tbuckley/cabot-search huggingface dataset:
A molecular defect in two families with hemolytic poikilocytic anemia: reduction of high affinity membrane binding sites for ankyrin.
Authors: P Agre, E P Orringer, D H Chui, V Bennett
Journal: Journal of Clinical Investigation (1981)

Patients from two families with chronic hemolytic anemia have been studied. The erythrocytes are very fragile and appear microcytic with a great variety of shapes. Clinical evaluation failed to identify traditionally recognized causes of hemolysis. Sodium dodecyl sulfate polyacrylamide gel electrophoresis (SDS-PAGE) showed no significant abnormality of the major polypeptide bands. Erythrocytes spectrin-ankyrin and ankyrin-membrane interactions were analyzed with 125I-labeled spectrin, 125I-labeled ankyrin, and inside-out vesicles. Patients' vesicles bound 125I-spectrin normally. Likewise, patients' spectrin and ankyrin competed normally for the binding sites on control membranes. None of the individual components appeared to have abnormal thermal sensitivity. Ankyrin-stripped, inside-out vesicles prepared from the patients bound less 125I-ankyrin than did vesicles prepared from normals (P less than 0.05 for all corresponding points in the high-affinity region). Scatchard analysis showed the most significant abnormality to be a 50% reduction in the high affinity ankyrin binding sites. Similar experiments were performed with blood from patients with spherocytosis and splenectomized controls, but no abnormalities were detected. The water soluble 43,000-dalton fragments of band 3 (the high-affinity ankyrin binding sites) were prepared from one of the patients and competed normally for 125I-ankyrin binding in solution. This suggests that the primary structural defect is a reduction in the number of high affinity membrane binding sites for ankyrin, and is consistent with an abnormal organization of band 3 in the membrane.
Evidence based medicine: does it make a difference?
Authors: Benjamin Druss
Journal: BMJ (2005)

Evidence based medicine: does it make a difference? Use wiselyEditor-The term "evidence based medicine" entered the scientific lexicon only a little more than a decade ago. 1 What caused its remarkable spread, and what are the implications of its broad and rapid diffusion?The team that coined the term at first considered using the phrase "scientific medicine" but rejected it because it implied that other approaches were by definition unscientific. 2However, critics have argued that the term evidence based medicine carries a similar moral valence and linguistic slipperiness. 3 Who could argue against the notion of providing care that integrates individual clinical skill and the best external evidence? 4 Originally developed as a method for teaching medical residents, evidence based medicine is being applied ever more broadly to the organisation and delivery of medical services.Multiple stakeholders now seek to assume its mantle for purposes that often contradict its original intent.Managers, equating lack of evidence with lack of effectiveness, use it as a rationale for cutting services.Industry generates evidence of questionable quality to promote its products.Medical researchers come to believe that they hold a monopoly on generating and interpreting evidence.Evidence based medicine, developed as a means of taming the unscientific and messy world of clinical practice, has itself entered the unscientific and messy world of politics. 5ike any technology, evidence based medicine carries risks and benefits and can be used appropriately or inappropriately.Overly inclusive definitions threaten to deprive the term of meaning, and unchecked use increases the risk of misuse.In the past decade, evidence based medicine has contributed much to how we teach, deliver, and think about clinical services.In the coming decade, we must continue to ensure that it is not only used widely but wisely.
Cl- absorption across the thick ascending limb is not altered in cystic fibrosis mice. A role for a pseudo-CFTR Cl- channel.
Authors: P Marvão, M C De Jesus Ferreira, C Bailly, M Paulais, M Bens, R Guinamard, R Moreau, A Vandewalle, J Teulon
Journal: Journal of Clinical Investigation (1998)

The cortical thick ascending limb (CTAL) absorbs Cl- via a Na+-K+-Cl- cotransport at the apical membrane and several Cl- channels at the basolateral membrane, including a 9-pS channel having several properties of the cystic fibrosis transmembrane conductance regulator (CFTR). Having checked that CFTR mRNA is present in the mouse CTAL, we investigated whether this channel is a CFTR molecule by applying the patch-clamp technique to CTALs microdissected from CFTR knockout mice (cftrm1Unc). The 9-pS channel was active in cell-attached patches from tubules of mice homozygous for the disrupted cftr gene [CFTR (-/-)] at the same frequency and with the same activity (NPo) as in normal [CFTR (+/+)] or heterozygous [CFTR (+/-)] mice. The conductive properties of the channel, studied on inside-out patches, were identical in CFTR (-/-), CFTR (+/+), and CFTR (+/-) tubules, as were the sensitivities to internal pH and internal ATP, two typical features of this channel. In addition, the Cl- absorption in isolated, microperfused CTALs and the Na+-K+-Cl- cotransport activity were identical in CFTR (-/-), CFTR (+/+), and CFTR (+/-) mice. These results show that the 9-pS Cl- channel is distinct from CFTR, and that the CFTR protein has no influence on the Cl- absorption in this part of the renal tubule.
Role of Protease-activated Receptor-2 in Idiopathic Pulmonary Fibrosis
Authors: Malgorzata Wygrecka, Grazyna Kwapiszewska, Ewa Jablonska, Susanne von Gerlach, Ingrid Henneke, Dariusz Zakrzewicz, Andreas Guenther, Klaus T. Preissner, Philipp Markart
Journal: American Journal of Respiratory and Critical Care Medicine (2011)

Rationale: Activation of the coagulation cascade has been demonstrated in pulmonary fibrosis. In addition to its procoagulant function, various coagulation proteases exhibit cellular effects that may also contribute to fibrotic processes in the lung.Objective: To investigate the importance of protease-activated receptor (PAR)-2 and its activators, coagulation factor VIIa (FVIIa)/tissue factor (TF), in the development of idiopathic pulmonary fibrosis (IPF).Methods: Expression and localization of PAR-2 and its activators were examined in IPF lung tissue. The ability of PAR-2 to mediate various cellular processes was studied in vitro.Measurements and Main Results: Expression of PAR-2 was strongly elevated in IPF lungs and was attributable to alveolar type II cells and fibroblasts/myofibroblasts. Transforming growth factor-β1, a key profibrotic cytokine, considerably enhanced PAR-2 expression in human lung fibroblasts. FVIIa stimulated proliferation of human lung fibroblasts and extracellular matrix production in a PAR-2–dependent manner, but did not initiate differentiation of fibroblasts into myofibroblasts. PAR-2/FVIIa-driven mitogenic activities were mediated via the p44/42 mitogen-activated protein kinase pathway and were independent of factor Xa and thrombin production. Proproliferative properties of FVIIa were markedly potentiated in the presence of TF and abrogated by TF antisense oligonucleotides. Hyperplastic alveolar type II cells overlying fibroblastic foci were found to be the source of FVII in IPF lungs. Moreover, TF colocalized with PAR-2 on fibroblasts/myofibroblasts in IPF lungs.Conclusions: The PAR-2/TF/FVIIa axis may contribute to the development of pulmonary fibrosis; thus, interference with this pathway confers novel therapeutic potential for the treatment of IPF.
232 EXPANDED CD14HI/CD16+ MONOCYTES IN ACUTE LIVER INJURY (ALI): ANGELS OR DEMONS?
Authors: R.D. Abeles, C.G. Antoniades, G.K. Manakkat Vijay, N. Vergis, J.R. Maggs, W. Bernal, D.L. Shawcross, J.A. Wendon, D. Vergani
Journal: Journal of Hepatology (2012)
Cardioprotective effects of mioflazine during 1 h normothermic global ischaemia in the canine heart
Authors: W. FLAMENG, R. XHONNEUX, H. VAN BELLE, M. BORGERS, A. VAN DE WATER, L. WOUTERS, J. WIJNANTS, F. THONE, P. VAN DAELE, P. A J JANSSEN
Journal: Cardiovascular Research (1984)

The cardioprotective effects of mioflazine, a recently developed cardiovascular drug, were investigated in 41 anaesthetised open chest Beagle dogs subjected to 1 h normothermic global myocardial ischaemia. The severity of the model is evidenced by the finding that only one out of 20 control dogs could be weaned from extracorporeal bypass. Oral pretreatment with mioflazine (2.5 mg·kg−1) resulted in complete functional recovery in 17 out of 20 animals. Biochemical analysis of left ventricular biopsies taken before, during and after aortic cross clamping showed a preservation of purines and a better recovery of ATP, ATP/ADP × Pi ratio and energy charge (p<0.05) in the pretreated animals. Morphological and cytochemical assessment of the myocardium demonstrated that the ultrastructure of the sarcolemma and its calcium binding capacity is remarkably well preserved in the drug treated animals. These results indicate a strong cardioprotective effect of mioflazine. The biochemical, cytochemical and ultrastructural findings suggest an interaction of the drug with the sarcolemma.
Adrenaline and non-life threatening allergic reactions: Intramuscular adrenaline is safe
Authors: Jo A Douglass, Robyn E O'Hehir
Journal: BMJ (2003)

EDITOR—The life saving effects of adrenaline in severe allergic reactions are well established, so Johnston et al's lesson of the week cautioning against using adrenaline in “non-life threatening” conditions is worrying.1 2 Anaphylaxis or angioedema of the upper airway is life threatening, and avoiding or delaying appropriate treatment is clearly associated with adverse outcomes, …
Shall The Journal be Removed to Washington?
Authors: Chas. G. Bacon
Journal: JAMA (1891)

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 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) Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More 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
Adult Chronic Bronchitis--the Infective Factor and its Treatment
Authors: G. Edwards, A. R. Buckley, E. C. Fear, G. M. Williamson, K. Zinnemann
Journal: BMJ (1957)

The Subcommittee wishes to express its thanks to those sur- geons who allowed their patients to be included in the trial.It is indebted to Messrs. Glaxo (Dr
MIGRATION AND HEALTH
Authors: J. S. Purdy
Journal: BMJ (1930)

608 -ROM 27t I9361 I ~~~~~~~~~~~~~~~~~~~~~~~~MEDICAL JouRCNAL hiad developed subsequently to the zoszter eruption.The questioni naturally arose whetlher the encephalomyelitis was due to thie zoster virus or not, but siniec botlh the vesicle fluid collecte(d fronm the cruptioni anid the cerebro-spinal fluid fixed complement in the presence of known zoster coinvalescent serurms, there wvas little doubt that the case was in' fact one of zoster cnceplhalomyelitis.It is true that the reactioni giveni bv the cerebro-spinal fluid was not as stroiig as tlhat obtained withl the vesicle fluid, but it was nevertlheless quite definite, and cerebro-spinial fluids from cases of encephalomyelitis complicatingdiphithelria anid scarlet fever gave entirely negative results.Recently I have lhad the opportulnity 6f examiniing, the serum of inidividuals wlho lhad recovered from psittacosis for hlc-presence of specific complement-fixinig antibodies, anid lhere.againi I have obtailled evidence of a specific reaction.Psittacosis may not be so rare a condition as is imagined, and e apart from extensive ouitbreaks like the recent one, wlhere tlhe iiunuber anid severity of the cases inevitably attracted attentioni, sporadic cases probably occur from time to time and pass ulnnioticed.The inioculation of animals is too slow a method to be of practical use as a diagnostic procedure, anid a specific serum reaction would be a great help in clearing up the diagnosis.It may be said that the examnples giren are both of rather rare conditions, but the principle is nevertlheless of genleral application.In this brief an-id rather sketchy survey of the present position of research in the realm of filterable viruses an attempt has been made to show that something of practical imporlt has been achieved.Un- doubtedly an eilormous amount remains to be done, but the niumber of workers in this fiefd is steadily increasing, and on every hand a determin-ed effort is being made to gaini a clearer understanding of this group of viruses.Some of the problenms seem almost inisoluble, but it must nllot be for(rotten that the commonplace in bacteriology to-day must have appeared equally obscure to those who lireccde(I Pasteur.
HLA antigens in Turkish race with rheumatic heart disease [see comment].
Authors: M Ozkan, M Carin, G Sönmez, M Senocak, M Ozdemir, C Yakut
Journal: Circulation (1993)

Rheumatic valvular disease has been reported to be associated with HLA antigens. To determine whether genetic factors could be involved in the pathogenesis of rheumatic heart disease (RHD), we analyzed the distribution of HLA-A, HLA-B, and HLA-DR antigens in Turkish patients with chronic rheumatic heart disease.The association of class I and class II HLA antigens was examined in 107 ethnic Turkish patients with chronic RHD. The diagnosis was supported by echocardiography, cardiac catheterization, angioventriculography, and histological findings in patients who underwent valve replacement. Two hundred three control subjects, also of Turkish origin, were chosen. The phenotypes B16, DR3, and DR7 were encountered in a significantly higher frequency in patients with RHD compared with the control population (corrected p < 0.05, p < 0.00005, and p < 0.0005, respectively). There also was a decrease in the antigen frequency of DR5 in patients compared with controls (corrected p < 0.005).The results are consistent with the hypothesis that susceptibility to RHD is genetically linked, and this in turn may be associated mainly with HLA class II antigens and weakly with class I antigens, with DR3, DR7, and B16 influencing susceptibility and DR5 conferring protection.
Book Review The Merck Druggernaut: The Inside Story of a Pharmaceutical Giant By Fran Hawthorne. 290 pp. New York, John Wiley &amp; Sons, 2003. $24.95. 0-471-22878-8 The Big Fix: How the Pharmaceutical Industry Rips Off American Consumers By Katharine Greider. 189 pp. New York, PublicAffairs, 2003. $14. 1-58648-185-1
Authors: David J. Rothman
Journal: New England Journal of Medicine (2003)
Patent Foramen Ovale and Cryptogenic Stroke in Older Patients
Authors: Michael Handke, Andreas Harloff, Manfred Olschewski, Andreas Hetzel, Annette Geibel
Journal: New England Journal of Medicine (2007)

Studies to date have shown an association between the presence of patent foramen ovale and cryptogenic stroke in patients younger than 55 years of age. This association has not been established in patients 55 years of age or older.We prospectively examined 503 consecutive patients who had had a stroke, and we compared the 227 patients with cryptogenic stroke and the 276 control patients with stroke of known cause. We examined the prevalences of patent foramen ovale and of patent foramen ovale with concomitant atrial septal aneurysm in all patients, using transesophageal echocardiography. We also compared data for the 131 younger patients (< 55 years of age) and those for the 372 older patients (> or = 55 years of age).The prevalence of patent foramen ovale was significantly greater among patients with cryptogenic stroke than among those with stroke of known cause, for both younger patients (43.9% vs. 14.3%; odds ratio, 4.70; 95% confidence interval [CI], 1.89 to 11.68; P<0.001) and older patients (28.3% vs. 11.9%; odds ratio, 2.92; 95% CI, 1.70 to 5.01; P<0.001). Even stronger was the association between the presence of patent foramen ovale with concomitant atrial septal aneurysm and cryptogenic stroke, as compared with stroke of known cause, among both younger patients (13.4% vs. 2.0%; odds ratio, 7.36; 95% CI, 1.01 to 326.60; P=0.049) and older patients (15.2% vs. 4.4%; odds ratio, 3.88; 95% CI, 1.78 to 8.46; P<0.001). Multivariate analysis adjusted for age, plaque thickness, and presence or absence of coronary artery disease and hypertension showed that the presence of patent foramen ovale was independently associated with cryptogenic stroke in both the younger group (odds ratio, 3.70; 95% CI, 1.42 to 9.65; P=0.008) and the older group (odds ratio, 3.00; 95% CI, 1.73 to 5.23; P<0.001).There is an association between the presence of patent foramen ovale and cryptogenic stroke in both older patients and younger patients. These data suggest that paradoxical embolism is a cause of stroke in both age groups.
Performance, length, and cost of endoscopic retrograde cholangiopancreatography guide wires: which is the best mix of features?
Authors: H. Neuhaus
Journal: Endoscopy (2006)

The use of guide wires has become an important part of endoscopic retrograde cholangiopancreatography (ERCP) procedures. Their successful use depends mainly on their physical attributes and correct manipulation. They can be helpful, even indispensable, for selective cannulation, stricture access, and accessory exchange. Cannulation is usually performed by careful injection of contrast for visualizing the biliopancreatic anatomy and targeted guidance of the tip of a sphincterotome or an inserted guide wire. This approach should succeed in approximately 90 % of cases [1] [2] [3]. Alternatively, a guide wire can be gently passed in the direction of the targeted duct under endoscopic and fluoroscopic guidance without opacification. A recent randomized trial compared biliary cannulation with a guide wire through a sphincterotome with cannulation using a sphincterotome alone. The success rates were comparably high for both methods but the pancreatitis rate was significantly lower in the guide wire group [4]. All these procedures were performed by a single endoscopist, however, and the results should be confirmed in a multicenter trial.
Pemphigus Vulgaris Successfully Treated with Cyclophosphamide
Authors: ALAN EBRINGER
Journal: Annals of Internal Medicine (1969)

A 49-year-old woman had pemphigus vulgaris for 8 years. She had been treated for 5 years with prednisolone in increasingly high doses, up to 200 mg daily, and had developed numerous and life-threatening side-effects. She was treated over the last 3 years with cyclophosphamide, 200 mg daily, in four separate courses. This allowed gradual reduction in the high maintenance doses of prednisolone and induced remissions.
The Occupational Risk of Cytomegalovirus Infection Among Day-care Providers
Authors: JR Murph, JC Baron, CK Brown, CL Ebelhack, Jr Jf Bale
Journal: JAMA (1991)

We prospectively studied day-care providers at six day-care centers in south-eastern Iowa to determine their occupational risk for primary cytomegalovirus infection and to define epidemiologic risk factors. Ninety-six (38%) of 252 day-care providers were seropositive for cytomegalovirus by latex agglutination at entry into the study. Among 82 seronegative providers available for follow-up, seven seroconversions occurred at only two of the six participating centers, yielding an annualized seroconversion rate of 7.9%. Median time to seroconversion among these providers was 13 months. Using Kaplan-Meier estimates of risk, we determined that the overall risk of seroconversion among providers at various centers ranged from 0% to 22% by 12 months and from 0% to 40% by 16 months. Risk of cytomegalovirus acquisition by providers was independent of race, age, education, the presence of a child at home, or caring for children younger than 2 or 3 years in the day-care center. However, the risk of seroconversion among day-care providers appeared to parallel rates of cytomegalovirus excretion and acquisition among children at each center.
Regulated progression of B lymphocyte differentiation from cultured fetal liver.
Authors: K A Denis, K Dorshkind, O N Witte
Journal: The Journal of Experimental Medicine (1987)

Lymphoid fetal liver cultures (LFLC) are long-term, nontransformed cultures of early B lymphoid lineage cells which appear developmentally blocked at the pre-B stage in vitro. When injected into severe combined immunodeficient (SCID) mice, cells from LFLC could reconstitute splenic B lymphocytes and serum IgM. T lymphocyte reconstitution was not observed and serum IgG levels were very low. IgG3 was the predominant gamma subisotype in the serum of the LFLC-reconstituted mice, indicating impaired class switching in these B lymphocytes. When thymocytes were coinjected with LFLC, the B lymphocytes were able to class switch fully and respond to T-dependent antigens. These serological responses were heterogeneous. This experimental system allows separation of three B lymphocyte developmental stages: early differentiation in vitro, progression to IgM secretion in vivo, and late differentiation dependent upon mature T lymphocytes in vivo. The unique advantage of this system is the ability to regulate the B lymphocyte developmental pathway in a defined, stepwise manner.
Abdominal Bruit after Renal Transplantation
Authors: S. J. Jachuck, R. Wilkinson
Journal: BMJ (1973)

Forty-seven recipients of renal allografts have been studied at varying intervals of up to five years after transplantation. Renal artery bruit occurred in eight of 16 patients observed over the first two post-transplant months and disappeared spontaneously in four of these. The disappearance of the bruit was associated with poor renal function. Renal bruits were audible in 10 patients examined more than two months after transplantation; nine of these were hypertensive and of six in whom arteriography was performed five were shown to have stenosis of the allograft artery. By contrast only eight of 37 patients without abdominal bruit were hypertensive, and arteriography in 10 normotensive patients without bruit showed no stenosis. It is concluded that while a renal artery bruit during the first two months after transplantation may be a marker of good renal blood flow at the time, its presence suggests a poor long-term prognosis since persistence of the murmur indicates that significant stenosis of the allograft artery is likely, while its disappearance is associated with poor renal function.
Biotin Deficiency: An Unusual Complication of Parenteral Alimentation
Authors: Donald M. Mock, Alfred A. deLorimer, William M. Liebman, Lawrence Sweetman, Herman Baker
Journal: New England Journal of Medicine (1981)

BIOTIN deficiency has been documented in human beings only in association with prolonged ingestion of raw egg white. In this paper, we report that clinically important biotin deficiency occurred in a child with short-gut syndrome during parenteral alimentation.Case ReportThe clinical history and growth of this 12-month-old girl were normal until four months of age, when malrotation and midgut volvulus resulted in extensive infarction of the small and large bowel. After the initial bowel resection, her clinical course was complicated by anastomosis failure, peritoneal infection, and bowel obstruction. Ultimately, the patient retained only 30 cm of jejunum, 0.5 cm . . .