tbuckley/cabot-search huggingface dataset:
Acute myelofibrosis terminating in acute lymphoblastic leukemia: Case report and review of the literature
Authors: Cherie H. Dunphy, Sarah Kitchen, Oscar Saravia, William S. Velasquez
Journal: American Journal of Hematology (1996)

Acute myelofibrosis (AMF), as defined by an acute panmyelopathy associated with marked megakaryocytic hyperplasia and marrow fibrosis, appears to be a stem cell disorder. Even though it is most difficult to distinguish from various myeloproliferative and myelodysplastic disorders as well as acute myelogenous leukemia, it has rarely been reported to terminate as acute lymphoblastic leukemia (ALL). Only five cases have been reported in the literature; two from the pediatric literature and only three from the adult literature. Of the three adult cases, two were defined by light microscopy alone. Among the cases with follow-up (3/5), all died within 2 weeks to 2 months of diagnosis. We report an additional case in an adult; the ALL was defined by morphology, flow cytometric immunophenotyping, and cytogenetic analysis. The interval from diagnosis of AMF to ALL was 3 months. Our patient was treated with standard therapy for ALL, was in complete remission at last follow-up (3 months off maintenance therapy), and represents the only reported case who attained a complete remission. There are too few cases to determine the prognostic significance of termination of AMF in an acute leukemia of lymphoid origin vs. myeloid origin.
Passive-Active Immunization in Certain Viral Diseases That Produce Permanent Immunity
Authors: Joseph Stokes
Journal: New England Journal of Medicine (1956)

THE name of Kenneth Blackfan, coupled almost inseparably with that of James L. Gamble, immediately brings to mind what one may call, using an appropriate terminology of astronomy, a galactic event in stellar space implemented by the initiation of full-time clinical chairs at Johns Hopkins Medical School. For in the relative pediatric void then existing in the United States it was as though an active mass of high energy and brilliance that was centered about John Howland, although obtaining more energy from the various elements than from the central figure, had suddenly burst into a number of galaxies, each in . . .
Long-Term Administration of Endothelin Receptor Antagonist Improves Coronary Endothelial Function in Patients With Early Atherosclerosis
Authors: Martin Reriani, Eugenia Raichlin, Abhiram Prasad, Verghese Mathew, Geralyn M. Pumper, Rebecca E. Nelson, Ryan Lennon, Charanjit Rihal, Lilach O. Lerman, Amir Lerman
Journal: Circulation (2010)

Endothelin (ET-1) is one of the most potent vasoconstrictors and plays a seminal role in the pathogenesis of atherosclerosis. The present study was designed to test the hypothesis that long-term treatment with an endothelin-A (ET(A)) receptor antagonist improves coronary endothelial function in patients with early coronary atherosclerosis.Forty-seven patients with multiple cardiovascular risk factors, nonobstructive coronary artery disease, and coronary endothelial dysfunction were randomized in a double-blind manner to either the ET(A) receptor antagonist atrasentan (10 mg) or placebo for 6 months. Coronary endothelium-dependent vasodilation was examined by infusing acetylcholine (10(-6) to 10(-4) mol/L) in the left anterior descending coronary artery. N(G)-monomethyl-l-arginine was administered to a subgroup of patients. Endothelium-independent coronary flow reserve was examined by use of intracoronary adenosine and nitroglycerin. Baseline characteristics and incidence of adverse effects were similar between the 2 groups. There was a significant improvement in percent change of coronary blood flow in response to acetylcholine at 6 months from baseline in the atrasentan group compared with the placebo group (39.67%, 95% confidence interval 23.23% to 68.21%, versus -2.22%, 95% confidence interval -27.37% to 15.28%; P<0.001). No significant difference in the percent change of coronary artery diameter or change in coronary flow reserve was demonstrated. Coronary blood flow, coronary artery diameter, and the effect of N(G)-monomethyl-l-arginine were similar between the groups at baseline and at 6 months.This study demonstrates that 6-month treatment with atrasentan improves coronary microvascular endothelial function and supports the role of the endogenous endothelin system in the regulation of endothelial function in early atherosclerosis in humans. Clinical Trial Registration Information- URL: http://www.clinicaltrials.gov. Unique identifier: NCT00271492.
Impaired Kupffer cell function precedes development of secondary amyloidosis.
Authors: A Fuks, D Zucker-Franklin
Journal: The Journal of Experimental Medicine (1985)

It has been demonstrated previously that the acute phase reactant, serum amyloid A (SAA), is subject to degradation by surface membrane-associated proteinases of peripheral blood monocytes. However, monocytes obtained from the blood of patients with amyloidosis degraded SAA incompletely, leaving a cleavage product that, biochemically and immunologically, resembled the amyloid protein A (AA) deposited in their tissues. To investigate the role of fixed macrophages in amyloidogenesis and to establish more definitively that amyloid deposition is attributable to faulty processing of the precursor protein rather than aberrant synthesis, secondary amyloidosis was induced in C57BL/6J mice by serial injections of casein. Kupffer cells (KC) were isolated from livers of mice that had received 0, 8, 13, 18, and greater than 30 injections of the stimulant. The cells were cultured with SAA for 4, 8, and 18 h and then subjected to electron microscopy and enzyme analyses. The medium was analyzed by SDS-PAGE to determine the amount of residual SAA and/or the appearance of AA. KC of healthy animals degraded SAA completely whereas KC of stimulated mice showed increasing amounts of residual SAA and the appearance of the AA cleavage product. The AA peptide appeared in KC cultures early during the course of casein injections and before any amyloid could be demonstrated in the organs of the stimulated mice. The addition of KC isolated from healthy mice to cultures that had produced AA eliminated the abnormal peptide. The results, indicate that defective KC function precedes amyloidosis. The abnormal AA cleavage product formed by such cells is still susceptible to hydrolysis by normal cells. In addition, ultrastructural evidence is presented that suggests that KC may also play a role in fibrillogenesis of the AA protein.
INSTANTANEOUS WAVE-FREE RATIO (IFR) HAS A SIMILAR DIAGNOSTIC EFFICIENCY TO FRACTIONAL FLOW RESERVE (FFR) IN SINGLE AND MULTI-VESSEL DISEASE: SECONDARY ANALYSIS OF ADVISE (ADENOSINE VASODILATOR INDEPENDENT STENOSIS EVALUATION) STUDY
Authors: Justin E. Davies, Sayan Sen, Javier Escaned, Carlo Di Mario, Rafael Mila, Iqbal Malik, darrel francis, Alun Hughes, Jamil Mayet
Journal: Journal of the American College of Cardiology (2012)
Aortic and Mitral Regurgitation
Authors: William Grossman
Journal: JAMA (1984)

AORTIC and mitral regurgitation represent two relatively common causes of left ventricular volume overload. Although diseases that produce aortic or mitral regurgitation may also produce narrowing of the valve orifice, resulting in some degree of stenosis, pure aortic and/or mitral regurgitation (without associated valve stenosis) occur commonly, and this review focuses on the natural history and treatment of patients with pure aortic and/or mitral regurgitation. In both aortic and mitral regurgitation, the left ventricle must accommodate an increased amount of blood in diastole: in aortic regurgitation, this extra diastolic blood is the direct consequence of the aortic valvular leak, whereas in mitral regurgitation, the extra diastolic load is the indirect result of the fact that blood regurgitated from the left ventricle into the left atrium during systole must ultimately return to the left ventricle during subsequent diastoles. The diastolic volume overload of aortic and mitral regurgitation initially increases diastolic wall
Absence of health insurance is associated with decreased life expectancy in patients with cystic fibrosis.
Authors: J R Curtis, W Burke, A W Kassner, M L Aitken
Journal: American Journal of Respiratory and Critical Care Medicine (1997)

Life expectancy for individuals with cystic fibrosis (CF) has increased dramatically in the last 30 yr, but it is unclear whether the improved survival has applied equally to individuals with different health insurance status. We developed a retrospective inception cohort of all 189 patients with CF born 1/1/55 to 12/31/70 who had at least one hospitalization at a university referral center. The median survival for patients with CF who were without health insurance was 6.1 yr compared with 20.5 yr for those with Medicaid and 20.5 yr for those with private insurance. Using multivariate Cox regression, health insurance and increased socioeconomic status were independently associated with longer survival. The adjusted relative risk of death was greater for the absence of health insurance than for factors previously shown to predict mortality in individuals with CF (female sex and presentation with meconium ileus). In summary, the absence of health insurance was associated with increased mortality rate in children with CF and was a stronger predictor of mortality than variables previously shown to be associated with mortality for CF. If increasing numbers of children with CF lose health insurance coverage, our results suggest that their life expectancy will decrease dramatically.
When NK cells overcome their lack of education
Authors: Baptiste N. Jaeger, Eric Vivier
Journal: Journal of Clinical Investigation (2012)

Cells of the immune system have evolved various molecular mechanisms to sense their environment and react to alterations of self. NK cells are lymphocytes with effector and regulatory functions, which are remarkably adaptable to changes in self. In a study published in this issue of the JCI, Tarek and colleagues report the clinical benefits of manipulating NK cell adaptation to self in an innovative mAb-based therapy against neuroblastoma (NB). This novel therapeutic strategy should stimulate further research on NK cell therapies.
Use of Morphine in Terminating Chills and as an Antipyretic
Authors: Walter E. Marchand
Journal: New England Journal of Medicine (1955)

FOURTEEN years ago I gave 10 mg. of morphine intravenously to a woman with severe abdominal pain and a shaking chill due to a septic abortion. The chill promptly stopped. A short while later the patient thanked me, noting that the pain had actually been more endurable than the chill and that she was glad that I had been able to stop the chill. When, several days later, another patient presented herself at the hospital with a shaking chill in addition to pain from a huge gluteal abscess, I decided again to give morphine intravenously for the pain, but more . . .
Femoral Artery Injury During Inguinal Herniorrhaphy
Authors: Elliott Mendenhall
Journal: JAMA (1963)

INJURY to the femoral vessels during inguinal herniorrhaphy is of constant concern to the general surgeon. In most repairs of the floor of the inguinal canal, sutures are placed through the inguinal ligament directly above the femoral artery and vein. The Cooper's ligament repair as described by McVay is particularly apt to endanger these vessels. Because of this unique anatomic arrangement and because of the vast numbers of inguinal hernias which are repaired, one would expect this complication to occur with some frequency. This does not seem to be the case, however, and a review of the literature for the last five years did not yield a single reported case! Marsden<sup>1</sup>has presented a three-year review of 2,000 inguinal herniorrhaphies with an infection rate of 6%. There were 24 patients who developed thrombotic incidents, including cornary, cerebral, axillary, and calf vessels and three additional patients without leg symptoms who
The molecular basis of T cell acute lymphoblastic leukemia
Authors: Pieter Van Vlierberghe, Adolfo Ferrando
Journal: Journal of Clinical Investigation (2012)

T cell acute lymphoblastic leukemias (T-ALLs) arise from the malignant transformation of hematopoietic progenitors primed toward T cell development, as result of a multistep oncogenic process involving constitutive activation of NOTCH signaling and genetic alterations in transcription factors, signaling oncogenes, and tumor suppressors. Notably, these genetic alterations define distinct molecular groups of T-ALL with specific gene expression signatures and clinicobiological features. This review summarizes recent advances in our understanding of the molecular genetics of T-ALL.
The Language of Life: An Introduction to the Science of Genetics
Authors: A. Donald Merritt
Journal: JAMA (1966)

The Language of Life is described as a lucid and literate primer for laymen—a challenging assertion. I am impressed, however, as a layman and physician, by the manner in which this purpose has been accomplished. The writing was done by Muriel Beadle, whose past experience as a newspaper writer is clearly seen in the brisk, rapid unfolding of the authors' story of man's quest for knowledge in the field of genetics. With considerable success they present a continuum from mendelism to the modern concepts of a gene and the biochemistry of the genetic code. They draw information and examples from the fields of evolution, anthropology, social sciences, and medicine, with particular emphasis on classical and biochemical genetics. The language is simple, clear, and often personal. Interspersed are opinions and anecdotes which add considerable color to a scientific tale, although some of the editorial comments seemed unnecessarily pointed.  The text is
Mesenteric Th1 polarization and monocyte TNF-α production: First steps to systemic inflammation in rats with cirrhosis
Authors: Leticia Muñoz, Agustín Albillos, Mónica Nieto, Eduardo Reyes, Lourdes Lledó, Jorge Monserrat, Eva Sanz, Antonio de la Hera, Melchor Alvarez‐Mon
Journal: Hepatology (2005)

A systemic inflammatory state with increased circulating tumor necrosis factor alpha (TNF-α) has been related to the bacterial infection susceptibility and hemodynamic derangement of patients with cirrhosis. We compared the activation status of immune cell subpopulations defined by 4-color cytometry in mesenteric and peripheral lymph nodes and blood of rats with CCl4-cirrhosis to define the immune response initiation site, the T-cell and monocyte contribution to pro-inflammatory cytokine production, as well as the pathogenic role of enteric bacteria in the cirrhosis immune response. Th1 cells and monocytes were expanded in the mesenteric nodes (P < .001) and blood (P < .001) of rats with cirrhosis, and activated to produce interferon gamma (P < .0001) and TNF-α (P < .0001), respectively. The greater numbers of recently activated CD134+ Th cells in mesenteric nodes compared with blood, the correlation between their numbers in mesenteric nodes and blood (r = 0.66, P < .001), and the expansion of activated CD45RC− Th cells, which are unable to re-enter lymph nodes, in mesenteric nodes but not in blood or axillary nodes points to mesenteric nodes as the origin site of activated Th cells. Abrogation of bacterial translocation by bowel decontamination reduced the number of activated Th cells and monocytes, and normalized interferon gamma production by Th cells and TNF-α production by monocytes in mesenteric nodes and blood, respectively. In conclusion, in cirrhosis, enteric bacteria start off an orchestrated immune response cascade in mesenteric nodes involving Th1 polarization and monocyte activation to TNF-α production. Later, the recirculation of these activated effector immune cells into blood promotes systemic inflammation. (HEPATOLOGY 2005;42:411–419.)
Pulmonary Dysfunction in Rheumatoid Disease.
Authors: S. T. Frank
Journal: Annals of Internal Medicine (1968)

s1 May 1968Pulmonary Dysfunction in Rheumatoid Disease.S. T. Frank, M.D., J. G. Weg, M.D. (Associate), R. E. Walsh, M.D., L. E. Harkleroad, B.S., R. F. Fitch, M.D., F.A.C.P.S. T. Frank, M.D.Search for more papers by this author, J. G. Weg, M.D. (Associate)Search for more papers by this author, R. E. Walsh, M.D.Search for more papers by this author, L. E. Harkleroad, B.S.Search for more papers by this author, R. F. Fitch, M.D., F.A.C.P.Search for more papers by this authorAuthor, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-68-5-1194_2 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptForty-one consecutive patients with classical or definite rheumatoid arthritis were studied in an attempt to determine the prevalence of pulmonary dysfunction in rheumatoid disease. Routine spirometry, lung volumes, and diffusion studies were performed on all patients. Arterial blood gas determinations were made on those with diffusion abnormalities, and an attempt was made to obtain histological correlation via percutaneous lung biopsy. Abnormal diffusion studies were noted in 17 patients (41.4%). Arterial hypoxemia was present in eight patients at rest and five desaturated with exercise. Chest roentgenograms were abnormal in eight (47%), and only six patients (39%) admitted to dyspnea on direct... This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAuthors: S. T. Frank, M.D.; J. G. Weg, M.D. (Associate); R. E. Walsh, M.D.; L. E. Harkleroad, B.S.; R. F. Fitch, M.D., F.A.C.P.Affiliations: Lackland Air Force Base, Texas PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byInterstitial Rheumatoid Lung Disease 1 May 1968Volume 68, Issue 5Page: 1194-1195KeywordsArterial blood gasBiopsyDyspneaExerciseHypoxiaLungsPulmonary diseasesRheumatoid arthritisSpirometryThorax ePublished: 1 December 2008 Issue Published: 1 May 1968 PDF downloadLoading ...
Hammering psychiatric practice: Secure in our insecurity
Authors: A J D Macdonald
Journal: BMJ (1993)

standard that centres could embellish as they wished.This advanced life support course was piloted in 1992 and is now available to centres that conform with the requirements.Advertisements often state that courses conform to the guide- lines of the European and British Resuscitation Councils.However, some centres fail to satisfy the standards laid down for the new advanced life support course.The Resuscitation Council (UK) has therefore agreed to approve on request every course that satisfies the advanced life support requirements.These courses will be given a number that can be checked with the coordinator at our London office and successful participants on the courses will be entitled to receive a certificate from the Resuscitation Council.
Diagnosis of thoracic aortic dissection. Magnetic resonance imaging versus transesophageal echocardiography.
Authors: C A Nienaber, R P Spielmann, Y von Kodolitsch, V Siglow, A Piepho, T Jaup, V Nicolas, P Weber, H J Triebel, W Bleifeld
Journal: Circulation (1992)

BACKGROUND Aortic dissection requires prompt and reliable diagnosis to reduce the high mortality. The purpose of this study was to assess the reliability of both ECG-triggered magnetic resonance imaging (MRI) and transesophageal two-dimensional echocardiography combined with color-coded Doppler flow imaging (TEE) for the diagnosis of thoracic aortic dissection and associated epiphenomena. METHODS AND RESULTS Fifty-three consecutive patients with clinically suspected aortic dissection were subjected to a dual noninvasive imaging protocol in random order; imaging results were compared and validated against the independent morphological "gold standard" of intraoperative findings (n = 27), necropsy (n = 7), and/or contrast angiography (n = 53). No serious side effects were encountered with either imaging method. In contrast to a precursory screening transthoracic echogram, the sensitivities of both MRI and TEE were 100% for detecting a dissection of the thoracic aorta irrespective of its location. The specificity of TEE, however, was lower than the specificity of MRI for a dissection (TEE, 68.2% versus MRI, 100%; p less than 0.005), which resulted mainly from false-positive TEE findings confined to the ascending segment of the aorta (TEE, 78.8% versus MRI, 100%; p less than 0.01). In addition, MRI proved to be more sensitive than TEE in detecting the formation of thrombus in the false lumen of both the aortic arch (p less than 0.01) and the descending segment of the aorta (p less than 0.05). There were no discrepancies between the two imaging techniques in detecting the site of entry to a dissection, aortic regurgitation, or pericardial effusion. CONCLUSIONS Both MRI and TEE are atraumatic, safe, and highly sensitive methods to identify and classify acute and subacute dissections of the entire thoracic aorta. TEE, however, is associated with lower specificity for lesions in the ascending aorta. These results may still favor TEE as a semi-invasive diagnostic procedure after a precursory screening transthoracic echogram in suspected aortic dissection, but they establish MRI as an excellent method to avoid false-positive findings. Anatomic mapping by MRI may emerge as the most comprehensive approach and morphological standard to guide surgical interventions.
Significance of Extreme Elevation of Erythrocyte Sedimentation Rate
Authors: Leo R. Zacharski
Journal: JAMA (1967)

The clinical significance of an erythrocyte sedimentation rate (ESR) elevation of 100 mm or more in one hour was determined for 263 patients. Malignancy was diagnosed in 152 (58%) of these, and was associated with metastasis in all but two instances. Inflammatory diseases (infection or collagen disease) accounted for the elevation in 66 (25%) of the patients, whereas renal disease was the cause in 22 (8%). For only 17 (6%) of the patients was the diagnosis initially indeterminate or not related to the degree of ESR elevation. A serious disease was subsequently diagnosed in only three of these 17 patients. The diagnosis was not apparent from the history, physical examination, and basic laboratory tests of 12 patients. Tests that ultimately led to the diagnosis included the serum protein electrophoresis, bone marrow examination, roentgenograms of the abdomen or lumbar spinal column, and blood urea determinations.
The Natural History of Lone Atrial Flutter
Authors: Sean C. Halligan, Bernard J. Gersh, Robert D. Brown, A. Gabriela Rosales, Thomas M. Munger, Win-Kuang Shen, Stephen C. Hammill, Paul A. Friedman
Journal: Annals of Internal Medicine (2004)

The natural history of atrial flutter is not well defined.To report the risk for stroke, conversion to atrial fibrillation, and anticoagulation for lone atrial flutter.Retrospective cohort analysis.A clinically based longitudinal study of inpatients and outpatients with atrial flutter.The authors compared the stroke rate in 59 patients with atrial flutter with rates in a sample in which age- and sex-specific ischemic cerebrovascular event rates were determined and in a sample of nonhypertensive patients with lone atrial fibrillation. The risk for developing atrial fibrillation after presenting with atrial flutter is also reported.Electrocardiograms and clinical data were collected and reviewed for each study participant.After adjustment for age and sex, patients with atrial flutter had a higher incidence of thromboembolic events than the sample control patients and patients with atrial fibrillation. Atrial fibrillation developed in 56% of patients with atrial flutter.Lone atrial flutter has a stroke risk at least as high as lone atrial fibrillation and carries a higher risk for subsequent development of atrial fibrillation than in the general population. Anticoagulation should be considered for all patients with atrial flutter who are older than 65 years of age.