is approximately 10%.2 A number of previous studies have attempted to identify biomarkers which have prognostic power in patients with acute PE. Many of these studies have focused on cardiac biomarkers including the troponins and natriuretic peptides which correlate with right ventricular dysfunction on echocardiography.3 Whether these systemic biomarkers correlate with an …
Hepatitis B vaccine to be available in Southeast Asia
Authors: Charles Marwick
Journal: JAMA (1983)

At $100 for the three doses needed to provide adequate protection, the hepatitis B vaccine is largely beyond the means of those who need it most—the populations of Southeast Asia, China, and Africa, among whom the disease is endemic. The American manufacturers of the vaccine, Merck &amp; Co, Rahway, NJ, have now taken the first steps to rectify this. In an unusual move, Merck has agreed to license the vaccine-making process to Singapore Biotech, a subsidiary of Temasek Holdings, a Singapore government-owned holding company. The agreement provides that technical information and training in manufacturing the vaccine will be made available, says Maurice R. Hilleman, PhD, senior vice-president of Merck Sharp &amp; Dohme, West Point, Pa. Hilleman, who signed the agreement with Andrew Chew, chair of the Singapore concern, led the vaccine development team. Since it will be some time before Biotech can start producing the vaccine, and even then it
410. Physiological Correction of Glycogen Storage Disease Type II Using Adeno-Associated Virus Serotype 1 Vectors
Authors: Cathryn Mah, Christina A. Pacak, Kerry O. Cresawn, Lara R. DeRuisseau, Sean Germain, Melissa A. Lewis, David D. Fuller, Barry J. Byrne
Journal: Molecular Therapy (2006)

Glycogen storage disease type II (GSDII) is caused by a lack of functional lysosomal acid |[alpha]|-glucosidase (GAA) and results in massive storage of glycogen in lysosomal compartments, ultimately leading to fatal severe hypertrophic cardiomyopathy and respiratory insufficiency. Previously, we demonstrated the ability of a single intravenous administration of recombinant adeno-associated virus serotype 1 (rAAV2/1) vector to restore therapeutic levels of cardiac and diaphragmatic GAA enzymatic activity with concomitant clearance of glycogen in vivo in a mouse model of GSDII (Gaa-/-). We have further characterized both cardiac and respiratory function in rAAV2/1-treated animals one year post-treatment. Similar to the GSDII patient population, electrocardiogram (ECG) measurements (P-R interval) are significantly shortened in the mouse model. In rAAV2/1-treated mice, we show a significant improvement in cardiac conductance with prolonged P-R intervals of 39.34 |[plusmn]| 1.6 ms, as compared to untreated controls (35.58 |[plusmn]| 0.57 ms) (p|[le]|0.05). In addition, using cardiac magnetic resonance imaging (MRI) we note a marked decrease in cardiac left ventricular mass from 181.99 |[plusmn]| 10.70 mg in untreated age-matched controls to 141.97 |[plusmn]| 19.15 mg in the rAAV2/1-treated mice. Furthermore the mice displayed increased diaphragmatic contractile force to approximately 90% of wild-type peak forces with corresponding significantly improved ventilation (particularly in frequency, minute ventilation, and peak inspiratory flow), as measured using barometric whole body plethysmography. These results demonstrate that in addition to biochemical and histological correction, rAAV2/1 vectors can mediate sustained physiological correction of both cardiac and respiratory function in a model of fatal cardiomyopathy and muscular dystrophy.
Kathryn Jane Hosker
Authors: J. Thomas
Journal: BMJ (2004)

General practitioner Halifax, West Yorkshire (b Southport 1965; q Nottingham 1987; BMedSci, MRCGP), died from breast cancer on 17 August 2003.​2003.




Figure 1




Kath Hosker worked as a general practitioner in the Plane Trees Group Practice for nine years before her premature death. She moved to Halifax for her surgical houseman post and did her vocational training on the Calderdale scheme. Several years ago she introduced medical student teaching into the practice. She received an award in 1989 for her study of parents' attitudes to the measles, mumps, and rubella (MMR) vaccine. She leaves a husband, Michael, and two children.
Experimental S mansoni infection in genetically selected mice through high and low inflammatory response
Authors: Myrthes Toledo Barros, Adenir Perini, Maria Lucia Bueno Garcia, Milena Acencio, Maria Fernanda Macedo Soares, Mahasti Sahihi Macedo, Olga Martinez Ibanez, Jorge Kalil, Milton Arruda Martins
Journal: Journal of Allergy and Clinical Immunology (2002)
Retrograde jejunogastric intussusception caused by a migrated gastrostomy tube
Authors: L. Pelosof, D. Ringold, E. Kuo, S. Bhalla, R. Whinney, G. Zuckerman
Journal: Endoscopy (2007)

A 43-year-old woman presented to our hospital with emesis and coffee-ground output from the gastrostomy tube that she had had placed by interventional radiology 1 year before. On arrival, her heart rate was 140 beats per minute and her systolic blood pressure was 50 mm Hg. Laboratory testing showed her white blood cell count to be 30 × 109/L and her lactate was 3.8 mmol/L. A gastrostomy was in place, without any external fixation device. Computed tomography showed a mass containing small bowel, which was thought to be a jejunogastric intussusception ([Fig. 1], [2]). Upper endoscopy revealed the gastrostomy, with its balloon inflated, approximately 10 cm from the gastric entry site ([Fig. 3]), and a large, purple, friable mass emanating from the pylorus ([Fig. 4], [5]). Because the intussusception could not be reduced endoscopically, an emergency laparotomy was performed and a retrograde jejunogastric intussusception with ischemia was found. The bowel was dusky but was not perforated ([Fig. 6]). We resected 20 cm of jejunum and pathological examination revealed hemorrhagic coagulative necrosis and acute inflammation. The patient made an uncomplicated recovery postoperatively.
Symptomatic carotid lesions in young adults
Authors: A. Goonetilleke
Journal: BMJ (1995)

correct dose rapidly, and one can imagine the reaction of parents if children are taken to be weighed before emergency treatment is given.Doses should be stated clearly according to expected body weight by age and should be displayed in accident and emergency departments.Current recommendations for the treatment of anaphylaxis in a 5 year old child are that 0 4 ml of 1/1000 adrenaline should be given by intramuscular injection.2'
TOTAL CARDIOPULMONARY BYPASS IN THE LABORATORY A PRELIMINARY TO OPEN HEART SURGERY
Authors: L.D. Abrams, E.J. Charles, E.J. Hamley, R.E. Lee, F. Ashton, J. Fejfar, W.A. Hudson, R. Lightwood, E.T. Mathews, A.L. D'Abreu
Journal: The Lancet (1958)
Abstract 4516: SC-2001, a SHP-1 enhancer, induces apoptosis in hepatocellular carcinoma through SHP-1 dependent STAT3 inactivation
Authors: Kuen-Feng Chen, Jung-Chen Su, Wei-Lin Chen, Chun-Yu Liu, Ann-Lii Cheng, Pei-Jer Chen, Chung-Wai Shiau
Journal: Cancer Research (2011)

Abstract The activation of signal transducers and activators of transcription 3 (STAT3) signaling cascade plays a role in regulating cell survival, proliferation, and angiogenesis in various types of cancer, including hepatocellular carcinoma (HCC). Agents that can suppress STAT3 pathway have potentials for the treatment of HCC. Here, we investigated the effects of SC-2001 ((Z)-2-((3-methoxy-2H-pyrrol-2-ylidene)methyl)-1H-pyrrole), a compound from bacterium on HCC. We found that SC-2001 down-regulated the phosphorylation of STAT3 (Tyr 705) and subsequently inhibited transcriptional activities of STAT3. Downstream molecules, driven by STAT3 were repressed by SC-2001. In addition, ectopic expression of STAT3 in HCC cells abolished the prominent apoptotic death in cells treated with SC-2001, indicating that STAT3 is indispensable in mediating drugs’ effects. Importantly, SC-2001 enhanced the expression of SHP1, a negative regulator of STAT3. Knockdown of SHP-1 by small interference RNA, but neither SHP-2, nor PTP-1B, reduced apoptotic effects of SC-2001, indicating SHP-1 played a key role in mediating SC2001-induced cell death. In conclusion, our results suggest that SC-2001 induced apoptosis in HCC, and this effect is mediated through a through SHP-1-dependent STAT3 inactivation.Further clinical investigations of SC-2001 may be warranted. Supported by NSC98-2314-B-002-067-MY3, NSC98-3112-B-002-037, and NSC-99-2314-B-002-017-MY2 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 4516. doi:10.1158/1538-7445.AM2011-4516
Hyperkalaemia and impaired renal function in patients taking spironolactone for congestive heart failure: retrospective study
Authors: M. Svensson
Journal: BMJ (2003)

Spironolactone reduces disease and death in patients with severe congestive heart failure and is well tolerated with regard to renal function and serum potassium concentrations.1 Guidelines recommend taking spironolactone in addition to angiotensin converting enzyme inhibitors and β blockers,2 3 but since spironolactone can lead to renal dysfuction or hyperkalaemia, we followed up a cohort of patients taking spironolactone to identify predictors of harmful effects.

We selected 125 consecutive patients from the congestive heart failure outpatient clinic of Frederiksberg University Hospital, Copenhagen (table).4 We included only patients with a left ventricular ejection fraction (LVEF) of no more than 45% or patients who were taking spironolactone. We started 65 patients on spironolactone; 60 patients were already taking spironolactone when they were referred. We measured blood electrolytes at baseline and then every two months. The study started in September 1999 …
Mortality among widowers.
Authors: D. Rees
Journal: BMJ (1969)

BRIsA 825 femoral head taken at the time of internal fixation yielded the interesting result that if the specific gravity of the biopsy specimen was below 1-35 internal fixation ended either in non-union or collapse of the femoral head.3Determination of the specific gravity of femoral head biopsy specimens is time con- suming and only of retrospective value, but it is hoped that in the near future it will be possible to estimate the degree of mineralization of a femur or lumbar vertebra by feeding the video signal produced by the television camera of an image intensifier into the deflection plates of a suitable oscilloscope.Pre- liminary unpublished work performed by myself and by Marconi has shown that the method is feasible and the results are repro- ducible and reasonably accurate.This method should make it possible to divide the cases into two groups: 'a) Those with well- mineralized femoral heads suitable for pinning ; (b) those with poorly mineralized femoral heads suitable only for prosthetic replacement.Finally, it is worth mentioning that some patients who are unfit for operative treatment do quite well if they are mobilized in spite of their fracture.The femoral neck becomes rubbed away and the end result is a Girdle- stone's operation-I am.etc..