tbuckley/cabot-search huggingface dataset:
Primary pulmonary hypertension in families with hereditary haemorrhagic telangiectasia
Authors: S.A. Abdalla, C.J. Gallione, R.J. Barst, E.M. Horn, J.A. Knowles, D.A. Marchuk, M. Letarte, J.H. Morse
Journal: European Respiratory Journal (2004)

Primary pulmonary hypertension (PPH) is a rare but severe and progressive disease characterised by obstructive lesions of small pulmonary arteries. Patients with PPH often have mutations in the bone morphogenetic protein receptor type II ( BMPR2 ) gene, whereas some carry mutations in the activin receptor-like kinase 1 ( ALK‐1 ) gene, generally associated with hereditary haemorrhagic telangiectasia (HHT) type 2, a vascular dysplasia affecting multiple organs. The aim of this study was to determine whether members of families with PPH and confirmed or probable HHT had ALK‐1 mutations. ALK‐1 and BMPR2 mutation analysis was performed on deoxyribonucleic acid from affected members of four families with PPH and confirmed or suspected HHT. ALK‐1 mutations were identified in all four families and three novel mutations found in exon 10, leading to truncated proteins. In the fourth family, a missense mutation, previously reported in four independent HHT families, was detected in exon 8. Analysis of the BMPR2 gene revealed no exonic mutations in the probands with both PPH and HHT. The present data bring to 10 the number of reported families with primary pulmonary hypertension and hereditary haemorrhagic telangiectasia type 2, representing 16% of the 61 families with known activin receptor-like kinase 1 mutations. Such mutations might predispose to primary pulmonary hypertension, and specialists should be aware of the potential link between these two disorders.
Single Inhalation Exposure to &lt;sup&gt;90&lt;/sup&gt;SrCI&lt;sub&gt;2&lt;/sub&gt; in the Beagle Dog: Late Biological Effects&lt;xref ref-type="fn" rid="FN2"&gt;2&lt;/xref&gt;
Authors: N A Gillett, B A Muggenburg, B B Boecker, W C Griffith, F F Hahn, R O McClellan
Journal: JNCI Journal of the National Cancer Institute (1987)

Late-occurring biologic effects were studied in beagle dogs that were given graded levels of90SrCI2 via single brief inhalation exposures and were subsequently observed for their life-span. Due to the soluble chemical form of the aerosol90Sr was rapidly translocated from lung and deposited in bone where it was subsequently retained for a long period of time. Radiation-induced lesions were confined to the bone, bone marrow, and adjacent soft tissue. Forty-five primary bone tumors occurred in 31 of 66 exposed dogs. Metastasis occurred from 21 tumors, with the lung being the most frequent site of metastasis (76%). Twenty-seven tumors were classified as different subtypes of osteosarcoma, 14 as hemangiosarcomas, 3 as fibrosarcomas, and 1 as a myxosarcoma. Four carcinomas arising from soft tissues adjacent to bone were also considered to be90Sr induced. In contrast to bone tumors arising in beagles chronically exposed to90Sr through ingestion, histologic lesions of radiation osteodystrophy were minimal in this study, indicating that these lesions are not a necessary precursor of osteosarcoma development. The incidences of hemangiosarcomas (31%) and telangiectatic osteosarcomas (11%) in addition to osteosarcomas suggest that the cell of origin for all of these neoplasms is a multipotent mesenchymal cell with the potential for various morphologic expressions dependent on local environmental factors.
Identification of a cell-surface antigen selectively expressed on the natural killer cell.
Authors: L Glimcher, F W Shen, H Cantor
Journal: The Journal of Experimental Medicine (1977)

We have studied the cell-surface phenotype of natural killer (NK) cells of NZB and B6 mice which react to an MuLV+ lymphoid tumor. (a) NK cells do not express Thy1, Ly2, or Ig surface markers. (b) NK cells express an antigen recognized by C3H anti-CE antiserum ('anti-Ly1.2 antiserum'). Inasmuch as NK activity of spleen cells from B6 and B6/Ly1.1 congenic strains were both equally sensitive to C3H anti-CE antiserum, the NK antigen is distinct from Ly1.2. This point was confirmed by the observation that alphaNK activity was removed by absorption of C3H anti-CE antiserum with spleen cells from either B6 or B6/Ly1.1 congenic strains. Absorption of C3H alphaCE serum with BALB/c thymocytes and spleen cells (which are Ly1.2+NK-) removed anti-Ly1.2 activity and left anti-NK activity intact. This absorption step could be circumvented by inserting the BALB/c genotype into the recipient immunized to CE cells (i.e., (C3H X BALB/c)F1 alphaCE spleen cells). This antiserum, provisionally termed 'anti-NK', defines a new subclass of lymphocytes which may play a central role in the immunosurveillance against tumors.
Influence of surgery on deglutitive upper oesophageal sphincter mechanics in Zenker's diverticulum.
Authors: D W Shaw, I J Cook, G G Jamieson, M Gabb, M E Simula, J Dent
Journal: Gut (1996)

BACKGROUND/AIMS: To evaluate the role of upper oesophageal sphincter (UOS) compliance in dysphagia, the functional consequences of surgery were evaluated in eight patients with pharyngeal diverticula. The study examined the hypotheses that hypopharyngeal intrabolus pressure is an indicator of UOS compliance and that UOS opening and intrabolus pressure are normalised by surgery. METHODS: In eight patients and nine healthy controls, we measured the timing of swallow events, UOS relaxation, maximal UOS dimensions, intrabolus pressure, and trans-sphincteric bolus flow rates by simultaneous videoradiography and pharyngeal manometry. RESULTS: Bolus flow rates were not changed by surgery. Surgery significantly increased UOS opening (p = 0.0001) and reduced hypopharyngeal intrabolus pressure (p = 0.0001). The slope of the relation between sphincter area and intrabolus pressure was steeper in patients than controls and was normalised by surgery. Surgery had minor effects on basal UOS tone and timing of swallow events. CONCLUSIONS: Upper oesophageal sphincter compliance is poor in Zenker9s diverticulum and is normalised by surgery. Hypopharyngeal intrabolus pressure, which correlates with resistance to trans-sphincteric bolus flow, is a useful indicator of UOS compliance. Intrabolus pressure may be a predictor of outcome after myotomy in pharyngeal dysphagia. Cricopharyngeal myotomy is a mandatory component of surgery for Zenker9s diverticulum.
The human squamous oesophagus has widespread capacity for clonal expansion from cells at diverse stages of differentiation
Authors: Mariagnese Barbera, Massimiliano di Pietro, Elaine Walker, Charlotte Brierley, Shona MacRae, Benjamin D Simons, Phil H Jones, John Stingl, Rebecca C Fitzgerald
Journal: Gut (2014)

<h3>Objective</h3> Knowledge of the cellular mechanisms involved in homeostasis of human squamous oesophagus in the steady state and following chronic injury is limited. We aimed to better understand these mechanisms by using a functional 3D approach. <h3>Design</h3> Proliferation, mitosis and the expression of progenitor lineage markers were assessed in normal squamous oesophagus from 10 patients by immunofluorescence on 3D epithelial whole mounts. Cells expressing differential levels of epithelial and progenitor markers were isolated using flow cytometry sorting and characterised by qPCR and IF. Their self-renewing potential was investigated by colony forming cells assays and in vitro organotypic culture models. <h3>Results</h3> Proliferation and mitotic activity was highest in the interpapillary basal layer and decreased linearly towards the tip of the papilla (p&lt;0.0001). The orientation of mitosis was random throughout the basal layer, and asymmetric divisions were not restricted to specific cell compartments. Cells sorted into distinct populations based on the expression of epithelial and progenitor cell markers (CD34 and EpCAM) showed no difference in self-renewal in 2D culture, either as whole populations or as single cells. In 3D organotypic cultures, all cell subtypes were able to recapitulate the architecture of the tissue of origin and the main factor determining the success of the 3D culture was the number of cells plated, rather than the cell type. <h3>Conclusions</h3> Oesophageal epithelial cells demonstrate remarkable plasticity for self-renewal. This situation could be viewed as an ex vivo wounding response and is compatible with recent findings in murine models.
Outcomes after alemtuzumab-containing reduced-intensity allogeneic transplantation regimen for relapsed and refractory non-Hodgkin lymphoma
Authors: Emma Morris, Kirsty Thomson, Charles Craddock, Prem Mahendra, Donald Milligan, Gordon Cook, Graeme Murray Smith, Anne Parker, Steve Schey, Rajesh Chopra, Christopher Hatton, Jane Tighe, Anne Hunter, Karl Peggs, David Linch, Anthony Goldstone, Stephen Mackinnon
Journal: Blood (2004)
Diabetes Promotes Cardiac Stem Cell Aging and Heart Failure, Which Are Prevented by Deletion of the p66 <sup>shc</sup> Gene
Authors: Marcello Rota, Nicole LeCapitaine, Toru Hosoda, Alessandro Boni, Antonella De Angelis, Maria Elena Padin-Iruegas, Grazia Esposito, Serena Vitale, Konrad Urbanek, Claudia Casarsa, Marco Giorgio, Thomas F. Lüscher, Pier Giuseppe Pelicci, Piero Anversa, Annarosa Leri, Jan Kajstura
Journal: Circulation Research (2006)

Diabetes leads to a decompensated myopathy, but the etiology of the cardiac disease is poorly understood. Oxidative stress is enhanced with diabetes and oxygen toxicity may alter cardiac progenitor cell (CPC) function resulting in defects in CPC growth and myocyte formation, which may favor premature myocardial aging and heart failure. We report that in a model of insulin-dependent diabetes mellitus, the generation of reactive oxygen species (ROS) leads to telomeric shortening, expression of the senescent associated proteins p53 and p16INK4a, and apoptosis of CPCs, impairing the growth reserve of the heart. However, ablation of the p66shc gene prevents these negative adaptations of the CPC compartment, interfering with the acquisition of the heart senescent phenotype and the development of heart failure with diabetes. ROS elicit 3 cellular reactions: low levels activate cell growth, intermediate quantities trigger cell apoptosis, and high amounts initiate cell necrosis. CPC replication predominates in diabetic p66shc-/-, whereas CPC apoptosis and myocyte apoptosis and necrosis prevail in diabetic wild type. Expansion of CPCs and developing myocytes preserves cardiac function in diabetic p66shc-/-, suggesting that intact CPCs can effectively counteract the impact of uncontrolled diabetes on the heart. The recognition that p66shc conditions the destiny of CPCs raises the possibility that diabetic cardiomyopathy is a stem cell disease in which abnormalities in CPCs define the life and death of the heart. Together, these data point to a genetic link between diabetes and ROS, on the one hand, and CPC survival and growth, on the other.
Trends in mortality after hospital admission for liver cirrhosis in an English population from 1968 to 1999
Authors: S E Roberts
Journal: Gut (2005)

<b>Background:</b> Population based mortality rates from liver cirrhosis, and alcohol consumption, have increased sharply in Britain in the past 35 years. Little is known about the long term trends over time in mortality rates after hospital admission for liver cirrhosis. <b>Aims:</b> To analyse time trends in mortality in the year after admission for liver cirrhosis from 1968 to 1999. <b>Subjects:</b> A total of 8192 people who were admitted to hospital in a defined population of Southern England. <b>Methods:</b> Analysis of hospital discharge statistics linked to death certificate data. The main outcome measures were case fatality rates (CFRs) and standardised mortality ratios (SMRs). <b>Results:</b> At 30 days after admission, CFR was 15.9% and the SMR was 93 (86 in men and 102 in women, compared with 1 in the general population). At one year, the overall CFR was 33.6% and SMR was 16.3. There was no improvement from 1968 to 1999 in mortality rates. SMRs were highest for alcoholic cirrhosis of the liver (27.4 at one year) but lower for biliary cirrhosis (11.4) and chronic hepatitis (10.0). Mortality from most of the main causes of death, including accidents and suicides, was increased. <b>Conclusions:</b> The high mortality rates after hospital admission, and the fact that they have not fallen in the past 30 years, show that liver cirrhosis remains a disease with a very poor prognosis. Increased mortality from accidents, suicides, and mental disorders, particularly among those with alcoholic cirrhoses, indicates that prognosis is influenced by behavioural as well as by physical pathology.
Bone marrow transplantation reveals an essential synergy between neuronal and hemopoietic cell neurokinin production in pulmonary inflammation
Authors: Mara Chavolla-Calderón, Meggan K. Bayer, J. Julio Pérez Fontán
Journal: Journal of Clinical Investigation (2003)

Neurogenic inflammation is believed to originate with the antidromic release of substance P, and of other neurokinins encoded by the preprotachykinin A (PPT-A) gene, from unmyelinated nerve fibers (C-fibers) following noxious stimuli. Consistent with this concept, we show here that selective sensory-fiber denervation with capsaicin and targeted deletion of the PPT-A gene protect murine lungs against both immune complex–mediated and stretch-mediated injuries. Reconstitution of PPT-A gene–deleted mice with WT bone marrow does not abrogate this protection, demonstrating a critical role for PPT-A gene expression by sensory neurons in pulmonary inflammation. Surprisingly, reconstitution of WT mice with PPT-A gene–deficient bone marrow also confers protection against pulmonary injury, revealing that PPT-A gene expression in hemopoietic cells has a previously unanticipated essential role in tissue injury. Taken together, these findings demonstrate a critical synergy between capsaicin-sensitive sensory fibers and hemopoietic cells in neurokinin-mediated inflammation and suggest that such synergy may be the basis for a stereotypical mechanism of response to injury in the respiratory tract.
Retroperitoneoscopic Donor Nephrectomy: A Retrospective, Non-Randomized Comparison of Early Complications, Donor and Recipient Outcome with the Standard Open Approach
Authors: Alexander Bachmann, Thomas Wolff, Robin Ruszat, Olivier Giannini, Michael Dickenmann, Lorenz Gürke, Jürg Steiger, Thomas C. Gasser, Christian G. Stief, Tullio Sulser
Journal: European Urology (2005)
Multimodality Treatment Approach for Locally Advanced Pancreatic Cancer
Authors: Pestalozzi Bernhard, Datta Niloy, Riesterer Oliver, De Oliveira Michelle, Seifert Burckhardt, Winder Thomas, Siebenhuener Alexander, Samaras Panagiotis, Mamot Christoph, Bodis Stephan, Clavien Pierre-Alain
Journal: Annals of Oncology (2014)
Colistin and rifampicin in the treatment of nosocomial infections from multiresistant Acinetobacter baumannii
Authors: Said Motaouakkil, Boubaker Charra, Abdelhamid Hachimi, Hicham Nejmi, Abdellatif Benslama, Naima Elmdaghri, Habiba Belabbes, Mohamed Benbachir
Journal: Journal of Infection (2006)
Long-term effects of glucosamine sulphate on osteoarthritis progression: a randomised, placebo-controlled clinical trial
Authors: Jean Yves Reginster, Rita Deroisy, Lucio C Rovati, Richard L Lee, Eric Lejeune, Olivier Bruyere, Giampaolo Giacovelli, Yves Henrotin, Jane E Dacre, Christiane Gossett
Journal: The Lancet (2001)
Stromal Matrix Metalloproteinase-9 Regulates the Vascular Architecture in Neuroblastoma by Promoting Pericyte Recruitment
Authors: Christophe F. Chantrain, Hiroyuki Shimada, Sonata Jodele, Susan Groshen, Wei Ye, David R. Shalinsky, Zena Werb, Lisa M. Coussens, Yves A. DeClerck
Journal: Cancer Research (2004)

Abstract Advanced stages of neuroblastoma show increased expression of matrix metalloproteinases MMP-2 and MMP-9 (Y. Sugiura et al., Cancer Res., 58: 2209–2216, 1998) that have been implicated in many steps of tumor progression, suggesting that they play a contributory role. Using pharmacological and genetic approaches, we have examined the role of these MMPs in progression of SK-N-BE (2).10 human neuroblastoma tumors orthotopically xenotransplanted into immunodeficient mice. Mice treated with Prinomastat, a synthetic inhibitor of MMPs, showed an inhibition of tumor cell proliferation in implanted tumors and a prolonged survival (50 versus 39 days in control group, P &amp;lt; 0.035). Treatment with Prinomastat did not affect formation of liver metastases (P = 0.52) but inhibited intravascular colonization by the tumor cells in the lung by 73.8% (P = 0.03) and angiogenesis in both primary tumors and experimental liver metastases. The primary tumors from Prinomastat-treated mice showed a 39.3% reduction in endothelial area detected by PECAM/CD31 staining in tumor sections (P &amp;lt; 0.001), primarily due to the presence of smaller vessels (P = 0.004). MMP-2 is expressed by neuroblastoma tumor cells and stromal cells, whereas MMP-9 is exclusively expressed by stromal cells, particularly vascular cells. To examine the contribution of MMP-9 to tumor angiogenesis, we generated RAG1/MMP-9 double-deficient mice. We observed a significant inhibition of angiogenesis in the immunodeficient RAG1/MMP-9 double-deficient mice orthotopically implanted with tumor cells (P = 0.043) or implanted s.c. with a mixture of tumor cells and Matrigel (P &amp;lt; 0.001). Using an FITC-labeled lectin, we demonstrated an inhibition in the architecture of the tumor vasculature in MMP-9-deficient mice, resulting in fewer and smaller blood vessels. These changes were associated with a 48% decrease in pericytes present along microvessels. Taken together, the data demonstrate that in neuroblastoma, stromally derived MMP-9 contributes to angiogenesis by promoting blood vessel morphogenesis and pericyte recruitment.
Ultrasound diagnosis of Achilles tendon pathology in runners.
Authors: N Maffulli, R Regine, M Angelillo, G Capasso, S Filice
Journal: British Journal of Sports Medicine (1987)

The great upsurge in popularity of running activities has increased the number of athletes presenting with pathology of the Achilles tendon. A clinical and ultrasonic study was performed on 47 middle and long distance runners referred to the Authors with such problems. The results of this study can be grouped as follows: 1. paratendonitis: enlargement of the antero-posterior diameter of the tendon, and hyperechogenicity of Kager's triangle; 2. tendonitis (with or without paratendonitis): thickening of the tendon, with the presence of degenerative nodules; 3. enthesopathy: thickening of the distal part of the tendon, enlargement of the hypoechogenic area behind the tendon itself and microcalcification.
Community-acquired pneumonia in the elderly: Spanish multicentre study
Authors: R. Zalacain, A. Torres, R. Celis, J. Blanquer, J. Aspa, L. Esteban, R. Menéndez, R. Blanquer, L. Borderías
Journal: European Respiratory Journal (2003)

Community-acquired pneumonia (CAP) in the elderly has increased as a consequence of an overall increase of the elderly population. A controversy about the aetiology and outcome of CAP in this population still exists and more epidemiological studies are needed. A prospective, 12-month, multicentre study was carried out to assess the clinical characteristics, aetiology, evolution and prognostic factors of elderly patients (≥65 yrs) admitted to hospital for CAP. The study included 503 patients (age 76±7 yrs). The clinical picture lasted ≤5 days in 318 (63%) and the main clinical features were cough (n=407, 81%) and fever (n=380, 76%). Aetiological diagnosis was achieved in 199 (40%) cases, with a definite diagnosis obtained in 164 (33%). Of the 223 microorganisms isolated the main agents found were Streptococcus pneumoniae in 98 (49%) and Haemophilus influenzae in 27 (14%). A total of 53 patients died (11%) and the multivariate analysis showed the following factors of bad prognosis: previous bed confinement, alteration in mental status, absence of chills, plasma creatinine ≥1.4 mg·dL −1 , oxygen tension in arterial blood/inspiratory oxygen fraction ratio &lt;200 at the time of admission, and shock and renal failure during the evolution. The results of this study may aid in the management of empiric antibiotic treatment in elderly patients with community-acquired pneumonia and the patients who have a greater probability of bad evolution may be identified based on the risk factors.
T-LYMPHOMA ASSOCIATED WITH HTLV-I OUTSIDE THE CARIBBEAN AND JAPAN
Authors: N. Tubiana, F. Lecaer, N. Horchowski, G. Sebahoun, C. Lejeune, D. Sainty, D. Bagarry, Y. Carcassonne, David Cunningham, NigelL. Gilchrist, Andrew Jack, AngusG. Dalgleish, Michael Soukop, F.D. Lee, J. Baas, L.L. Thomas, J.W. Van Oostveen, S. Daenen, R.M. Slater
Journal: The Lancet (1985)
Carbachol and Vitamin B12 Absorption
Authors: A. W. Dellipiani, D. A. Seaton
Journal: BMJ (1965)

below that usually necessary to destroy the normal gland.If, therefore, myxoedema was related to radiotherapy, either the effect of irradiation was indirect or the thyroid glands were not normal initially.That myxoedema was not a direct effect of irradiation is suggested by the finding of thyroid auto-antibodies in the serum of all five patients.Although only one antibody in low titre was found in each case, the serological findings were similar to those in " spontaneous " myxoedema, which is now generally believed to be of autoimmune origin (Owen and Smart, 1958; have been unable to demonstrate increase of intrinsic-factor secretion after administration of carbachol to eight " normal " subjects.Ca rbachol is, however, often used in tests of vitamin-B12 absorption in the belief that the test is then more
Occlusion Angiography Using Intraoperative Contrast-enhanced Ultrasound Scan (CEUS): A Novel Technique Demonstrating Segmental Renal Blood Supply to Assist Zero-ischaemia Robot-assisted Partial Nephrectomy
Authors: Amrith Raj Rao, Robert Gray, Erik Mayer, Hanif Motiwala, Marc Laniado, Omer Karim
Journal: European Urology (2012)
Natural history of peanut allergy and predictors of resolution in the first 4 years of life: A population-based assessment
Authors: Rachel L. Peters, Katrina J. Allen, Shyamali C. Dharmage, Jennifer J. Koplin, Thanh Dang, Kate P. Tilbrook, Adrian Lowe, Mimi L.K. Tang, Lyle C. Gurrin
Journal: Journal of Allergy and Clinical Immunology (2015)
IκB kinase (IKK)β, but not IKKα, is a critical mediator of osteoclast survival and is required for inflammation-induced bone loss
Authors: Maria Grazia Ruocco, Shin Maeda, Jin Mo Park, Toby Lawrence, Li-Chung Hsu, Yixue Cao, Georg Schett, Erwin F. Wagner, Michael Karin
Journal: The Journal of Experimental Medicine (2005)

Transcription factor, nuclear factor κB (NF-κB), is required for osteoclast formation in vivo and mice lacking both of the NF-κB p50 and p52 proteins are osteopetrotic. Here we address the relative roles of the two catalytic subunits of the IκB kinase (IKK) complex that mediate NF-κB activation, IKKα and IKKβ, in osteoclast formation and inflammation-induced bone loss. Our findings point out the importance of the IKKβ subunit as a transducer of signals from receptor activator of NF-κB (RANK) to NF-κB. Although IKKα is required for RANK ligand-induced osteoclast formation in vitro, it is not needed in vivo. However, IKKβ is required for osteoclastogenesis in vitro and in vivo. IKKβ also protects osteoclasts and their progenitors from tumor necrosis factor α–induced apoptosis, and its loss in hematopoietic cells prevents inflammation-induced bone loss.