tbuckley/cabot-search huggingface dataset:
Sialic acid storage diseases. A multiple lysosomal transport defect for acidic monosaccharides.
Authors: G M Mancini, C E Beerens, P P Aula, F W Verheijen
Journal: Journal of Clinical Investigation (1991)

A defective efflux of free sialic acid from the lysosomal compartment has been found in the clinically heterogeneous group of sialic acid storage disorders. Using radiolabeled sialic acid (NeuAc) as a substrate, we have recently detected and characterized a proton-driven carrier for sialic acid in the lysosomal membrane from rat liver. This carrier also recognizes and transports other acidic monosaccharides, among which are uronic acids. If no alternative routes of glucuronic acid transport exist, the disposal of uronic acids can be affected in the sialic acid storage disorders. In this study we excluded the existence of more than one acidic monosaccharide carrier by measuring uptake kinetics of labeled glucuronic acid [( 3H]GlcAc) in rat lysosomal membrane vesicles. [3H]GlcAc uptake was carrier-mediated with an affinity constant of transport (Kt) of 0.3 mM and the transport could be cis-inhibited or trans-stimulated to the same extent by sialic acid or glucuronic acid. Human lysosomal membrane vesicles isolated from cultured fibroblasts showed the existence of a similar proton-driven transporter with the same properties as the rat liver system (Kt of [3H]GlcAc uptake 0.28 mM). Uptake studies with [3H]NeuAc and [3H]GlcAc in resealed lysosome membrane vesicles from cultured fibroblasts of patients with different clinical presentation of sialic acid storage showed defective carrier-mediated transport for both sugars. Further evidence that the defective transport of acidic sugars represents the primary genetic defect in sialic acid storage diseases was provided by the observation of reduced, half-normal transport rates in lymphoblast-derived lysosomal membrane vesicles from five unrelated obligate heterozygotes. This study reports the first observation of a human lysosomal transport defect for multiple physiological compounds.
John Benjamin Murphy Chicago Surgeon
Authors: Arthur F. Abt
Journal: JAMA (1968)

<h3>To the Editor:—</h3> Your interesting article on John Murphy, 1857-1916, Chicago surgeon, invites the following comments: Murphy was never known in Chicago as John Murphy. Mostly he was known as J.B. or John Benjamin Murphy. He designated himself as J.B. Murphy in all the articles noted in your bibliography. Your statement, "no one carried on his work after he died" is not exactly true. His son-in-law, John Golden, a talented surgeon, succeeded him; and he left many excellent pupils in Chicago who were first class surgeons. Finally, you omit noting that when Theodore Roosevelt was shot by an assassin in Milwaukee, he was brought by special train to Mercy Hospital in Chicago where John Benjamin Murphy treated his wound.
Skeletal Injury in the Child
Authors: John Connolly
Journal: JAMA (1990)

This meticulous review of children's skeletal injuries, by a premier investigator of skeletal embryology and morphology, has been completely revised. The second edition reflects considerable effort in each chapter to include pertinent new clinical and research information. The text, therefore, is approximately one-third longer than the first edition, with considerably more illustrations. Unlike many multiauthored texts, this one, written entirely by Ogden, provides the reader with a consistently thorough approach to each fracture problem. An in-depth discussion of the literature is followed by classification of each injury. Ogden then deals briefly with the diagnosis and treatment options for the injury and follows this with results, particularly as reported in the literature. A very pertinent discussion is usually included to inform the reader about potential complications and how they might be anticipated and managed. This thorough, painstaking presentation does not read easily. It provides more of a definitive review of the
Physical Activity in U.S. Adults With Diabetes and At Risk for Developing Diabetes, 2003
Authors: Elaine H. Morrato, James O. Hill, Holly R. Wyatt, Vahram Ghushchyan, Patrick W. Sullivan
Journal: Diabetes Care (2007)

Given the risk of obesity and diabetes in the U.S., and clear benefit of exercise in disease prevention and management, this study aimed to determine the prevalence of physical activity among adults with and at risk for diabetes.The Medical Expenditure Panel Survey is a nationally representative survey of the U.S. population. In the 2003 survey, 23,283 adults responded when asked about whether they were physically active (moderate or vigorous activity, > or =30 min, three times per week). Information on sociodemographic characteristics and health conditions were self-reported. Additional type 2 diabetes risk factors examined were age > or =45 years, non-Caucasian ethnicity, BMI > or =25 kg/m(2), hypertension, and cardiovascular disease.A total of 39% of adults with diabetes were physically active versus 58% of adults without diabetes. The proportion of active adults without diabetes declined as the number of risk factors increased until dropping to similar rates as people with diabetes. After adjustment for sociodemographic and clinical factors, the strongest correlates of being physically active were income level, limitations in physical function, depression, and severe obesity (BMI > or =40 kg/m(2)). Several traditional predictors of activity (sex, education level, and having received past advice from a health professional to exercise more) were not evident among respondents with diabetes.The majority of patients with diabetes or at highest risk for developing type 2 diabetes do not engage in regular physical activity, with a rate significantly below national norms. There is a great need for efforts to target interventions to increase physical activity in these individuals.
Results of a Clinical Care Pathway for Radical Prostatectomy Patients in an Open Hospital &amp;ndash; Multiphysician System
Authors: EdwardL. Gheiler, JonA.J. Lovisolo, Rabi Tiguert, MarcosV. Tefilli, Timothy Grayson, Gregory Oldford, IsaacJ. Powell, Gary Famiglietti, Mousumi Banerjee, J.Edson Pontes, DavidP. Wood, Jr.
Journal: European Urology (1999)
On the Control and Restraint of Habitual Drunkards
Authors: G. F. Bodington
Journal: BMJ (1875)

governing his will and restraining his passions by the precepts of the Gospel; and looking on its agitators, perhaps, as actuated by mate- rialistic leanings, and losing sight of moral influences and the dis- cipline of the church, which last, however, cuts the inebriate off from its pale, and too often casts him adrift to his fate.If these conjectures are so, surely this must be attributable to want of discrimination respecting the true position of dipsomaniacs, contrasted with other drunk- ards, on whose conduct in their state of liberty, or, rather, license, religious precept, or the example of the wise and good, cannot have the slightest influence.It has been said, without any exaggeration, that were immediate eternal punishment and a glass of intoxicating liquor put in a dipsomaniac's choice, he could not resist choosing the latter.As, therefore, spiritual benefits usually flow and operate through human agencies and instrumentalities, we believe that facility for legal control will be the likeliest medium for the transmission of a precious changing influence.Only by enforced abstinence from the accursed thing, as well as by other means, already detailed, brought to bear for months, or, perhaps, even years, on the unhappy subject of this propensity, can we hope to see him return to society, as it were, clothed and in right mind".But some others of the general public who look down on a wretched habitual drunkard with mixed feelings of pity and disgust, on account of the shame and disgrace of his position, may speak of his fate in terms like these, " Poor fellow, he killed himself with drink !" or of
Conservative treatment for patients over 80 years with acute myelogenous leukemia
Authors: Roberto Latagliata, Giuliana Alimena, Ida Carmosino, Massimo Breccia, Paola Anticoli Borza, Velia Bongarzoni, Carolina Copia, Antonio Spadea, Beatrice Pinazzi, Natalia Frattarelli, Felicetto Ferrara, Maria Concetta Petti, Franco Mandelli
Journal: American Journal of Hematology (2002)

Abstract In order to evaluate the best treatment of very elderly patients with AML, we have retrospectively analyzed 60 cases of patients aged more than 80 years, with a diagnosis of AML and observed from January 1988 to December 1998. Six of these patients were subsequently referred to other centers; of the remaining 54 patients, 20 (37%) received only supportive care, whereas 34 (63%) required palliative chemotherapy to control leukocytosis, after a median time from diagnosis of 9 days (range 0–253). Median overall survival was 13 weeks (range 1–105): 21 (39%) and 6 (11%) patients survived more than 6 and 12 months, respectively. Twenty‐eight patients (51.8%) died from progressive disease, 19 (35.1%) died from AML‐related or unrelated causes in the phase of stable disease, while in 7 patients the cause of death was unknown. In univariate analysis, PS &gt; 2 and WBC &gt; 50 × 10 9 /L had an adverse prognostic significance on survival. Our results, as compared with those reported in the literature for patients over 80 years treated with intensive chemotherapy, support the idea that intensive chemotherapy is usually not indicated in very elderly patients with AML, and that conservative treatment and the primary strategy of “watch‐and‐wait” presently seems to be the best choice. Am. J. Hematol. 71:256–259, 2002. © 2002 Wiley‐Liss, Inc.
A p53-dependent mechanism underlies macrocytic anemia in a mouse model of human 5q– syndrome
Authors: Jillian L Barlow, Lesley F Drynan, Duncan R Hewett, Luke R Holmes, Silvia Lorenzo-Abalde, Alison L Lane, Helen E Jolin, Richard Pannell, Angela J Middleton, See Heng Wong, Alan J Warren, James S Wainscoat, Jacqueline Boultwood, Andrew N J McKenzie
Journal: Nature Medicine (2009)
Mitochondrial Transporter ATP Binding Cassette Mitochondrial Erythroid Is a Novel Gene Required for Cardiac Recovery After Ischemia/Reperfusion
Authors: Marc Liesa, Ivan Luptak, Fuzhong Qin, Brigham B. Hyde, Ergun Sahin, Deborah A. Siwik, Zhengkun Zhu, David R. Pimentel, X. Julia Xu, Neil B. Ruderman, Karl D. Huffman, Susan R. Doctrow, Lauren Richey, Wilson S. Colucci, Orian S. Shirihai
Journal: Circulation (2011)

Background Oxidative stress and mitochondrial dysfunction are central mediators of cardiac dysfunction after ischemia/reperfusion. ATP binding cassette mitochondrial erythroid (ABC-me; ABCB10; mABC2) is a mitochondrial transporter highly induced during erythroid differentiation and predominantly expressed in bone marrow, liver, and heart. Until now, ABC-me function in heart was unknown. Several lines of evidence demonstrate that the yeast ortholog of ABC-me protects against increased oxidative stress. Therefore, ABC-me is a potential modulator of the outcome of ischemia/reperfusion in the heart. Methods and Results Mice harboring 1 functional allele of ABC-me (ABC-me +/− ) were generated by replacing ABC-me exons 2 and 3 with a neomycin resistance cassette. Cardiac function was assessed with Langendorff perfusion and echocardiography. Under basal conditions, ABC-me +/− mice had normal heart structure, hemodynamic function, mitochondrial respiration, and oxidative status. However, after ischemia/reperfusion, the recovery of hemodynamic function was reduced by 50% in ABC-me +/− hearts as a result of impairments in both systolic and diastolic function. This reduction was associated with impaired mitochondrial bioenergetic function and with oxidative damage to both mitochondrial lipids and sarcoplasmic reticulum calcium ATPase after reperfusion. Treatment of ABC-me +/− hearts with the superoxide dismutase/catalase mimetic EUK-207 prevented oxidative damage to mitochondria and sarcoplasmic reticulum calcium ATPase and restored mitochondrial and cardiac function to wild-type levels after reperfusion. Conclusions Inactivation of 1 allele of ABC-me increases the susceptibility to oxidative stress induced by ischemia/reperfusion, leading to increased oxidative damage to mitochondria and sarcoplasmic reticulum calcium ATPase and to impaired functional recovery. Thus, ABC-me is a novel gene that determines the ability to tolerate cardiac ischemia/reperfusion.
Clonality in hepatocellular carcinoma: Analysis of methylation pattern of polymorphic X–chromosome-linked phosphoglycerate kinase gene in females
Authors: Shigenobu Kawai, Fumio Imazeki, Osamu Yokosuka, Masao Ohto, Shuichiro Shina, Naoya Kato, Masao Omata
Journal: Hepatology (1995)

Analysis of X-chromosome inactivation patterns in women has been used to assess the clonality of various tumors. In this report, we analyzed 27 liver tumors in women, including 18 samples obtained by the performance of ultrasonically guided thin-needle biopsies. By analysis of the heterogeneity of phosphoglycerate kinase gene, 11 of 27 (41%) cases were found to be heterozygous at the gene. Of these informative 11 cases with liver tumors, 7 cases were "large" tumors (> 25 mm in diameter) and 4 cases were "small" tumors (< 25 mm in diameter). All 7 large tumors showed monoclonal patterns by the phosphoglycerate kinase gene analysis. Of the 4 small tumors, 2 showed monoclonal, and 2 showed polyclonal patterns. The 2 with monoclonal patterns were pathologically diagnosed as hepatocellular carcinoma despite their small sizes (20 mm and 23 mm). Of the two with polyclonal patterns, the smallest one (15 mm) was diagnosed as benign adenomatous hyperplasia, and the other as hepatocellular carcinoma heavily infiltrated by lymphocytes. These data suggest that analysis of the methylation pattern of the phosphoglycerate kinase gene may be helpful on rare occasions in elucidating the nature of liver tumors but must in fact be used in conjunction with histological appearances to avoid errors secondary to inflammatory infiltrates.
Favorable prognostic impact of NPM1 gene mutations in childhood acute myeloid leukemia, with emphasis on cytogenetically normal AML
Authors: I H I M Hollink, C M Zwaan, M Zimmermann, T C J M Arentsen-Peters, R Pieters, J Cloos, G J L Kaspers, S S N de Graaf, J Harbott, U Creutzig, D Reinhardt, M M van den Heuvel-Eibrink, C Thiede
Journal: Leukemia (2008)
Inhibitory receptors specific for MHC class I educate murine NK cells but not CD8αα intestinal intraepithelial T lymphocytes
Authors: Sylvie Taveirne, Jessica Filtjens, Els Van Ammel, Veerle De Colvenaer, Tessa Kerre, Tom Taghon, Bart Vandekerckhove, Jean Plum, Werner Held, Georges Leclercq
Journal: Blood (2011)
Relationship Between Depression and Other Medical Illnesses
Authors: Steven P. Roose
Journal: JAMA (2001)

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Thalassaemia major: the murky story of deferiprone
Authors: Julian Savulescu
Journal: BMJ (2004)

Conducting life saving research properly and quickly is a moral imperative 

Every year over 200 000 babies are born with thalassaemia major. They have a life expectancy of less than 30 years and are dependent on blood transfusions. Repeated transfusions result in cirrhosis of the liver, cardiomyopathy, endocrinopathies, and death due to haemosiderosis. Desferoxamine, an iron chelator, has been used for more than 30 years to treat haemosiderosis. It is given by daily, subcutaneous, slow injection, with inconvenience and local reactions resulting in suboptimal compliance in about half the patients.1 Despite desferoxamine, cardiac disease is still responsible for 70% of all deaths in these patients. Developing an orally administered chelating agent has therefore been a major objective in the care of patients with thalassaemia. Unfortunately the development of such a drug (deferiprone) has resulted in one of the most acrimonious and destructive of conflicts between a clinical researcher (Nancy Olivieri)and a drug company (Apotex).2 w1-w4 Thisdispute raises several ethical issues, which are discussed in a symposium in the Journal of Medical Ethics 3 and in part here.

Dr Olivieri began to study deferiprone in 1989. In 1993 Apotex began to provide financial support for her trials. By March 1996 Dr …
A pipe dream
Authors: T. Dalrymple
Journal: BMJ (2010)

The protagonist of Somerset Maugham’s novel The Narrow Corner , published in 1932, is a doctor by the name of Saunders, very like Maugham himself in philosophy if not in medical career. Maugham, of course, was a doctor, but unlike Saunders he never practised. (“One of our failures,” an eminent surgeon at St Thomas’s later said of the then richest author in the world, who made the modern equivalent of £30m (€34m; $48m) from one short story alone.)

Perhaps it would be fairer to say that Dr Saunders’s philosophy was as Maugham would have liked his own to be. Unlike Dr Saunders, Maugham was not a real cynic, unless a cynic be defined as a man who …
Bleeding Esophageal Varices
Authors: Euriko Torrazza-Perez, Norberto Carreno
Journal: New England Journal of Medicine (2010)

A 52-year-old man with cirrhosis presented to the emergency department with hematemesis and lightheadedness. He was alert and oriented, with a pulse rate of 140 beats per minute, a blood pressure of 90/60 mm Hg, and a hematocrit of 21%. He underwent emergency upper endoscopy.
Abstract 3862: The potential role of CD44 in liver regeneration
Authors: CT Lam, ZF Yang, MN Ng, T Wan, J Lau, DW Ho, ST Fan, RT Poon
Journal: Cancer Research (2012)

Abstract Background and Aim: Liver regeneration occurs after liver damage by ischaemia, hepatitis, or in hepatocellular carcinoma (HCC) patients receiving hepatic resection or liver transplantation. Understanding the molecular bases of this process will provide novel therapeutic approaches for patients with various liver diseases. Previous studies suggested the linkage between CD44, a multifunctional cell surface receptor, and liver regeneration. The detailed role of this receptor in liver regeneration, however, remained unclear. The present study aimed at elucidating the role of CD44 in liver regeneration. Materials and Methods: A mouse model of liver regeneration induced by partial hepatectomy (PH) was employed. Serum and liver tissues were collected at different time points after PH. ELISA was performed to measure hyaluronic acid (ligand for CD44) level in serum. Flow cytometry was done to characterize liver cell populations after PH. Liver regeneration was quantified by measurement of liver mass and by immunohistochemical staining for PCNA (a proliferation marker). Quantitative PCR was performed to compare gene expression pattern of 46 genes between CD45− CD44+ cells and their CD44− counterparts. Results: Firstly, serum hyaluronic acid level was elevated at the early stage of liver regeneration. Secondly, by flow cytometry, an increase in CD45− CD44+ cell population was found. Thirdly, immunohistochemical analysis revealed active hepatocyte proliferation during this period. Interestingly, the hepatectomy-induced cell proliferation was suppressed after treating mice with anti-CD44 antibody, suggesting a functional role of CD44 in liver regeneration. Lastly, dysregulation of genes involving in cell proliferation, angiogenesis, and metabolism of hyaluronic acid was disclosed in CD45− CD44+ cells. Conclusion: The present study indicated that CD44-mediated pathways are required for liver regeneration, which may have therapeutic implications in treating liver diseases, including HCC. Citation Format: {Authors}. {Abstract title} [abstract]. In: Proceedings of the 103rd Annual Meeting of the American Association for Cancer Research; 2012 Mar 31-Apr 4; Chicago, IL. Philadelphia (PA): AACR; Cancer Res 2012;72(8 Suppl):Abstract nr 3862. doi:1538-7445.AM2012-3862
Risk of Malignancies in Patients With Rheumatoid Arthritis Treated With Biologic Therapy
Authors: Maria A. Lopez-Olivo, Jean H. Tayar, Juan A. Martinez-Lopez, Eduardo N. Pollono, Jose Polo Cueto, M. Rosa Gonzales-Crespo, Stephanie Fulton, Maria E. Suarez-Almazor
Journal: JAMA (2012)

Concerns exist regarding the potential development of malignancies in patients with rheumatoid arthritis (RA) who are receiving biologic response modifiers (BRMs).To assess the risk of malignancy in patients with RA enrolled in randomized controlled trials (RCTs) of BRMs.Electronic databases, conference proceedings, and websites of regulatory agencies were searched for RCTs evaluating abatacept, adalimumab, anakinra, certolizumab, etanercept, golimumab, infliximab, rituximab, and tocilizumab in RA from inception through July 9, 2012.Independent selection of studies included RCTs that compared the safety of any BRMs used in RA patients with placebo and/or any traditional disease-modifying antirheumatic drugs with a minimum of 24 weeks of follow-up.Independent reviewers selected studies and extracted data on quality and outcomes. Pooled estimates and 95% confidence intervals were calculated for each BRM.Sixty-three RCTs with 29,423 patients were analyzed. No statistically significant increased risk of developing malignancy was observed. Of the 29,423 patients, 211 developed a malignancy during the trial (118 solid tumors, 48 skin cancers, 14 lymphomas, 5 hematologic nonlymphomas, and 26 not specified). The incidence rate for any malignancy during the first year of therapy was very low in the BRM plus methotrexate group (0.77%; 95% CI, 0.65%-0.92%), the BRM monotherapy group (0.64%; 95% CI, 0.42%-0.95%), and the controls (0.66%; 95% CI, 0.52%-0.84%). Anakinra plus methotrexate showed lower odds compared with methotrexate alone (Peto odds ratio, 0.11; 95% CI, 0.03-0.45). No statistically significant risk was observed for specific cancer sites, although the Peto odds ratio for lymphoma was 2.1 (95% CI, 0.55-8.4) in patients receiving tumor necrosis factor inhibitors compared with controls.The use of BRMs among patients with RA included in RCTs of at least 6 months' duration was not significantly associated with an increased risk of malignancy compared with other disease-modifying antirheumatic drugs or with placebo.
Common Bile Duct in Patients with Lung Neoplasms: a Reply
Authors: J. H. Siegel, R. G. Yapp
Journal: Endoscopy (1994)

See also: Unexplained Biliary Tract Dilatation in Lung Cancer PatientsEndoscopy 1992; 24(06): 593-595DOI: 10.1055/s-2007-1010553
G12-G13–LARG–mediated signaling in vascular smooth muscle is required for salt-induced hypertension
Authors: Angela Wirth, Zoltán Benyó, Martina Lukasova, Barbara Leutgeb, Nina Wettschureck, Stefan Gorbey, Petra Örsy, Béla Horváth, Christiane Maser-Gluth, Erich Greiner, Björn Lemmer, Günther Schütz, J. Silvio Gutkind, Stefan Offermanns
Journal: Nature Medicine (2007)