tbuckley/cabot-search huggingface dataset:
Asymptomatic homozygous deletional β<sup>0</sup>‐thalassemia in an African individual
Authors: Paula Faustino, Ana Batalha Reis, Helena Feliciano, Lénia Ferrão, Patrícia Pereira, Isabel Picanço, Armandina Miranda, Teresa Seixas, Luísa Romão, Esmeraldina Correia Júnior, João Lavinha
Journal: American Journal of Hematology (2002)

Homozygosity or compound heterozygosity for beta(0)-thalassemia mutations most commonly results in a transfusion-dependent thalassemia major phenotype. In this report, we describe a 55-year-old male, from Guinea-Bissau, that had been asymptomatic and never transfused until being admitted to hospital with anemia, fever, splenomegaly, and asthenia. Following hospital admission, HIV-2 and Mycobacterium tuberculosis infections were diagnosed, and biochemical and molecular studies revealed homozygosity for beta(0)-thalassemia. At the molecular level, this is the first description of homozygosity for the beta(0)-Black 1,393-bp deletion. In this case, the complete absence of beta-globin gene expression seems to be compensated by an unusually high fetal globin gene expression (Hb F 96%). Beta-globin haplotyping results were compatible with the propositus being homozygous for the Black 2 haplotype and for the absence of the XmnI polymorphism at -158 of (G)gamma-globin gene (-/-). Co-inheritance of genetic factors usually associated with high Hb F levels was not detected. Otherwise, the propositus is a heterozygote for the alpha-globin gene 3.7-kb deletion that is a beneficial modulating factor but not sufficient to explain this extremely mild phenotype. This unusual genotype/phenotype association is discussed in terms of the mechanisms underlying hemoglobin switching during development.
The computer's contribution to information content of laboratory data
Authors: E. R. Gabrieli
Journal: JAMA (1966)

Historically, in the process of diagnostic decision making, emphasis was on the skills of effective history taking and physical examination. When deemed necessary, simple laboratory tests (eg, blood count, urinalysis) were also performed by the examiner. This flow of information was a powerful way of making the diagnosis. Admittedly, the ambiguous information content of these data frequently had to be interpreted by nonscientific methods; on the other hand, even these vague data when enriched by the good diagnostician's experience and intuition could lead to remarkably accurate diagnoses. For thousands of years this simple system of information flow was the art of medicine, justifiably the pride of distinguished line of great clinicians, and it should be remembered with considerable nostalgia.  Recent progress in various fields of medical sciences caused a palpable shift in emphasis. Following World War II, the scope of laboratory medicine enlarged at a spectacular rate. The practitioner could
Hemolytic Anemia Complicating Aortic-Valve Surgery
Authors: Bernard Pirofsky, Donald W. Sutherland, Albert Starr, Herbert E. Griswold
Journal: New England Journal of Medicine (1965)

HEMOLYTIC anemia has occasionally been reported as complicating various open-heart surgical procedures performed in laboratory animals1 and human patients.2 3 4 These cases have been characterized by destruction of erythrocytes through mechanical means. We have recently encountered 7 cases of acquired hemolytic anemia after aortic-valve surgery that did not appear to result solely from mechanical breakdown of erythrocytes. The association of positive serologic tests and a characteristic clinical pattern suggests a previously undescribed syndrome. The purpose of the present report is to outline the clinical nature of this state and to indicate a possible autoimmune pathogenesis for the hemolytic process.Case Reports . . .
Decreased peripheral blood γδ T cells in patients with bronchial asthma
Authors: J. Krejsek, B. Král, D. Vokurková, V. Derner, M. Toušková, Z. Paráková, O. Kopecký
Journal: Allergy (1998)

Many cell populations are thought to be involved in the etiopathogenesis of bronchial asthma. We examined by flow cytometry the relative and absolute number of CD3*, CD4*, CD8*γδ TcR* T cells. CD19* B cells; and CD56* natural killer (NK) cells in the peripheral blood of 26 adult patients with difficult‐to‐control asthma (DCA) and 22 patients with minimally symptomatic asthma (MSA). Statistically higher relative and absolute numbers of NK cells (18.39±10.67% and 0.38±0.17×10 9 /l) in comparison with healthy controls (ll.77±8.06% and 0.25±0.19×10 9 /l) and significantly decreased relative and absolute numbers of γδ T cells (3.02±2.16% and 0.06±0.04×10 9 /l) in comparison with controls (5.65+2.90% and 0.13±0.08×10 9 /l) in the DCA patient group were found. After pooling of data from both MSA and DCA patients and dividing the patients according to the presence of allergy, the relative and absolute numbers of 78 T celts were found to be diminished in both the allergy (3.77±2.98 and O.O7±0.O5 ×10 9 /l) and nonallergy (3.06±1.78% and 0.06±0.03 ×10 9 /l) groups in comparison with healthy controls. The reason for the low number of 78 T cells in the peripheral blood of patients suffering from bronchial asthma is under investigation.
Cellular mechanisms of normal growth in the mammalian heart. II. A quantitative and qualitative comparison between the right and left ventricular myocytes in the dog from birth to five months of age.
Authors: M J Legato
Journal: Circulation Research (1979)

This paper describes and contrasts the changes in myocytes taken from the right and left ventricules of dog heart over the first 5 months of life. The development of the two populations of cells differs in important respects: sarcomeric volume, the proportion of the myocyte occupied by the contractile apparatus, and the volume and surface area of the transverse tubular system all were greater on the average in the left than in the right ventricle (P less than 0.001). Other intracellular structures also changed significantly as development progressed, but did so in both chambers; the surface area and surface-to-volume (s/v) ratio of both right and left ventricular myocytes increased with age (P less than 0.001) as did mitochondrial and mitochondrial-myocyte volume (P less than 0.001). The surface area (P less than 0.01) and the s/v ratio of the mitochondria (P less than 0.001) also increased with development. Nuclear and nuclear myocyte volume grew smaller with age in both chambers (P less than 0.002), as did the surface area (P less than 0.001) and the s/v ratio of the nucleus (P less than 0.01). Not only does myocyte composition change quantitatively, striking changes in intracellular architecture and the appearance and arrangement of intracellular organelles occur during postnatal life.
Abstract IA16: Targeting cancer stem cells to overcome chemotherapy resistance
Authors: Ronald J. Buckanovich
Journal: Clinical Cancer Research (2013)

Abstract While most ovarian cancer patients will have a complete response with surgery and chemotherapy, the majority will relapse and die of their disease. The high relapse rate in ovarian cancer after complete clinical response is highly consistent with a cancer stem-like cell (CSC) model. The CSC hypothesis suggests that only rare, inherently chemoresistant cancer cells with stem cell like characteristics are capable of proliferating and differentiating to regenerate the various cell types within a tumor, thereby causing relapse of the disease. In theory and in experimental models, the elimination of CSC can overcome chemotherapy resistance. We are developing novel therapies to specifically target ovarian CSC (OvCSC). Aldehyde Dehydrogenase (ALDH) enzymatic activity was one of the first, and remains one of the best supported CSC markers. ALDH enzymes perform numerous critical functions including the biosynthesis of retinoic acid. Our group and others have found that ALDH identifies a population cancer cells with ovarian CSC (OvCSC) activity. Primary human ALDH+ cells initiate and propagate tumors in mice while ALDH-cells do not. ALDH used in combination with the stem cell marker CD133 enriches CSC isolation. ALDH1A+ or ALDH1A1+CD133+ cells in primary tumor samples predict poor prognosis for ovarian cancer patients [9, 11]. ALDH and CD133 are enriched in tumor specimens immediately following chemotherapy [12]. ALDH1A1 is 100 fold upregulated in chemoresistant ovarian cancer cells and ALDH1A knockdown restores chemosensitivity. Finally, normal ovary epithelial stem cells also express ALDH and CD133, and when mutated for p53 and Rb, these cells gain CSC capacity and initiate serous tumors. Taken together these data strongly support a role for ALDH in serous ovarian CSC. Based on the CSC specific function of ALDH and the function of we generated and screened. We have therefore identified and screened dozens of ALDH inhibitors and molecular analogs of known ALDH inhibitors. Using cell lines with well-defined CSC populations, we have identified several compounds which kill cells expressing CSC markers in vitro. CSC die via necrosis. Lead compounds prevent tumor sphere formation in vitro and restrict tumor initiation in vivo. The novel ALDH inhibitors are highly synergistic with chemotherapy and reverse chemotherapy resistance in primary human tumor xenografts. Taken together our data support ALDH as a CSC specific target and identifies novel compounds as potential new therapeutics. Citation Format: Ronald J. Buckanovich. Targeting cancer stem cells to overcome chemotherapy resistance. [abstract]. In: Proceedings of the AACR Special Conference on Advances in Ovarian Cancer Research: From Concept to Clinic; Sep 18-21, 2013; Miami, FL. Philadelphia (PA): AACR; Clin Cancer Res 2013;19(19 Suppl):Abstract nr IA16.
Hormonal Evaluation of the Patient with an Incidentally Discovered Adrenal Mass
Authors: Jane F. Desforges, Nathan S. Ross, David C. Aron
Journal: New England Journal of Medicine (1990)

THE finding of an adrenal mass in the course of abdominal computerized tomography (CT) performed for other reasons poses an increasingly common clinical problem. The prevalence of such incidentally recognized masses ranges from 0.6 to 1.3 percent.1 2 3 An autopsy study performed in conjunction with one study of CT scans revealed a 1.9 percent prevalence of cortical adenoma in patients for whom no evidence of endocrinopathy was recorded during life.2 In addition, a single pheochromocytoma was found (prevalence, 0.1 percent). Another autopsy study found an 8.7 percent prevalence of adrenocortical adenoma.4 These data suggest that as scanning techniques continue to improve, . . .
THE PROGNOSTIC IMPACT OF ETIOLOGIC SUBGROUP AND BASELINE CLINICAL, HEMODYNAMIC AND NEUROHORMONAL CHARACTERISTICS IN PATIENTS WITH PULMONARY HYPERTENSION AND THE RISK OF PARADOXICAL DETERIORATION IN THE INTERMEDIATE RISK GROUPS: INSIGHTS FROM A SINGLE-CENTER STUDY
Authors: Cihangir Kaymaz, Nertila Poci, Tugba Aktemur, Selcuk Ozturk, Ozgur Y Akbal, Fatih Yilmaz, Hacer Ceren Tokgoz, Ibrahim Halil Tanboga, Nihal Ozdemir
Journal: Journal of the American College of Cardiology (2014)
Casein-specific IL-4- and IL-13-secreting T cells: a tool to implement diagnosis of cow's milk allergy
Authors: B. Michaud, J. Aroulandom, N. Baiz, F. Amat, R. Gouvis-Echraghi, S. Candon, A.-P. Foray, R. Couderc, J.-F. Bach, L. Chatenoud, J. Just
Journal: Allergy (2014)

Background Cow's milk allergy (CMA) is a frequent food allergy in young children. The oral food challenge is the gold standard for diagnosis, and there is currently no reliable biological test. Our aim was to evaluate the diagnostic potential of a functional assay quantifying allergen-specific Th2 cells in CMA children. Methods A total of 29 children aged 2.8–10.5 years underwent a double-blind, placebo-controlled food challenge (DBPCFC) to cow's milk. Blood was collected before performing the DBPCFC, and peripheral mononuclear cells were cultured in an 18-h ELISpot assay with casein, α-lactalbumin, or β-lactoglobulin. Numbers of antigen-specific IL-4- and IL-13-secreting lymphocytes and serum-specific IgE, IgG4, and total IgE levels were assessed. Receiver operating characteristic (ROC) curves were generated. Results A total of 17 (59%) children reacted to cow's milk and were therefore considered as allergic to cow's milk (CMA). The mean number of casein-specific IL-4- and IL-13-secreting T cells was higher in CMA than in non-CMA children (P = 0.009, 0.004, respectively). Moreover, it was inversely correlated with the cumulative dose of cow's milk tolerated (P = 0.003, 0.0009, respectively). ROC curve of combined IL-4 and IL-13 analysis showed an area under the curve of 0.98 (95% CI 0.90–1.06). For a cutoff of 10 IL-4- and 12 IL-13-secreting T cells, sensitivity and negative predictive value were 100%. Conclusions Enumeration of casein-specific IL-4- and IL-13-secreting T cells appears a promising tool to improve diagnosis and, if confirmed in larger studies, could permit less frequent use of the oral food challenge.
Cyclin B as a prognostic factor in low-risk node negative breast cancer.
Authors: A Koliadi, C Nilsson, C Ahlin, M Fjällskog
Journal: Cancer Research (2009)

Abstract Abstract #4161 Background: The cell cycle regulation is a well-controlled mechanism in normal non-transformed cells. Cyclins are important proteins for this regulation. Cyclin B1 plays an essential role as a mitotic cyclin in the entry of mitosis from G2 phase. Previous studies have suggested that cyclin B1 over-expression could be a poor prognostic factor in breast cancer. We wanted to examine cyclin B1 as a prognostic factor in a group of patients with low-risk node negative breast cancer.&amp;#x2028; Material and Methods: We compared women that died from their breast cancer (n=17) with women free from relapse &amp;gt; 8 years after initial diagnosis (n=24). All women had stage I, low-risk breast cancer. The groups were matched regarding tumour size, receptor status, adjuvant chemotherapy and tumor differentiation. Tumor samples were analyzed regarding cyclin B1 expression using immunohistochemistry with commercial antibodies and optical microscopy.&amp;#x2028; Results: The mean percentage of cyclin B1 was significantly higher (p=0,00008) in women that died from their breast cancer (13%) compared with women free from relapse (5%). Mortality was significantly higher (p=0,001) in patients with high cyclin B (85%) than with low cyclin B (21%) using 9% as cut-off value.&amp;#x2028; Conclusion: In our study, we found that low-risk node negative patients with high expression of cyclin B1 had a significantly worse outcome than patients with low expression of cyclin B1. We suggest that cyclin B1 could be a potent prognostic factor in this low-risk patient group. However, further studies are needed to confirm our findings. Citation Information: Cancer Res 2009;69(2 Suppl):Abstract nr 4161.
Neurotransmitter transporters (plus): a promising new gene family
Authors: George R. Uhl
Journal: Trends in Neurosciences (1992)

Neurotransmitter transporter genes encode proteins with 12 putative transmembrane regions, which mediate Na+ -dependent reaccumulation of released neurotransmitters into presynaptic terminals and are the sites of action of important abused and therapeutic drugs. Studies of these genes show promise for improving our understanding of transport mechanisms and modes of drug action, and may even uncover previously unanticipated neurotransmitters.
Returning Astronaut Study Helps Some Reeling Patients
Authors: Mike Mitka
Journal: JAMA (2002)

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
The Signaling Protein Wnt4 Enhances Thymopoiesis and Expands Multipotent Hematopoietic Progenitors through β-Catenin-Independent Signaling
Authors: Isabelle Louis, Krista M. Heinonen, Jalila Chagraoui, Seppo Vainio, Guy Sauvageau, Claude Perreault
Journal: Immunity (2008)
Incidence and Predictors of Low Dose-Intensity in Adjuvant Breast Cancer Chemotherapy: A Nationwide Study of Community Practices
Authors: Gary H. Lyman, David C. Dale, Jeffrey Crawford
Journal: Journal of Clinical Oncology (2003)

This retrospective study was undertaken to assess practice patterns in adjuvant chemotherapy for early-stage breast cancer (ESBC) and to define the incidence and predictive factors of reduced relative dose-intensity (RDI).A nationwide survey of 1,243 community oncology practices was conducted, with data extracted from records of 20,799 ESBC patients treated with adjuvant chemotherapy. Assessments included demographic and clinical characteristics, chemotherapy dose modifications, incidence of febrile neutropenia, and patterns of use of colony-stimulating factor (CSF). Dose-intensity was compared with published reference standard regimens.Dose reductions > or =15% occurred in 36.5% of patients, and there were treatment delays > or =7 days in 24.9% of patients, resulting in 55.5% of patients receiving RDI less than 85%. Nearly two thirds of patients received RDI less than 85% when adjusted for differences in regimen dose-intensity. Multivariate analysis identified several independent predictors for reduced RDI, including increased age; chemotherapy with cyclophosphamide, methotrexate, and fluorouracil, or cyclophosphamide, doxorubicin, and fluorouracil; a 28-day schedule; body-surface area greater than 2 m2; and no primary CSF prophylaxis. CSF was often initiated late in the chemotherapy cycle.Patients with ESBC are at substantial risk for reduced RDI when treated with adjuvant chemotherapy. Patients at greatest risk include older patients, overweight patients, and those receiving three-drug combinations or 28-day schedules. Predictive models based on such risk factors should enable the selective application of supportive measures in an effort to deliver full dose-intensity chemotherapy.
Making the best of a little
Authors: S. Straube, R. A. Moore, S. Derry, P. J. Wiffen
Journal: BMJ (2010)

Nuesch and colleagues confirm that the effects of small studies can distort the results of meta-analyses.1 More important than this potential distortion, however, is the potential for getting completely the wrong answer from a meta-analysis that contains only small trials.

The problem, of course, is that many, if …
PL-01 * A PRECLINICAL JUVENILE RAT MODEL OF BRAIN INJURY AFTER FRACTIONATED WHOLE BRAIN IRRADIATION
Authors: R. J. Brown, B. J. Jun, A. Beighley, J. Blanchard, K. Iwamoto, N. Harris, S. Bluml, W. H. McBride
Journal: Neuro-Oncology (2014)

BACKGROUND: Late effects of radiotherapy in survivors of pediatric brain tumors are life-long and debilitating. We describe the behavior and imaging characteristics of a rat model given fractionated whole brain radiation equivalent to doses used to treat children with high-risk medulloblastoma. METHODS: One month-old Fischer rats were given 9 fractions of either 0 or 3 Gray (Gy). A third group was given eight 3Gy fractions followed by a single 10Gy “top off” to serve as a positive control. The 27Gy dose is biologically equivalent to 36Gy given to children. The open-field exploration task and the elevated plus maze were performed at 4 months post-irradiation. Declarative memory was tested by novel object recognition at 4- and 7-months post-irradiation. Magnetic resonance imaging, including diffusion tensor imaging (DTI) and spectroscopy (MRS), was obtained at baseline, 4-, and 7-months post-irradiation. RESULTS: 4 months post-irradiation, there were no differences in the open-field exploration task among the groups. Both the 27Gy (p = 0.002) and 34Gy (p = 0.04) groups demonstrated less exploration of the open arms of the elevated plus maze. There were no differences in novel object recognition at 4 months, but both irradiated groups demonstrated deficits by 7 months post-irradiation (p27 = 0.02, p34 = 0.02). Fractional anisotropy of the corpus callosum at 7 months post-irradiation was decreased only in the 34Gy group (p = 0.02) and not in the 27Gy group. N-acetyl aspartate remained higher than controls at 7-months post-irradiation in both hippocampus (p27 < 0.01, p34 < 0.03) and corpus callosum (p27 < 0.08, p34 < 0.04). CONCLUSIONS: This model of a juvenile rat given fractionated, clinically-relevant doses of radiation displays cognitive deficits described in survivors of medulloblastoma who have received radiotherapy. Imaging characteristics indicate a potential disruption of the normal developmental myelination by MRS that correlates with memory deficits and may be useful for evaluating novel therapeutic strategies of radiation mitigation.
P3-14-10: Early Predictive Value of Non-Responder to Docetaxel in Neoadjuvant Chemotherapy in Breast Cancer Using 18F- FDG-PET.
Authors: T Hirakata, T Fujisawa, Y Yanagita, H Horikoshi, N Oya, T Akiyoshi, T Kinoshita, H Kuwano
Journal: Cancer Research (2011)

Abstract Background; Clinical response is determined after several cycles of chemotherapy by changes in tumor size in imaging procedures including ultrasound, MRI or CT. The aim of this prospective study was to early detect non-responder to docetaxel (DTX) in neoadjuvant chemotherapy using 18F- FDG-PET in patients with breast cancer. Method; 41 patients were eligible for this study. 37 were assessable (4 were not eligible or not evaluable) with Invasive carcinoma (T1:10%, T2: 83%). All 37 patients were treated with 4 cycles of docetaxel (75mg per square meter) followed by 4 cycles of FEC(cyclophosphamide/epirubicin/fluorouracil: 500/75/500 mg per square meter) before surgery. 18F-FDG-PET response rate was evaluated between before and after the first cycle of DTX. 18F-FDG-PET images were analyzed by standardized uptake value, SUVmax. Clinical response is determined by reduction rate in tumor size with MRI (RECIST criteria) between before the first cycle and after the fourth cycle of DTX. Pathological response evaluated by core needle biopsy after the fourth cycle of DTX. Result; 18F-FDG-PET response rate after the first cycle of DTX showed correlation with tumor size reduction rate with MRI image after the fourth cycle of DTX (r=0.746)(P &amp;lt;.001). 0 out of 8 patients (0%) revealed cPR with MRI changes in SUV decrease less than 18% (p &amp;lt;.001). 8 out of 13 patients (62%) showed cPR (cCR = 0) with MRI in SUV decrease over 19 to 44% (P &amp;lt;.001). 9 out of 16 patients (56%) were cPR, and 7 out of them (44%) showed cCR with MRI in SUV decrease over 45% (P &amp;lt;.001). Conclusion; Changes of 18F-FDG SUVs in tumors were statistically significantly different between responding and nonresponding (P &amp;lt;.001). SUV decrease less than 18% after the first cycle indicated potential failure to DTX in neoadjuvant chemotherapy. Citation Information: Cancer Res 2011;71(24 Suppl):Abstract nr P3-14-10.
Diagnosis of Constrictive Pericarditis by Tagged Cine Magnetic Resonance Imaging
Authors: Sunao Kojima, Naoaki Yamada, Yoichi Goto
Journal: New England Journal of Medicine (1999)

To the Editor: Constrictive pericarditis is characterized by a thickened and adherent pericardium that restricts diastolic filling of the heart.1 Although many diagnostic criteria for constrictive ...