tbuckley/cabot-search huggingface dataset:
Sickle-cell disease in a British urban community.
Authors: E Anionwu, D Walford, M Brozovic, B Kirkwood
Journal: BMJ (1981)

Seventy cases of sickle-cell disease were identified in the London Borough of Brent from records dating back to 1962. All but three were still alive and, with one exception, were recalled for confirmation of the diagnosis and to provide personal and family histories. The group consisted of 22 individuals with homozygous sickle-cell anaemia (Hb SS), 12 with sickle-cell/beta-thalassaemia double heterozygosity, 34 with sickle-cell/haemoglobin C disease (Hb SC), and two with the combination of haemoglobin S and hereditary persistence of fetal haemoglobin. They were predominantly of West Indian origin, more than half had been born in Britain, and most were aged under 25. The records for 304 patient admissions between 1962 and 1979 were analysed. There were 199 sickle-cell-disease-related admissions, 61 unrelated to sickle-cell disease, and 44 for pregnancy or its complications. Admissions per patient-year averaged less than one, except for children with Hb SS under the age of 5 years, who were admitted more frequently. The commonest reasons for admission were painful crises (74% of all admissions) and the "chest syndrome" (21%). There were four pneumococcal infections, all in children with Hb SS under the age of 8 years; all recovered. Three patients, aged 10, 15, and 50 years, died. The two children with Hb SS died in their sleep without gross evidence of sickling at necropsy. Multiple brain infarcts were found at necropsy in the 50-year-old woman with Hb SC who, having survived nine uneventful pregnancies, succumbed to an infection after cryosurgery to the cervix. Obstetric records were available for 18 term pregnancies in 11 women. Three antenatal sickling crises and three postpartum thromboembolic complications were encountered. There were no maternal or perinatal deaths. Fifteen asymptomatic individuals with sickle-cell disease were diagnosed as a result of routine screening procedures. There are likely to be many such individuals currently undiagnosed in the community. They urgently need identification because of their increased risks from pregnancy, surgery, and infection.
Mechanism of Hb F stimulation by S-stage compounds. In vitro studies with bone marrow cells exposed to 5-azacytidine, Ara-C, or hydroxyurea.
Authors: R Galanello, G Stamatoyannopoulos, T Papayannopoulou
Journal: Journal of Clinical Investigation (1988)

The in vitro effect of S-stage-specific drugs on the fetal hemoglobin (Hb F) potential of erythroid precursors and progenitors was tested by exposing bone marrow cells to 5-aza-2'-deoxycytidine, Ara-C, or hydroxyurea in suspension cultures and reculturing the cells in drug-free clonal cultures. Analysis of Hb F in the erythroblasts present at the end of suspension cultures and in the erythroid colonies formed from treated progenitors showed that 1 X 10(-9)-5 X 10(-8) M 5-aza-2'-deoxycytidine produced a concentration-related increase in the proportion of Hb F-positive erythroblasts, of Hb F-positive erythroid CFU (CFUe) colonies, and at the higher doses used, an increased Hb F expression in erythroid burst-forming unit (BFUe)-derived colonies. Preincubation of bone marrow cells with Ara-C produced significant megaloblastic changes by the end of the 2-d incubation and increased the proportion of Hb F-positive erythroblasts, CFUe colonies, and e-clusters, but BFUe-derived progeny was unaffected. Hydroxyurea failed to produce significant changes in Hb F at the range of concentrations used. The data raise the possibility of more than one mechanism underlying the stimulation of Hb F by S-stage drugs.
A Sonographic Enthesitic Index of lower limbs is a valuable tool in the assessment of ankylosing spondylitis
Authors: M Alcalde, J. C Acebes, M Cruz, L Gonzalez-Hombrado, G Herrero-Beaumont, O Sanchez-Pernaute
Journal: Annals of the Rheumatic Diseases (2006)

Enthesitis is a remarkable feature of ankylosing spondylitis (AS) not specifically approached by the available measuring tools for the disease. Ultrasonography (US) has proved to be an excellent technique for the assessment of tendon pathology.To test a Sonographic Entheseal Index (SEI) of the lower limbs in a cohort of patients with AS, as a potential measuring tool.44 patients with AS and 10 healthy controls were enrolled. Bath Ankylosing Spondylitis Functional Index and Bath Ankylosing Spondylitis Disease Activity Index, pain at entheseal points, severity of symptoms, acute-phase reactants, Schober's test and stage of sacroiliitis were recorded. Patients underwent US examination of five entheseal regions from both lower limbs by two experts. Hypoechogenicity, increased tendon thickness, peritendinous oedema and bursitis were considered signs of active inflammation. Insertional bone erosions, intratendinous calcifications, decreased thickness and tears were considered signs of chronic injury. Each alteration independently scored one point. Data were analysed with Spearman's correlation method.A significant interobserver correlation in SEI scores (p<0.001) and a fine discriminative power between controls and patients were observed. Acute entheseal lesions predominated (63% vs 37%), the most frequent alteration being tendon hypoechogenicity (43%). 72% of all lesions were located at the foot. The SEI correlated with reduction of Schober's test (p<0.02), but not with other activity or severity parameters.A scoring method such as the SEI may be of help in characterising entheseal injury in AS, and for decision making in these patients.
Citrullination of autoantigens implicates NETosis in the induction of autoimmunity
Authors: Nishant Dwivedi, Marko Radic
Journal: Annals of the Rheumatic Diseases (2013)

Tolerance blocks the expression of autoantibodies, whereas autoimmunity promotes it. How tolerance breaks and autoantibody production begins thus are crucial questions for understanding and treatment of autoimmune diseases. Evidence implicates cell death and autoantigen modifications in the initiation of autoimmune reactions. One form of neutrophil cell death called NETosis deserves attention because it requires the post-translational modification of histones and results in the extracellular release of chromatin. NETosis received its name from NET, the acronym given to N eutrophil E xtracellular T rap. The extracellular chromatin incorporates histones in which arginines have been converted to citrullines by peptidylarginine deiminase IV (PAD4). The deiminated chromatin may function to capture or ‘trap’ bacterial pathogens, thus generating an extracellular complex of deiminated histones and bacterial cell adjuvants. The complex of bacterial antigens and deiminated chromatin may be internalised by host phagocytes during acute inflammatory conditions, as arise during bacterial infections or chronic autoinflammatory disorders. The uptake and processing of deiminated chromatin together with bacterial adjuvants by phagocytes may induce the presentation of modified histone epitopes and co-stimulation, thus yielding a powerful stimulus to break tolerance. Autoantibodies to deiminated histones are prevalent in Felty's syndrome patients and are present in systemic lupus erythematosus (SLE) and patients with rheumatoid arthritis (RA). These observations clearly implicate histone deimination as an epigenetic mark that can act as an autoantibody stimulant.
Phase II Study of Allogeneic Transplantation for Older Patients With Acute Myeloid Leukemia in First Complete Remission Using a Reduced-Intensity Conditioning Regimen: Results From Cancer and Leukemia Group B 100103 (Alliance for Clinical Trials in Oncology)/Blood and Marrow Transplant Clinical Trial Network 0502
Authors: Steven M. Devine, Kouros Owzar, William Blum, Flora Mulkey, Richard M. Stone, Jack W. Hsu, Richard E. Champlin, Yi-Bin Chen, Ravi Vij, James Slack, Robert J. Soiffer, Richard A. Larson, Thomas C. Shea, Vera Hars, Alexander B. Sibley, Sergio Giralt, Shelly Carter, Mary M. Horowitz, Charles Linker, Edwin P. Alyea
Journal: Journal of Clinical Oncology (2015)

Long-term survival rates for older patients with newly diagnosed acute myeloid leukemia (AML) are extremely low. Previous observational studies suggest that allogeneic hematopoietic stem-cell transplantation (HSCT) may improve overall survival (OS) because of lower rates of relapse. We sought to prospectively determine the value of HSCT for older patients with AML in first complete remission.We conducted a prospective multicenter phase II study to assess the efficacy of reduced-intensity conditioning HSCT for patients between the ages of 60 and 74 years with AML in first complete remission. The primary end point was disease-free survival at 2 years after HSCT. Secondary end points included nonrelapse mortality (NRM), graft-versus-host disease (GVHD), relapse, and OS.In all, 114 patients with a median age of 65 years received transplantations. The majority (52%) received transplantations from unrelated donors and were given antithymocyte globulin for GVHD prophylaxis. Disease-free survival and OS at 2 years after transplantation were 42% (95% CI, 33% to 52%) and 48% (95% CI, 39% to 58%), respectively, for the entire group and 40% (95% CI, 29% to 55%) and 50% (95% CI, 38% to 64%) for the unrelated donor group. NRM at 2 years was 15% (95% CI, 8% to 21%). Grade 2 to 4 acute GVHD occurred in 9.6% (95% CI, 4% to 15%) of patients, and chronic GVHD occurred in 28% (95% CI, 19% to 36%) of patients. The cumulative incidence of relapse at 2 years was 44% (95% CI, 35% to 53%).Reduced-intensity conditioning HSCT to maintain remission in selected older patients with AML is relatively well tolerated and appears to provide superior outcomes when compared with historical patients treated without HSCT. GVHD and NRM rates were lower than expected. Future transplantation studies in these patients should focus on further reducing the risk of relapse.
IS COLLAGENOME ERUPTIF A SEPARATE ENTITY?
Authors: M. J. WOERDEMAN
Journal: British Journal of Dermatology (1960)

Journal Article IS COLLAGÉNOME ÉRUPTIF A SEPARATE ENTITY? Get access M. J. WOERDEMAN, M. D. M. J. WOERDEMAN, M. D. Department of Dermatology and Venereology, Binnen‐Gasthuis, Amsterdam Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 72, Issue 6, 1 June 1960, Pages 217–220, https://doi.org/10.1111/j.1365-2133.1960.tb13881.x Published: 01 June 1960
Chemotherapy for Pulmonary Large Cell Neuroendocrine Carcinoma: Similar to that for Small Cell Lung Cancer or Non-Small Cell Lung Cancer?
Authors: J.-M. Sun, M.-J. Ahn, J.S. Ahn, H. Kim, Y.S. Choi, K. Kim, J. Han, J. Kim, O.J. Kwon, Y.M. Shim, E.Y. Heo, K. Park
Journal: Annals of Oncology (2012)
Th1 and Th2 Cytokine mRNA Profiles in Childhood Nephrotic Syndrome
Authors: HUI-KIM YAP, WAI CHEUNG, BELINDA MURUGASU, SZE-KEEN SIM, CHING-CHING SEAH, STANLEY C. JORDAN
Journal: Journal of the American Society of Nephrology (1999)

Abstract. Idiopathic nephrotic syndrome of childhood is thought to be associated with T lymphocyte dysfunction often triggered by viral infections, with the production of circulating factor(s) resulting in proteinuria. In view of the conflicting evidence of T cell activation and Th1 or Th2 pattern of cytokine synthesis in this disease, this study examined the mRNA expression of interleukin-2 (IL-2), interferon-γ, IL-4, and IL-13 from CD4+ and CD8+ T cells in steroid-responsive nephrotic patients in relapse and remission. Fifty-five children with steroid-responsive nephrotic syndrome were included in this study, together with 34 normal controls and 24 patient controls with viral infections. RNA was isolated from purified CD4+ or CD8+ cells from peripheral blood and subjected to reverse transcription-PCR. Cytokine mRNA expression was measured semiquantitatively, and a cytokine index was derived from densitometric readings, with cyclophilin as the housekeeping gene. Both cross-sectional and paired data showed an increased CD4+ and CD8+ IL-13 mRNA expression in patients with nephrotic relapse as compared to remission, normal, and patient controls ( P &lt;0.008). This was also associated with increased cytoplasmic IL-13 expression in phorbol myristate acetate/ionomycin-activated CD3+ cells (6.66 ± 3.39%) from patients with nephrotic relapse compared to remission (2.59 ± 1.35%) ( P &lt; 0.0001). However, there was no significant difference in CD4+ or CD8+ IL-2, interferon-γ and IL-4 mRNA expression. IL-13 is an important T cell cytokine with anti-inflammatory and immunomodulatory functions on B cells and monocytes. It is conceivable that IL-13 may act on monocytes to produce vascular permeability factor(s) involved in the pathogenesis of proteinuria in patients with relapse nephrotic syndrome.
THE EPILEPSIES AND LIFE ASSURANCE
Authors: S. A. K. Wilson
Journal: BMJ (1927)

my ol-.chief a`nd teaclher, wrote a paper witlh the arresting title, " At hat age do tabeties die?Those wlho know the woirk of this master of neulrology will aIree that on uccasron it lias been characterized by a somewlhat icono- clastic riending of l)reconceptions or of established and coiveiitional views from their pedestal; and the above-
What <i>is</i> the dynamical hypothesis?
Authors: Nick Chater, Ulrike Hahn
Journal: Behavioral and Brain Sciences (1998)

Van Gelder's specification of the dynamical hypothesis does not improve on previous notions. All three key attributes of dynamical systems apply to Turing machines and are hence too general. However, when a more restricted definition of a dynamical system is adopted, it becomes clear that the dynamical hypothesis is too underspecified to constitute an interesting cognitive claim.
Prevalence of Polycystic Ovary Syndrome Among Premenopausal Women With Type 2 Diabetes
Authors: Heather R. Peppard, Jennifer Marfori, Maria J. Iuorno, John E. Nestler
Journal: Diabetes Care (2001)

OBJECTIVE—Women with polycystic ovary syndrome (PCOS) have an increased risk for developing type 2 diabetes. Few studies have assessed women with type 2 diabetes to determine the frequency of PCOS in this population. RESEARCH DESIGN AND METHODS—To determine the prevalence of PCOS among premenopausal women with type 2 diabetes, we conducted a retrospective cross-sectional prevalence study. We reviewed the medical records of all women seen in the Diabetes Clinic of the Medical College of Virginia Hospitals between January 1995 through February 2000. A diagnosis of PCOS was based on 1) oligomenorrhea, 2) hyperandrogenism (biochemical or clinical), and 3) exclusion of other related disorders. RESULTS—We reviewed the medical records of 618 women with diabetes and identified 47 women eligible for study. Of the 47 women, 30 consented to an evaluation. Of the 30 women evaluated, 8 were identified as having PCOS (6 women reported a previous PCOS diagnosis and 2 women were newly diagnosed), resulting in a prevalence of 26.7%. CONCLUSIONS—We concluded that PCOS occurs frequently among premenopausal women with type 2 diabetes.
Wasting as an independent predictor of mortality in patients with cystic fibrosis
Authors: R Sharma
Journal: Thorax (2001)

<h3>BACKGROUND</h3> Cystic fibrosis (CF) is the most common life threatening autosomal recessive disorder in the white population. Wasting has long been recognised as a poor prognostic marker in CF. Whether it predicts survival independently of lung function and arterial blood gas tensions has not previously been reported. <h3>METHODS</h3> 584 patients with CF (261 women) of mean (SD) age 21 (7) years were studied between 1985 and 1996, all of whom were being followed up in a tertiary referral centre. Lung function tests, body weight, arterial blood oxygen (Pao<sub>2</sub>) and carbon dioxide (Paco<sub>2</sub>) tensions were measured. The weight was calculated as a percentage of the ideal body weight for age, height, and sex. <h3>RESULTS</h3> Forced expiratory volume in one second (FEV<sub>1</sub>) recorded at the start of the study was 1.8 (1.0) l (52 (26)% predicted FEV<sub>1</sub>), Pao<sub>2</sub> 9.8 (1.9) kPa, Paco<sub>2</sub> 5.0 (0.9) kPa, and % ideal weight 92 (18)%. During the follow up period (45 (27) months) 137 patients died (5 year survival 72%, 95% CI 67 to 73). FEV<sub>1</sub>, % predicted FEV<sub>1</sub>, Pao<sub>2</sub>, % ideal weight (all p&lt;0.0001), and Paco<sub>2</sub> (p=0.04) predicted survival. In multivariate analysis, % predicted FEV<sub>1</sub> (p&lt;0.0001), % ideal weight (p=0.004), and Paco<sub>2</sub> (p=0.02) were independent predictors of outcome. Patients with &gt;85% ideal body weight had a better prognosis at 5 years (cumulative survival 84%, 95% CI 79 to 89) than those with ⩽85% ideal weight (survival 53%, 95% CI 45 to 62), p&lt;0.0001. Percentage predicted FEV<sub>1</sub> (area under curve 0.83; 95% CI 0.78 to 0.87) and % ideal weight (area under curve 0.74; 95% CI 0.68 to 0.79) were accurate predictors of survival at 5 years follow up (receiver-operating characteristic analysis). <h3>CONCLUSIONS</h3> Body wasting is a significant predictor of survival in patients with CF independent of lung function, arterial blood oxygen and carbon dioxide tensions.
A Defined Tuberculosis Vaccine Candidate Boosts BCG and Protects Against Multidrug-Resistant <i>Mycobacterium tuberculosis</i>
Authors: Sylvie Bertholet, Gregory C. Ireton, Diane J. Ordway, Hillarie Plessner Windish, Samuel O. Pine, Maria Kahn, Tony Phan, Ian M. Orme, Thomas S. Vedvick, Susan L. Baldwin, Rhea N. Coler, Steven G. Reed
Journal: Science Translational Medicine (2010)

A vaccine with a four-protein fusion peptide from Mycobacterium tuberculosis effectively boosts the current childhood TB vaccine and protects against drug-resistant TB.
General Health Checks in Adults—Reply
Authors: Tanner J. Caverly, Allan V. Prochazka
Journal: JAMA Internal Medicine (2013)

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Photodynamic therapy using methylene blue in lung cancer animal models
Authors: Berengere Obstoy, Mathieu Salaun, Pierre Bohn, Liana Veresezan, Richard Sesboüé, Luc Thiberville
Journal: European Respiratory Journal (2013)

Introduction: Photodynamic therapy (PDT) is used to treat early proximal bronchial cancer during a flexible bronchoscopy. The technique relies on the excitation of a photosensitizer by an appropriate wavelength, which is delivered into the bronchus in close contact with the tumor.  Objective: To assess methylene blue (MB) as a PDT agent for the treatment of lung cancer in preclinical models.  Methods: MB induced PDT was performed on cancerous lesions obtained both in the cheek pouch model, and on NCI-H460 tumoral xenografts in nude mice. Bilateral tumors were induced in each animal. PDT was carried out on right-sided tumors after intratumoral injection of methylene blue 1% (w/v) and illumination at 630 nm at 200 J/cm (Diomed PDT 630). The left tumor was used as control (illumination alone or methylene blue alone). The tumoral volume was assessed before and 15 days after PDT.  Results: Nine carcinomas were treated in the cheek pouch model. MB-PDT produced a mean volume decrease of 85.8% (from 44.8% to 100%) (initial mean volume = 210 mm3 vs. final mean volume = 97 mm3). Histology analysis showed 4/9 complete responses.  14 xenografts were treated in mice, including 7 treated with MB-PDT, producing a 52% mean tumor volume regression (1568mm3 vs. 544mm3) compared to 7 controls in which tumor volume increased (p=0.007, Mann-Whitney test).  Conclusion: Topical methylene blue appears unexpensive and efficient as PDT agent for lung cancer treatment. Further studies are needed to assess the safety and efficacy of MB-associated PDT for the treatment of cancerous bronchial lesions in humans.  Financial support: French Ligue nationale against Cancer (Seine maritime and Eure Commitees) and ADIR, Rouen France.
Somatic mosaic IDH1 and IDH2 mutations are associated with enchondroma and spindle cell hemangioma in Ollier disease and Maffucci syndrome
Authors: Twinkal C Pansuriya, Ronald van Eijk, Pio d'Adamo, Maayke A J H van Ruler, Marieke L Kuijjer, Jan Oosting, Anne-Marie Cleton-Jansen, Jolieke G van Oosterwijk, Sofie L J Verbeke, Daniëlle Meijer, Tom van Wezel, Karolin H Nord, Luca Sangiorgi, Berkin Toker, Bernadette Liegl-Atzwanger, Mikel San-Julian, Raf Sciot, Nisha Limaye, Lars-Gunnar Kindblom, Soeren Daugaard, Catherine Godfraind, Laurence M Boon, Miikka Vikkula, Kyle C Kurek, Karoly Szuhai, Pim J French, Judith V M G Bovée
Journal: Nature Genetics (2011)
Long-term follow-up after high-dose chemotherapy and autologous stem-cell transplantation for high-grade B-cell lymphoma suggests an improved outcome for high-risk patients with respect to the age-adjusted International Prognostic Index
Authors: H. Bertz, R. Zeiser, W. Lange, S. Fetscher, C.F. Waller, J. Finke
Journal: Annals of Oncology (2004)
Kidney Function and Mortality among Patients with Left Ventricular Systolic Dysfunction
Authors: Nadia A. Khan, Irene Ma, Christopher R. Thompson, Karin Humphries, Deeb N. Salem, Mark J. Sarnak, Adeera Levin
Journal: Journal of the American Society of Nephrology (2005)

Kidney disease has emerged as a risk factor for mortality in heart failure populations. The objective of this study was to determine the impact of different stages of kidney dysfunction (defined using the Kidney Disease Outcomes Quality Initiative [K/DOQI] classification system) and changes in kidney function on mortality in a cohort of patients with heart failure. A retrospective analysis was conducted of data from the randomized controlled trials Studies of Left Ventricular Dysfunction. A total of 6640 participants with asymptomatic and symptomatic heart failure were studied. Estimated GFR (eGFR) were calculated and then categorized according to the K/DOQI classification system into the following categories: > or =90, 60 to 89, 30 to 59, and 15 to 29 ml/min per 1.73 m2. Reduction in eGFR from baseline was calculated and subsequently categorized according to rate of decline (<5, 5 to 10, 11 to 15, and >15 ml/min per 1.73 m2 per year). Independent of baseline differences, lower levels of eGFR were associated with a higher total mortality compared with those with eGFR > or =90 ml/min (30 to 59 ml/min per 1.73 m2: hazard ratio [HR] 1.32, 95% confidence interval [CI] 1.10 to 1.59, P = 0.004; 15 to 29 ml/min per 1.73 m2: HR 2.54, 95% CI 1.54 to 4.19, P < 0.001). eGFR deteriorated rapidly (>15 ml/min per 1.73 m2 per year) in 12% of participants. This decline was associated with a significant increase in mortality compared with slower decline (<5 ml/min per 1.73 m2 per year), despite adjustments for baseline kidney function, baseline heart failure, or change in heart failure (HR 5.63; 95% CI 4.90 to 6.46; P < 0.0001). The levels of eGFR from the K/DOQI classification system are associated with mortality in a well-characterized heart failure population. Rate of decline in kidney function is a strong predictor of increased mortality in this population, independent of worsening heart failure and baseline kidney function.