tbuckley/cabot-search huggingface dataset:
Purification of human very-long-chain acyl-coenzyme A dehydrogenase and characterization of its deficiency in seven patients.
Authors: T Aoyama, M Souri, S Ushikubo, T Kamijo, S Yamaguchi, R I Kelley, W J Rhead, K Uetake, K Tanaka, T Hashimoto
Journal: Journal of Clinical Investigation (1995)

Mitochondrial very-long-chain acyl-coenzyme A dehydrogenase (VLCAD) was purified from human liver. The molecular masses of the native enzyme and the subunit were estimated to be 154 and 70 kD, respectively. The enzyme was found to catalyze the major part of mitochondrial palmitoylcoenzyme A dehydrogenation in liver, heart, skeletal muscle, and skin fibroblasts (89-97, 86-99, 96-99, and 78-87%, respectively). Skin fibroblasts from 26 patients suspected of having a disorder of mitochondrial beta-oxidation were analyzed for VLCAD protein using immunoblotting, and 7 of them contained undetectable or trace levels of the enzyme. The seven deficient fibroblast lines were characterized by measuring acyl-coenzyme A dehydrogenation activities, overall palmitic acid oxidation, and VLCAD protein synthesis using pulse-chase, further confirming the diagnosis of VLCAD deficiency. These results suggested the heterogenous nature of the mutations causing the deficiency in the seven patients. Clinically, all patients with VLCAD deficiency exhibited cardiac disease. At least four of them presented with hypertrophic cardiomyopathy. This frequency (> 57%) was much higher than that observed in patients with other disorders of mitochondrial long-chain fatty acid oxidation that may be accompanied by cardiac disease in infants.
Hepatitis Vaccine and the Acquired Immunodeficiency Syndrome
Authors: ARNOLD M. SCHWARTZ
Journal: Annals of Internal Medicine (1983)

Letters and Corrections1 October 1983Hepatitis Vaccine and the Acquired Immunodeficiency SyndromeARNOLD M. SCHWARTZ, M.D., PH.D., TERENCE L. CHORBA, M.D.ARNOLD M. SCHWARTZ, M.D., PH.D.Search for more papers by this author, TERENCE L. CHORBA, M.D.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-99-4-567_2 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptTo the editor: The source for the hepatitis B vaccine is derived from human serum that may have been obtained from homosexual men or drug addicts, groups at high risk for the acquired immunodeficiency syndrome (AIDS) (1). Although the vaccine is rendered noninfectious (2), there has been concern that a presumptive transmissible agent for the syndrome may withstand the preparative techniques. As yet, no vaccine recipients outside the high-risk group have developed the syndrome (2); to evaluate the presumed risk for the syndrome associated with the vaccine, we have formulated a decision analysis. We propose that, after the decision to...References1. . Update on acquired immune deficiency syndrome—United States. MMWR. 1982;31:507-14. MedlineGoogle Scholar2. . The safety of the hepatitis B virus vaccine. MMWR. 1983;32:134-6. MedlineGoogle Scholar3. BRYANGREGG JM. Viral hepatitis in the United States (1970-1973): an analysis of morbidity trends and the impact of HBsAG testing on surveillance and epidemiology. In: Proceedings of a Symposium on Viral Hepatitis. Thorofare, New Jersey: Charles B. Slack; 1975; 271-82. CrossrefGoogle Scholar4. MULLEYSILVERSTEINDIENSTAG AMJ. Indications for use of hepatitis B vaccine based on cost-effectiveness analysis. N Engl J Med. 1982;307:644-52. CrossrefMedlineGoogle Scholar5. FRANCISHADLERTHOMPSON DSS. The prevention of hepatitis B with vaccine: report of the Centers for Disease Control multi-center efficacy trial among homosexual men. Ann Intern Med. 1982;97:362-6. LinkGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: George Washington University Washington, DC 20037 PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics 1 October 1983Volume 99, Issue 4Page: 567-568KeywordsAIDSAddictsDecision analysisHepatitisHepatitis BHomosexualsVaccines Issue Published: 1 October 1983 PDF DownloadLoading ...
High prevalence of somatic MAP2K1 mutations in BRAF V600E–negative Langerhans cell histiocytosis
Authors: Noah A. Brown, Larissa V. Furtado, Bryan L. Betz, Mark J. Kiel, Helmut C. Weigelin, Megan S. Lim, Kojo S. J. Elenitoba-Johnson
Journal: Blood (2014)
P3-Specific Amplitude Reductions to Respiratory and Auditory Stimuli in Subjects with Asthma
Authors: Kate E. Webster, Ian M. Colrain
Journal: American Journal of Respiratory and Critical Care Medicine (2002)

The respiratory-related evoked potential (RREP) is the electroencephalographic response to brief inspiratory occlusion. The P3 component of the response reflects the active cognitive processing of stimulus information. This study investigated the RREP in 16 subjects with asthma and 16 control subjects. In addition, auditory-evoked potentials were recorded using an oddball paradigm in both groups and were compared with the RREP results. Perceptual sensitivity was assessed using a load magnitude estimation task, whereby subjects estimated the magnitude of four added resistive loads using a modified Borg scale. For RREP assessment, inspiratory occlusions were presented for 200 milliseconds. For auditory-evoked potential assessment, 1,000- and 2,000-Hz pure tones were presented at 60 dB for 100 milliseconds, with the 2,000-Hz tone presented with 20% probability and identified as a target stimulus. Scalp electroencephalographic activity was measured from 29 channels. Resistive load intensity was linearly related to magnitude estimation in both subjects with asthma (r2 = 0.96) and control (r2 = 0.99) subjects. Results showed that both the respiratory and auditory P3 components were markedly reduced in the group with asthma compared with the control group. Other components were similar between the groups. These results suggest an intrinsic reduction in P3 amplitude for patients with asthma, which may relate to differences in processing perceptual information.
Improvement in symptoms and left ventricular performance after balloon aortic valvuloplasty in patients with aortic stenosis and depressed left ventricular ejection fraction.
Authors: R D Safian, S E Warren, A D Berman, D J Diver, L L McKay, P C Come, W Grossman, R G McKay
Journal: Circulation (1988)

The application of balloon aortic valvuloplasty to elderly patients with severe aortic stenosis and a low ventricular ejection fraction is undefined. Balloon aortic valvuloplasty was performed in a subset of 28 patients with low left ventricular ejection fraction (37 +/- 11%), severe aortic stenosis, and a mean age of 79 +/- 5 years. After valvuloplasty, significant increases were seen in aortic valve area (from 0.5 +/- 0.1 to 0.9 +/- 0.2 cm2), aortic systolic pressure (from 120 +/- 12 to 135 +/- 22 mm Hg), and cardiac output (from 4.2 +/- 1.1 to 4.8 +/- 1.6 l/min) (p less than 0.01), and significant decreases were seen in transaortic pressure gradient (from 69 +/- 25 to 35 +/- 15 mm Hg) and pulmonary capillary wedge pressure (from 24 +/- 9 to 20 +/- 7 mm Hg) (p less than 0.01). All patients had symptomatically improved at the time of discharge. Serial radionuclide ventriculography showed an increase in left ventricular ejection fraction from 37 +/- 11% before valvuloplasty to 44 +/- 14% within 48 hours after dilatation and to 49 +/- 13% at 3 months after dilatation. However, there was substantial heterogeneity of response. Thirteen patients (group A) showed progressive increases in left ventricular ejection fraction (from 34 +/- 11% to 49 +/- 15% to 58 +/- 11%, p less than 0.0001), whereas 15 patients (group B) showed no significant change in ejection fraction (from 41 +/- 10% to 40 +/- 13% to 41 +/- 10%, p = NS) over 3 months. There was no difference between these groups with respect to age, extent of coronary artery disease, history of myocardial infarction, and aortic valve area before and after valvuloplasty. However, peak systolic wall stress and left ventricular dimensions were higher in group B compared with group A. In conclusion, balloon aortic valvuloplasty may result in symptomatic improvement in patients with aortic stenosis and depressed left ventricular ejection fraction; some patients develop progressive increases in ejection fraction, whereas others fail to show improvement.
Medical audit in practice.
Authors: R Gruer, A A Gunn, A M Ruxton
Journal: BMJ (1977)

A stage by stage approach was adopted to solve some of the problems of diagnosing and managing acute abdominal pain. Audit started in hopsital and was extended, with the help of a community physician, to cover the practice of a group of general practitioners. Referrals to the accident and emergency department for acute abdominal pain were analysed, and the diagnostic accurancy of general practitioners, accident department staff, and ward staff was assessed. The accuracy of hopsital staff was improved by issuing guidelines and checklists to help in diagnosis. The general practitioners9 problems were defined and discussed with the surgeons. The audit continues with the aim of improving the general practitioners9 diagnostic accuracy.
Activation of inflammatory cells by immune complexes containing IgE in serum and synovial fluid of patients with rheumatoid arthritis: a study using flow cytometric analysis.
Authors: L S De Clerck, N J Struyf, C H Bridts, W J Stevens
Journal: Annals of the Rheumatic Diseases (1991)

Neutrophil and monocyte activation by immune complexes containing IgE from serum and synovial fluid of patients with rheumatoid arthritis is reported. Activation of the inflammatory cells was measured by stimulation of the respiratory burst with production of intracellular hydrogen peroxide. Generation of hydrogen peroxide was analysed by a flow cytometric method, using the fluorochrome dichlorofluorescein. The technique was modified to allow measurement of cell activation of both neutrophils and monocytes by immune complexes in suspension. Ten of 14 polyethylene glycol precipitates from serum of patients with rheumatoid arthritis and 10/16 synovial fluids of these patients could activate neutrophils. A positive relation was found between the activation of neutrophils and the total concentration of immune complexes, the presence of IgG, and the presence of IgE in the immune complexes. Activation of monocytes was also shown, but to a lesser extent (8/14 rheumatoid serum samples and 8/16 rheumatoid synovial fluids activated monocytes). There was a weak correlation between the concentration of IgE immune complexes and the intensity of fluorescence measured in the monocytes.
Anecdotes on asthma
Authors: J. Rees
Journal: BMJ (1995)

Channel 4's Cutting Edge on 9 October dealt with the problems of severe asthma. The programme featured a patient in a severe attack, two deaths, chronic severe asthma in two boys, and brittle asthma treated by subcutaneous infusion of terbutaline. Its message was that asthma can be fatal and lives can be ruined. It failed to take things any further.

The series title Cutting Edge gives the impression of scientific discovery hot out of the test tubes. The news here was the …
What Happens When Hospitalized Patients See Their Own Records
Authors: DAVID P. STEVENS
Journal: Annals of Internal Medicine (1977)

The effect of giving hospitalized medical patients access to their problem-oriented hospital records was investigated. Twenty-five subjects were given free access to their records while 25 other subjects served as controls. Few of the open record group actively sought and studied their records; most elected to see them only as part of routine ward rounds; and one objected to seeing the record at all. Taking the experimental group as a whole, there was no measurable effect of seeing the record on the subjects' ability to list their diagnoses or medications, their self-assessment of depression, anxiety or contentment, or their attitudes toward selected components of the health care system. On the other hand, in individual instances access to the hospital record seemed to facilitate communication and provide an opportunity for hospital inpatients to monitor objectively their hospital course.
TCT-837 Everolimus-eluting versus other rapamycin derivatives-eluting stents in patients with coronary artery disease: a meta-analysis of 16 randomized trials
Authors: Yao-Jun Zhang, Christos Bourantas, Shao Liang Chen, Javaid Iqbal, Takashi Muramatsu, Dong Sheng-Jie, Bo Xu
Journal: Journal of the American College of Cardiology (2013)
AOPP-induced activation of human neutrophil and monocyte oxidative metabolism: A potential target for N-acetylcysteine treatment in dialysis patients
Authors: Véronique Witko-Sarsat, Valérie Gausson, Anh-Thu Nguyen, Malik Touam, Tilman Drüeke, Francesco Santangelo, Béatrice Descamps-Latscha
Journal: Kidney International (2003)
<i>In Vivo</i> Magnetic Resonance Imaging of the Estrogen Receptor in an Orthotopic Model of Human Breast Cancer
Authors: Adi Pais, Chidambaram Gunanathan, Raanan Margalit, Biton Inbal Eti, Ady Yosepovich, David Milstein, Hadassa Degani
Journal: Cancer Research (2011)

Histologic overexpression of the estrogen receptor α (ER) is a well-established prognostic marker in breast cancer. Noninvasive imaging techniques that could detect ER overexpression would be useful in a variety of settings where patients' biopsies are problematic to obtain. This study focused on developing, by in vivo MRI, strategies to measure the level of ER expression in an orthotopic mouse model of human breast cancer. Specifically, novel ER-targeted contrast agents based on pyridine-tetra-acetate-Gd(III) chelate (PTA-Gd) conjugated to 17β-estradiol (EPTA-Gd) or to tamoxifen (TPTA-Gd) were examined in ER-positive or ER-negative tumors. Detection of specific interactions of EPTA-Gd with ER were documented that could differentiate ER-positive and ER-negative tumors. In vivo competition experiments confirmed that the enhanced detection capability of EPTA-Gd was based specifically on ER targeting. In contrast, PTA-Gd acted as an extracellular probe that enhanced ER detection similarly in either tumor type, confirming a similar vascular perfusion efficiency in ER-positive and ER-negative tumors in the model. Finally, TPTA-Gd accumulated selectively in muscle and could not preferentially identify ER-positive tumors. Together, these results define a novel MRI probe that can permit selective noninvasive imaging of ER-positive tumors in vivo.
Telomere Shortening Is Associated with Genetic Anticipation in Chinese Von Hippel–Lindau Disease Families
Authors: Xiang-hui Ning, Ning Zhang, Teng Li, Peng-jie Wu, Xi Wang, Xue-ying Li, Shuang-he Peng, Jiang-yi Wang, Jin-chao Chen, Kan Gong
Journal: Cancer Research (2014)

Von Hippel-Lindau (VHL) disease is a rare autosomal dominant cancer syndrome. A phenomenon known as genetic anticipation has been documented in some hereditary cancer syndromes, where it was proved to relate to telomere shortening. Because studies of this phenomenon in VHL disease have been relatively scarce, we investigated anticipation in 18 Chinese VHL disease families. We recruited 34 parent-child patient pairs (57 patients) from 18 families with VHL disease. Onset age was defined as the age when any symptom or sign of VHL disease first appeared. Anticipation of onset age was analyzed by paired t test and the other two special tests (HV and RY2). Relative telomere length of peripheral leukocytes was measured in 29 patients and 325 healthy controls. Onset age was younger in child than in parent in 31 of the 34 parent-child pairs. Patients in the first generation had older onset age with longer age-adjusted relative telomere length, and those in the next generation had younger onset age with shorter age-adjusted relative telomere length (P < 0.001) in the 10 parent-child pairs from eight families with VHL disease. In addition, relative telomere length was shorter in the 29 patients with VHL disease than in the normal controls (P = 0.003). The anticipation may relate to the shortening of telomere length in patients with VHL in successive generations. These findings indicate that anticipation is present in families with VHL disease and may be helpful for genetic counseling for families with VHL disease families and for further understanding the pathogenesis of VHL disease.
An early marker of fetal infection after primary cytomegalovirus infection in pregnancy.
Authors: H Stern, G Hannington, J Booth, D Moncrieff
Journal: BMJ (1986)

Fourteen patients with primary cytomegalovirus infection diagnosed by serological screening at antenatal attendances were examined for their responses in the lymphocyte transformation test against cytomegalovirus. Tests were done during pregnancy, shortly after the diagnosis of primary infection. Eight women showed positive lymphocyte transformation responses and gave birth to uninfected babies. Six showed negative responses and four of the babies were born congenitally infected. Cellular immunity therefore plays a part in preventing intrauterine transmission of cytomegalovirus, and its depression after primary infection in the mother during pregnancy may be used as an early marker of fetal infection.
Should Every Pregnant Woman Undergo Ultrasonography?
Authors: Richard L. Berkowitz
Journal: New England Journal of Medicine (1993)

Diagnostic ultrasonography has unquestionably revolutionized the care of obstetrical patients. Before this procedure became available, the uterus of a pregnant woman was a closed space that effectively hid most of its secrets. Since the mid-1970s, however, remarkable improvements in ultrasonography have exposed that protected space to investigation throughout the course of pregnancy and have thereby provided sophisticated insight into life before birth.Ultrasonography has proved to be of invaluable assistance in a variety of high-risk situations. Recognition of what could be learned in problem pregnancies led to the question of whether examining every pregnant woman with ultrasonography offered advantages over . . .
Increased Circulating IgE in Trichinosis
Authors: EUGENE B. ROSENBERG
Journal: Annals of Internal Medicine (1971)

A patient with acute trichinosis was studied to determine the time course of development of elevated IgE levels after ingesting pork infected with Trichinella spiralis. The patient's serum IgE concentration was normal on the 21st day after ingestion. A threefold rise in IgE concentration occurred between the 22nd and 27th days. Elevation of IgE was a late component of the humoral immune response to T. spiralis infection. Trichinosis is the fourth parasitic disease to be associated with elevated IgE levels and the first in which the time course of the IgE rise has been documented.
AIDS at 30: a chance to treat is now a chance to prevent
Authors: Douglas Kamerow
Journal: BMJ (2011)

The question for tomorrow is how do we pay for it

Thirty years ago, on 4 June 1981, the Centers for Disease Control and Prevention published a small case series of previously healthy homosexual men in Los Angeles who had contracted pneumocystis pneumonia.1 A month later, 26 more cases were described, from New York as well as California, and including Kaposi’s sarcoma as one of their opportunistic diseases. 

So it began.

Gay men were the initial focus, but then people with haemophilia, others receiving transfusions, and injection drug users were stricken. And stricken was the appropriate word. In those early days almost everyone with AIDS died, and most died within months of the appearance of symptoms. Many of the deaths were horrible: pneumonia, complicated by intractable diarrhoea, severe stomatitis, skin lesions, and more. Drugs commonly used to treat their infections and cancers didn’t work. I took care of a few early AIDS patients during my residency, and it was awful and frustrating for them, their loved ones, and their doctors and nurses.

It is stunning to look back and realise that …
Lower Socioeconomic Status and Increased Mortality
Authors: Redford B. Williams
Journal: JAMA (1998)

Our website uses cookies to enhance your experience. By continuing to use our site, or clicking "Continue," you are agreeing to our Cookie Policy | Continue JAMA HomeNew OnlineCurrent IssueFor Authors Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More Publications JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry (1919-1959) JN Learning / CMESubscribeJobsInstitutions / LibrariansReprints & Permissions Terms of Use | Privacy Policy | Accessibility Statement 2023 American Medical Association. All Rights Reserved Search All JAMA JAMA Network Open JAMA Cardiology JAMA Dermatology JAMA Forum Archive JAMA Health Forum JAMA Internal Medicine JAMA Neurology JAMA Oncology JAMA Ophthalmology JAMA Otolaryngology–Head & Neck Surgery JAMA Pediatrics JAMA Psychiatry JAMA Surgery Archives of Neurology & Psychiatry Input Search Term Sign In Individual Sign In Sign inCreate an Account Access through your institution Sign In Purchase Options: Buy this article Rent this article Subscribe to the JAMA journal
Acute excited states and sudden death
Authors: F. R Farnham, H. G Kennedy
Journal: BMJ (1997)

Thank you for your interest in spreading the word about The BMJ.NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address. Username * Your Email * Send To * You are going to email the following Acute excited states and sudden death Your Personal Message CAPTCHA This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Smoking and blood basophils.
Authors: S Walter, A Walter
Journal: Thorax (1986)

Chemotherapy in non-small cell bronchial carcinoma SIR,-In the first paragraph of your editorial on the chemotherapy of non-small cell bronchial carcinoma (September 1985;40:641-5) it is stated that very few patients with squamous or large cell carcinoma or adenocarcinoma of the lung are cured by surgery.This is indeed true in relation to the total number of cases of lung cancer each year, but on the other hand it should be emphasised that in the relatively small group of patients who are suitable for surgical treat- ment the five year survival rate (operative mortality ex- cluded) is as high as 25% (to be precise 25.5-26.8% in the review by Belcher of 8781 patients with bronchial carcinoma operated on by seven surgeons in England during the years
Mercury and Health
Authors: P Michael Bolger, B A Schwetz
Journal: New England Journal of Medicine (2002)

Mercury, particularly methylmercury, is an established worldwide environmental pollutant with known toxicity in humans. The toxic effects of methylmercury in fish were first brought to light after several episodes of poisoning in Japan that involved a spectrum of adverse clinical outcomes. These ranged from paresthesias and blurred vision to more specific signs of methylmercury intoxication, such as concentric vision and deafness, and to coma and death in some cases. Pronounced deficits in neurologic development were also noted. These outcomes were associated with the ingestion of fish that contained methylmercury at levels of 10 ppm or higher — levels clearly linked . . .