tbuckley/cabot-search huggingface dataset:
Wilson Disease: Clinical Presentation, Treatment, and Survival
Authors: Wolfgang Stremmel, Karl-Wilhelm Meyerrose, Claus Niederau, Harald Hefter, Georg Kreuzpaintner, Georg Strohmeyer
Journal: Annals of Internal Medicine (1991)

To evaluate the diagnostic features, clinical course, and overall long-term survival of patients with Wilson disease.Retrospective cohort study with a mean follow-up period of 14.2 years.A university medical center and a community hospital.Fifty-one consecutive patients with Wilson disease were evaluated between 1957 and 1989.Patients were treated with D-penicillamine (600 to 1800 mg/d). Two patients with end-stage liver disease had liver transplantation.Initial symptoms occurred at a mean age of 15.5 years. At diagnosis, the most common neurologic signs were dysarthria, tremor, writing difficulties, and ataxia followed by hypersalivation and headache. Somatic symptoms included abdominal pain, hepatomegaly, splenomegaly, cirrhosis of the liver, and thrombocytopenia. The mean serum concentrations of ceruloplasmin and copper were 44 mg/L and 4.7 mumol/L, respectively. The mean basal urinary copper excretion was 5.5 mumol/d, and the mean hepatic copper concentration was 19.6 mumol/g dry weight. Free serum copper concentration (mean, 2.7 mumol/L) was a reliable indicator of disease and was useful in assessing the effectiveness of therapy (values less than 1.6 mumol/L). Treatment with D-penicillamine improved most of the hematologic and neurologic abnormalities but had little effect on hepatomegaly and splenomegaly and did not reverse cirrhosis. Two patients died of fulminant hepatic failure during the observation period, whereas two others with end-stage liver disease had successful liver transplantation and remain asymptomatic. Long-term survival of patients with Wilson disease was similar to that of age- and sex-matched controls.Our results suggest that long-term treatment of patients with Wilson disease with D-penicillamine can relieve symptoms and improve prognosis.
The Cell Envelope of the Legionnaires' Disease Bacterium
Authors: ALAN R. FLESHER
Journal: Annals of Internal Medicine (1979)

We studied the cell-envelope structure of the Legionnaires' disease (LD) bacterium by electron microscopy and biochemical assays. There were apparent differences in cell structure by electron microscopy using two different prefixation methods. Organisms prefixed with gluteraldehyde had a single surrounding membrane. The typical two-membrane structure of gram-negative bacteria, however, was observed after prefixation with a combination of gluteraldehyde, formalin, and creosol. The cell wall (peptidoglycan) was seen in electron micrographs of plasmolyzed bacteria and in papaindigested cells. Both cytoplasmic and outer membranes were separated by differential centrifugation of spheroplast sonicates followed by sucrose density gradient ultracentrifugation. We identified each membrane by characteristic enzyme activity (cytoplasmic membrane) and 2-keto-3-deoxyoctonate content (outer membrane).
Molecular basis of beta thalassemia in the south of thailand
Authors: V. Laosombat, S‐P. Fucharoen, V. Panich, G. Fucharoen, M. Wongchanchailert, W. Sriroongrueng, C. Nopparatana, K. Kenpitak, M. Maipang, Y. Fukumaki
Journal: American Journal of Hematology (1992)

Abstract A total of 103 beta thalassemia genes from 78 children (45 with Hb E/beta thalassemia, 8 with beta thalassemia heterozygotes, and 25 with homozygous beta thalassemia) were analyzed using dot‐blot hybridization of the polymerase chain reaction‐amplified DNA and direct DNA sequencing. Nine mutations were characterized in 98/103 (95%) of beta thalassemia alleles, of which six (a 4 bp deletion in codons 41–42, a G‐C transition at position 5 of IVS‐1, A‐G transition at codon 19, an A‐T transition at codon 17, an A‐G transition at position –28 upstream of the beta globin gene, a G‐T transition at position 1 of IVS‐1), accounted for 92%. The spectrum of beta thalassemia mutations in Chinese Thai is similar to that reported among the Chinese from other parts of the world. The distribution of beta thalassemia mutations in Muslim Thai is similar to that reported among Malaysians. The most common beta thalassemia mutation in Thai and Chinese Thai patients is the frameshift mutation at codons 41–42, in comparison with the Muslim Thai in whom the G‐C transition at position 5 of the IVS‐1 mutation predominates. The heterogeneity of molecular defects causing beta thalassemia should aid in the planning of a prenatal diagnosis program for beta thalassemia in the South of Thailand. © 1992 Wiley‐Liss, Inc.
Caring for Patients at the End of Life
Authors: Peter D. Eisenberg
Journal: Annals of Internal Medicine (2002)

Letters19 March 2002Caring for Patients at the End of LifePeter D. Eisenberg, MDPeter D. Eisenberg, MDCalifornia Cancer CareGreenbrae, CA 94904Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-136-6-200203190-w1 SectionsAboutFull Text ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail TO THE EDITOR:Dr. Parker (1) is to be commended for his excellent end-of-life care. I wish that I had only 95 deaths in my past 12 years of practice! I agree with his conclusion that “discussing end-of-life issues rarely takes a lot of time.” In addition, it is the proper thing to do. At our institution, we have adopted a single-page form developed by Thomas J. Smith, MD, professor of medicine at Massey Cancer Center, Virginia Commonwealth University, Richmond, Virginia. It asks patients what they want to know about their illness and gives them an opportunity to hear their ...Reference1. Parker RA. Caring for patients at the end of life: reflections after 12 years of practice. Ann Intern Med. 2002;136:72-5. [PMID: 11777365] LinkGoogle Scholar Author, Article, and Disclosure InformationAffiliations: California Cancer CareGreenbrae, CA 94904 PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetailsSee AlsoCaring for Patients at the End of Life: Reflections after 12 Years of Practice Richard A. Parker Metrics Cited byAcupuncture for the Management of Low Back PainFeasibility and perception of a question prompt list in outpatient cancer care 19 March 2002Volume 136, Issue 6Page: W1KeywordsEnd of life care ePublished: 19 March 2002 Issue Published: 19 March 2002 Copyright & PermissionsCopyright © 2002 by American College of Physicians. All Rights Reserved.Loading ...
Dissolution of man-made vitreous fibers in rat alveolar macrophage culture and Gamble's saline solution: influence of different media and chemical composition of the fibers.
Authors: K Luoto, M Holopainen, K Karppinen, M Perander, K Savolainen
Journal: Environmental Health Perspectives (1994)

The effect of different chemical compositions of man-made vitreous fibers (MMVF) on their dissolution by alveolar macrophages (AM) in culture and in Gamble's solution was studied. The fibers were exposed to cultured rat AMs, culture medium alone; or Gamble's saline solution for 2, 4, or 8 days. The dissolution of the fibers was studied by measuring the amount of silicon (Si), iron (Fe), and aluminum (Al) in each medium. The AMs in culture dissolved Fe and Al from the fibers but the dissolution of Si was more marked in the cell culture medium without cells and in the Gamble's solution. The dissolution of Si, Fe, and Al was different for different fibers, and increased as a function of time. The Fe and Al content of the fibers correlated negatively with the dissolution of Si by AMs from the MMVF, i.e., when the content of Fe and Al of the fibers increased the dissolution of Si decreased. These results suggest that the chemical composition of MMVFs has a marked effect on their dissolution. AMs seem to affect the dissolution of Fe and Al from the fibers. This suggests that in vitro models with cells in the media rather than only culture media or saline solutions would be preferable in dissolution studies of MMVFs.
HISTONE DEACETYLASE INHIBITOR PCI-24781 SHOWS ANTIPROLIFERATIVE EFFECTS AGAINST PEDIATRIC GLIOBLASTOMA CELLS
Authors: E. T. Valera, P. V. Andrade, A. F. Andradre, R. G. d. P. Queiroz, C. A. Scrideli, L. Tone
Journal: Neuro-Oncology (2014)

BACKGROUND: Glioblastoma (GBM) is one of the most aggressive tumors affecting the central nervous system (CNS). Despite combined aggressive treatment schedules, the prognosis of children with GBM remains very poor. Epigenetic mechanisms may interfere with the process of carcinogenesis, and the acetylation of DNA can modulate the expression of genes related to the development and progression of malignancies. Clinical studies have shown that histone deacetylases inhibitors (HDACi are clinically active and well tolerated in the treatment of several tumors. Therefore, this study aims to evaluate the therapeutic potential of HDACi PCI-24781 in pediatric GBM lines, SF188 and KNS42 by proliferation assay. METHODS: Pediatric GMB cell-lines were cultured in HAM-F10 supplemented with 10% fetal bovine serum, at 37°C in a humidified 5% CO2 incubator. Cells were seeded in 96-well plates at 2x103 per well. After 24h, cells were treated with PCI-24781 at different concentrations (0.5, 1, 2, 4, 8 and 16 µM for 24, 48, 72 and 96h). Culture medium was removed and replaced with culture medium containing 5 µL of Resazurin dye in each well. Cells were incubated for 6h at 37°C, and the product was measured at 570 nm and 595 nm using a microplate reader. Statistical analysis was performed by One- and Two-way ANOVA and Bonferoni post-hoc. RESULTS: Both cell-lines were sensitive to PCI-24781 treatment, demonstrating an inhibition of proliferation (P < 0.05). For the SF188 cell-line, the strongest effect was observed at the doses of 4 µM, 8 µM and 16 µM at 96h (growth inhibition of 93%). The KNS42 cell-line showed a time-dependent antiproliferative pattern; the best effect was observed at 96h, when growth inhibition was of approximately 84%. It was observed time effect difference of proliferation between 24h and 48h (P < 0.05), but not between 48 h and 72 h. CONCLUSIONS: These primary data demonstrates that PCI-24781 can induce the inhibition of cell proliferation in pediatric GBM cells. Further experiments will be performed to assess the ability of this HDACi in modulating the cellular response in combinatin to ionizing radiation. Financial Support: FAPESP (process no. 2013/15891-8). SECONDARY CATEGORY: Preclinical Experimental Therapeutics.
Torsion of the testis: why is the prognosis so poor?
Authors: K I Bickerstaff, K K Sethia, J A Murie
Journal: BMJ (1987)

Population Censuses and Surveys) would not have been possible using routinely available data.It will, however, be possible using incidence data once the office has completed the postcoding of addresses in the cancer registration files (working retrospectively back to 1971).It will not be pos- sible using mortality data for the years before 1981 without a major recoding of address information.In conclusion, the Office ofPopulation Censuses and Surveys' report was not, and could not have been, a study of cancer risk among people living within two to three miles of nuclear installations.It is clear, however, from reports discussed in chapter 2 ofthe study that for some members ofthe public concern about possible risks extends at least as far as 10 miles.Data have now been presented that indicate cancer levels within these limits.'Dr Russell Jones considers the epidemiological evidence for an increase in childhood cancer in the vicinity of nuclear installations to be "consistent and compelling." In my opinion, a more balanced conclusion is that there have been some increases relating to different age groups and different distance zones in the vicinity of some installations and that there is as yet no clear evidence on what the cause of these increases might be.'-'2
How to predict the outcome in mature T and NK cell lymphoma by currently used prognostic models?
Authors: H-N Lin, C-Y Liu, J-T Pai, F-P Chang, C-F Yang, Y-B Yu, L-T Hsiao, T-J Chiou, J-H Liu, J-P Gau, C-H Tzeng, P-M Chen, Y-C Hong
Journal: Blood Cancer Journal (2012)

To select an appropriate prognostic model in the treatment of mature T- and natural killer (NK) -cell lymphoma (peripheral T-cell lymphoma (PTCL) and NK-/T-cell lymphoma (NKTCL)) is crucial. This study investigated the usefulness of Ann Arbor staging classification International prognostic index (IPI), prognostic index for T-cell lymphoma (PIT) and International peripheral T-cell lymphoma Project score (IPTCLP). Between 2000 and 2009, 176 patients (122 males) with PTCL and NKTCL were diagnosed and treated from a single institute in Taiwan. The correlation between complete response (CR) rate, 3-year overall survival (OS), early mortality rate and four prognostic models was analyzed. Thirty-one patients received hematopoietic stem cell transplantation (HSCT) and were analyzed separately. Three-year OS rate was 34.7%, and anaplastic large-cell lymphoma harbored better outcome than others. IPI score had the lowest Akaike information criterion value (1081.197) and was the best score in predicting OS and early mortality (P=0.009). Ann Arbor stage classification can predict CR rate more precisely (P=0.006). OS was significantly better in patients who received HSCT, even in patients with unfavorable features compared with chemotherapy alone. All prognostic models were useful to evaluate the outcome of patients with PTCL and NKTCL but IPI score did best in predicting OS in PTCL and PIT score in NKTCL. This study also supported the role of HSCT in patients with high-risk or refractory PTCL or NKTCL.
Emotional Disturbance and Subarachnoid Haemorrhage
Authors: R. Penrose, P. Storey
Journal: Psychotherapy and Psychosomatics (1970)

Subarachnoid haemorrhage (SAH) is fairly common, especially between the ages of 45 and 60 years, with an estimated incidence of 6 per 100,000 per annum in the area from which these patients were drawn. The mortality is high, as only 65% of those admitted to hospital are alive six months later. Morbidity is also high, both physical and mental; and 55% of 261 patients I followed up [Storey, 1967] had some mental changes. During the course of that study a number of patients, and their relatives, attributed the stroke to emotional stress. It is of course well known that such cases occur, but there appears to have been no systematic study of the subject. This present paper is in two parts: the first describes some of the findings in that follow-up study; the second is an account of a more recent and more prospective study.
Cardiomyopathy of limb-girdle muscular dystrophy
Authors: Daniel A.N. Mascarenhas, David H. Spodick, David A. Chad, James Gilchrist, Philip L. Townes, Umberto Degirolami, Gilbert H. Mudge, Dennis W. Maki, Richard L. Bishop
Journal: Journal of the American College of Cardiology (1994)
High levels of grass pollen inside European dairy farms: a role for the allergy‐protective effects of environment?
Authors: B. Sudre, M. Vacheyrou, C. Braun‐Fahrländer, A.‐C. Normand, M. Waser, G. Reboux, P. Ruffaldi, E. Von Mutius, R. Piarroux
Journal: Allergy (2009)

Background: There is evidence of an allergy protective effect in children raised on farm. It has been assumed that microbial exposure may confer this protection. However in farm, little attention has been given to the pollen level and to concomitant microbiological exposure, and indoor pollen concentrations have never been precisely quantified. Methods: The kinetics of pollen in dairy farms have been studied in a pilot study ( n = 9), and exposure in a sub‐sample of the ongoing European birth cohort PASTURE ( n = 106). Measurements of viable microorganisms and pollen were performed in air samples. To identify factors that modulate the pollen concentration multivariate regression analyses were run. Results: Indoor pollen (95% of Poaceae fragments and grains) were significantly higher in winter than in summer ( P = 0.001) and ranged between 858 to 11 265 counts/m 3 during feeding in winter, thus exceeding typical outdoor levels during the pollen season. Geometric mean in French farms was significantly higher than in German and Swiss farms (7 534, 992 and 1 079 count/m 3 , respectively). The presence of a ventilation system and loose housing systems significantly reduced indoor pollen levels. This pollen concentration rise after feeding was accompanied by an increase in fungal and actinomycetal levels, whereas the concentration of bacteria was not associated with feeding. Conclusions: Farmers and their children who attend cowsheds during the feeding sessions are exposed perennially to high pollen concentrations. It might be speculated that the combined permanent exposure to microbes from livestock and grass pollen may initiate tolerance in children living on a farm.
Gallbladder Mucosa in Cholecystography
Authors: Elliott C. Lasser
Journal: JAMA (1965)

A histochemical study of the mucosa of 85 freshly resected gallbladders was carried out. These were obtained from patients who had had a recent cholecystogram prior to surgery. There appeared to be a positive correlation between the degree of opacification of the gallbladder by the opaque material in the cholecystogram and the presence of (1) a relatively abundant mucosa, and (2) fluorescent granules in the basilar mucosal cells containing hydrolyzing enzymes. Consideration is given to what bearing, if any, these findings may have on a postulated reabsorption of cholecystographic media from the gallbladder lumen—a phenomenon that may play a role in some instances of poor or nonvisualization of the gallbladder in clinical cholecystography.
Childhood Malnutrition and Postwar Reconstruction in Rural El Salvador
Authors: Paula E. Brentlinger, Miguel A. Hernán, Sonia Hernández-Díaz, Lenore S. Azaroff, Maureen McCall
Journal: JAMA (1999)

ContextThe 1992 peace settlement that ended the civil war in El Salvador included land redistribution and other provisions designed to improve the socioeconomic status of ex-combatants and vulnerable civilians.ObjectiveTo describe associations between postwar social and economic assistance programs, especially land reform, and current child health status as reflected by nutrition in a population of resettled rural refugees.DesignA population-based cross-sectional survey of child nutritional status and principal elements of the reconstruction process.SettingA single rural municipality in northern El Salvador.ParticipantsA representative sample of 761 children younger than 5 years, living in 27 villages.Main Outcome MeasurePrevalence of stunting (low height for age) in children younger than 5 years.ResultsPrevalence of stunting was 32.4%. Stunting was significantly more prevalent among children whose families cultivated less land (odds ratio [OR] for stunting per additional hectare of redistributed land cultivated, 0.64; 95% confidence interval [CI], 0.44-0.93). Less than half of newly transferred land was being cultivated by its owners. Most of the children (84.7%) lived in families cultivating 2 hectares or less of redistributed land. Stunting was also more prevalent among children whose households lacked piped water (adjusted OR, 2.72; 95% CI, 1.87-3.96) vs those who had had piped water since before the cease-fire.ConclusionsMalnutrition, particularly stunting, persisted at high levels and was strongly associated with delay in full cultivation of redistributed land and in provision of water.
Filaggrin R501X Mutation is Associated with an Increased Susceptibility to Atopic Dermatitis and Eczema Herpeticum, and Associated Phenotypes in a European Population
Authors: K.C. Barnes, P. Gao, N.M. Rafaels, T. Hand, M. Boguniewicz, T.R. Hata, L. Schneider, J.M. Hanifin, R.L. Gallo, L. Gao, M. Yang, T.H. Beaty, L.A. Beck, D.Y. Leung
Journal: Journal of Allergy and Clinical Immunology (2009)
Double standards exist in judging traditional and alternative medicine
Authors: H. Bower
Journal: BMJ (1998)

The double standards that exist in judging orthodox and alternative medicine should be challenged, and reliable tools that can validate both approaches need to be found. The call came last week in London at a conference on integrated medicine which was organised by the Prince of Wales.

Dr Iain Chalmers, the director of the UK Cochrane Centre and a vociferous proponent of systematic reviews, told delegates: “Critics of complementary medicine …
The process of producing recommendations for rheumatic diseases: what is evidence?
Authors: J Zochling
Journal: Annals of the Rheumatic Diseases (2005)

The EULAR Standing Committee for International clinical Studies Including Therapeutics (ESCISIT) encourages the production of international evidence based recommendations for the management of rheumatic diseases. This has already led to published recommendations in osteoarthritis,1,2 and ankylosing spondylitis (AS),3 and recommendations for other disease states, including early rheumatoid arthritis and gout, have recently been completed. As a guide to the process for developing recommendations, standard operating procedures (SOPs) have been published,4 which include recommendations for the formation of a steering group, defining a target population, and the use of research evidence.

Although the SOP is generally straight­forward, several problems came up during the development of the recommen­dations for AS3 about wording and interpretation, which led to the decision to write this …
Presence of Sebaceous Glands in the Esophagus
Authors: L. Finet, J. P. Joly, H. Sevestre, J. L. Dupas
Journal: Endoscopy (1991)

See also: Methods of Disinfecting Endoscopic Material: Results of an International SurveyEndoscopy 1989; 21(06): 247-250DOI: 10.1055/s-2007-1012962
Increased Susceptibility to Pulmonary Emphysema among HIV-Seropositive Smokers
Authors: Philip T. Diaz, Mark A. King, Eric R. Pacht, Mark D. Wewers, James E. Gadek, Haikady N. Nagaraja, Janice Drake, Thomas L. Clanton
Journal: Annals of Internal Medicine (2000)

Background: Previous uncontrolled reports have suggested that HIV-seropositive persons develop an accelerated form of emphysema. Objective: To characterize the risk for emphysema in a stable HIV-seropositive outpatient population. Design: Controlled, cross-sectional analysis. Setting: Midwestern urban community. Participants: HIV-seropositive persons (n = 114) without AIDS-related pulmonary complications and HIV-seronegative controls (n = 44), matched for age and smoking history. Measurements: Measurement of pulmonary function, bronchoalveolar lavage, and high-resolution computed tomography of the chest. Results: The incidence of emphysema was 15% (17 of 114) in the HIV-seropositive group compared with 2% (1 of 44) in the HIV-seronegative group (P = 0.025). The incidence of emphysema in participants with a smoking history of 12 pack-years or greater was 37% (14 of 38 persons) in the HIV-seropositive group compared with 0% (0 of 14 persons) in the HIV-seronegative group (P = 0.011). The percentage of cytotoxic lymphocytes in lavage fluid was much higher in HIV-seropositive smokers with emphysema. Conclusions: Infection with HIV accelerates the onset of smoking-induced emphysema. The results of this study support the emerging concept that cytotoxic lymphocytes may have an important role in emphysema pathogenesis.