tbuckley/cabot-search huggingface dataset:
Extreme Insulin Resistance in Ataxia Telangiectasia
Authors: Robert S. Bar, William R. Levis, Matthew M. Rechler, Len C. Harrison, Carolyn Siebert, Judith Podskalny, Jesse Roth, Michele Muggeo
Journal: New England Journal of Medicine (1978)

The syndrome of ataxia telangiectasia is associated with glucose intolerance and insulin resistance. We examined the status of insulin receptors on circulating monocytes and on cultured fibroblasts from two siblings with ataxia telangiectasia and severe insulin resistance. 125I-insulin binding to monocytes of the two patients consistently demonstrated an 80 to 85 per cent decrease in receptor affinity. In contrast, the defect in receptor affinity was not expressed on the patients' cultured fibroblasts or on monocytes or fibroblasts obtained from unaffected family members. Whole plasma and immunoglobulin-enriched fractions of plasma from the patients inhibited the normal binding of insulin to its receptors on cultured human lymphocytes (IM -9 line) and on human placental membranes. We conclude that the insulin resistance in the two siblings with ataxia telangiectasia was associated with defects in the affinity of the receptors for insulin, probably caused by circulating inhibitors of insulin binding.
Child Life Investigations: A Clinical and Pathological Study of 1673 Cases of Dead-Births and Neonatal Deaths.
Journal: JAMA (1927)

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
Regional Enteritis and Glucose-6-Phosphate Dehydrogenase Deficiency
Authors: Dimitrios Katsaros, Sidney C. Truelove
Journal: New England Journal of Medicine (1969)

In a previous study, about 10 per cent of patients with regional enteritis were found to be deficient in glucose-6-phosphate dehydrogenase (G-6-PD). In the present study, enzymatic assay was performed in 69 patients with regional enteritis and 49 patients with ulcerative colitis. No examples of G-6-PD deficiency were found, so that it is concluded that G-6-PD deficiency is unlikely to be an etiologic factor in either of these diseases.
PTH-270 β-catenin expression in the primary tumour is associated with lymph node status in colorectal cancer
Authors: J Evans, N Al-Khafaj, PA Sutton, D Bowden, PS Rooney, D Vimalchandran, JR Jenkins
Journal: Gut (2015)

Introduction Accurate assessment of lymph node status is essential in determining optimal patient management in colorectal cancer (CRC). Local excision is feasible for early rectal and polyp cancers but without lymph node harvesting, postoperative histological stage may be inaccurate and patients may not receive the survival benefit conferred from appropriate adjuvant therapy.1,2 Biomarkers in the primary tumour may predict patients at risk of nodal metastases who would benefit from standard surgical resection. We aimed to assess the relationship between β-Catenin expression within the primary tumour and lymph node status. Method Patients with colorectal adenocarcinoma and complete lymph node staging were included. A tissue microarray (TMA) of multiple cores from formalin fixed paraffin embedded blocks was created. TMA sections were stained in duplicate for β-Catenin, and scored (0–3) for nuclear staining intensity relative to cytoplasm, an adaptation of the breast cancer H-Score method.3These scores were correlated with lymph node status as reported in the resection specimen. Statistical analysis was performed using SPSS Statistics. Results Complete data were available on 19 patients comprising; two polyp cancers, six Dukes’ A, four Dukes’ B and seven Dukes’ C tumours. There were eight right-sided, seven left-sided and four rectal tumours. Higher levels of nuclear β-Catenin staining were noted in the 11 patients with node negative disease (mean H-score = 1.8; SD = 0.4) than in node positive disease (mean H-score = 1.3; SD = 0.2; p = 0.03, Mann-Whitney). Interestingly lower nuclear expression of β-Catenin was seen in the patient with a polyp cancer and node positive disease than in the polyp cancer without nodal disease (H-score 1.4 vs. 2.4). Conclusion Reduced nuclear expression of β-Catenin in the primary tumour is associated with node positive disease. Further evaluation of these findings in a larger data set is required to assess if this phenotype should be considered for formal surgical resection to ensure accurate staging and prevent under-treatment. Disclosure of interest J. Evans Grant/ Research Support from: Cancer Research UK, N. Al-Khafaj: None Declared, P. Sutton: None Declared, D. Bowden: None Declared, P. Rooney: None Declared, D. Vimalchandran: None Declared, J. Jenkins: None Declared. References 1. Sakamoto T, Mori G, Yamada M, Kinjo Y, So E, Abe S, et al . Endoscopic submucosal dissection for colorectal neoplasms: a review. World journal of gastroenterology: WJG. 2014;20(43):16153–8 2. Wu XR, Liang J, Church JM. Management of sessile malignant polyps: is colonoscopic polypectomy enough? Surgical endoscopy. 2014 3. Shousha S. Oestrogen receptor status of breast carcinoma: Allred/H score conversion table. Histopathology. 2008;53(3):346–7
PTU-029 Finding the dysplastic lesion: outcome of endoscopic mucosal resection after detecting dysplasia on biopsies in barrett’s oesophagus
Authors: A Koutsoumpas, LM Wang, A Bailey, R Gillies, R Marshall, B Sgromo, N Maynard, B Braden
Journal: Gut (2015)

<h3>Introduction</h3> High grade dysplasia (HGD) is often reported after Seattle protocol biopsies have been taken or the exact position of a targeted lesion within a Barrett’s segment has not been precisely documented. We investigated how often the histology of subsequent endoscopic resection agreed with the initial biopsy of HGD. <h3>Method</h3> We searched our prospectively maintained database for patients referred for endoscopic therapy of high grade dysplasia or early cancer in Barrett’s oesophagus. All biopsies from referring hospitals had been reviewed and confirmed by our expert pathologists. Endoscopy was routinely performed using acetic acid spraying and narrow band imaging to identify and delineate lesions for endoscopic resection. Endoscopic resection was performed using band ligation mucosectomy. <h3>Results</h3> 100 patients with HGD (71) or early cancer (29) were included. In 34 (47.9%) of the patients with HGD on biopsy, no lesion or abnormality had been reported on the initial endoscopy report. In all of the cancer patients and in 70 (98.6%) of the HGD patients a suspicious lesion could be identified using advanced imaging. One patient with focal HGD but without detectable mucosal lesion was directly treated using RFA. EMR confirmed HGD in 35 of 70 (50%) patients with HGD on initial biopsy but upgraded to intramucosal in 30 patients (42.8%) and to submucosal cancer in 5 patients (7.1%). <h3>Conclusion</h3> High grade dysplasia in Barrett’s oesophagus is almost always detectable using acetic acid spraying and/or narrow band imaging. Endoscopic resection of all detected lesions before RFA is recommended due to the high risk of intramucosal and even submucosal cancer. <h3>Disclosure of interest</h3> None Declared.
A Co-operative Study for the Detection of the Carrier State of Classic Hemophilia
Authors: Harvey G. Klein, Louis M. Aledort, Bonno N. Bouma, Leon W. Hoyer, Theodore S. Zimmerman, David L. DeMets
Journal: New England Journal of Medicine (1977)

To determine the specificity and sensitivity of current technics for detecting carriers of classic hemophilia, three investigators simultaneously tested obligate carriers and noncarriers for immunologic and procoagulant factor VIII activity. Overall correct classification ranged from 72 per cent (36 of 50) to 94 per cent (47 of 50). The maximum accuracy obtained with the same linear-discriminant-function method on all data was 90 per cent (26 of 29) in detecting carriers without misclassifying normal persons as carriers (none of 21). Lowest accuracy by the same technic was 66 per cent (19 of 29) carrier detection while misclassifying 19 per cent (four of 21) normal persons. Precision of testing for both factor VII activity and antigen was high (standard deviations from 0.004 to 0.026 on a log scale). Differences between participants seemed related to laboratory technics rather than to statistical methods. The factor VIII activity/antigen measurement is a valid technic for detecting in the carrier state of hemophilia A.
Physicochemical Basis of the Buffered Acetylsalicylic Acid Controversy
Authors: Gerhard Levy, Barbara A. Hayes
Journal: New England Journal of Medicine (1960)

NUMEROUS conflicting reports about the relative efficacy of regular acetylsalicylic acid and the form in combination with buffering agents have appeared in the literature. The purpose of this paper is to present data that may explain the apparent contradictions in the results obtained by different investigators. In addition, the physicochemical principles outlined here have been utilized to interpret and correlate the relevant data found in the clinical literature.The controversy over buffered and unbuffered drug is centered upon two principal factors — namely, the incidence and severity of gastric irritation and the rate and extent of absorption of acetylsalicylic acid . . .
M-mode and Cross-sectional Echocardiographic Evaluation of Rheumatic Mitral Valve Disease : SYMPOSIUM ON CURRENT TOPICS AND FUTURE PROSPECTS IN ECHOCARDIOGRAPHY
Authors: Tohru Umeda, Kenji Kuwako, Kiyoshi Machii
Journal: Circulation (1979)

M-mode and cross-sectional echocardiograms were obtained in 30 patients with rheumatic mitral valve disease (24 : MS, 6 : MR) pre and post-operatively for the evaluation of their severity. Echocardiographic findings were compared with operative findings. Mitral valve area (MVA), E-F slope (DDR) and mean circumferential fiber lengthening during rapid filling course (mVcF(RF)) were measured in patients with MS (1.2 cm2, 14mm/sec 1.3 circ/sec respectively) and they were significantly improved after operation (2.2 cm2, 35mm/sec, 19 circ/sec respectively). Assessment of fusion of both commissures could be detected in 8 of 13 cases (62%) who underwent papillotomy in addition to commissurotomy. In patients with MR, short axis images of cross-sectional echocardiogram showed mitral incompetence during systole with marked enlargement of left atrium and mitral annulus (63 mm, 46 mm respectively). It was suggested that M-mode echocardiogram was more useful to estimate the axial dimensions and the cardiac cycle and was sensitive to detect rheumatic mitral valve disease, but it was of limited valve for the assessment of its severity. On the other hand, cross-sectional echocardiogram was proved to be valuable for the assessment of the whole mitral complex, which was useful not only for the evaluation of the severity of mitral valve disease but for the assignment of operative category.
S112 Variability in susceptibility to antibiotics and bacteriophages between individual colonies of Pseudomonas aeruginosa from cystic fibrosis sputum samples: implications for future clinical trial design
Authors: V Khoo, R Pabary, H Lund Palau, A Turnbull, N Madden, S Schelenz, A Jones, S Morales, EWFW Alton, JC Davies
Journal: Thorax (2015)

<h3></h3> Antimicrobial resistance is a growing problem. As part of the Cystic Fibrosis (CF) Trust-funded Strategic Research Centre for research into <i>Pseudomonas aeruginosa</i> (Pa) (http://www.cysticfibrosis.org.uk/research-care/research/about-cystic-fibrosis-research/how-we-invest-in-research/strategic-research-centres/src-1), we are exploring the therapeutic potential of bacteriophages. To date, we have a) identified a mix of 4 bacteriophages to which &gt;80% of our CF isolates appear sensitive; b) confirmed efficacy in an acute murine model and c) confirmed compatibility with clinically relevant nebulisers. In the clinical trial, patients will be selected on the basis of confirmed sensitivity of their Pa strain (s) to the bacteriophage mix. Significant variability has previously been reported when multiple, morphologically indistinguishable colonies were tested against antibiotics; here, we explored whether a similar phenomenon occurs with bacteriophages. CF sputum (n = 6 patients) was cultured on Pa-specific agar. Up to 10 individual morphologically identical colonies were inoculated separately into broth. After overnight culture each was subjected to disc diffusion (12 antibiotics) and phage testing (standard plaque assays). Reproducibility (repeat testing of same broth) was excellent for susceptibility/resistance to antibiotics (97.2% within 3 mm) and bacteriophages (93.3% within one log dilution). Variability in antibiotic susceptibility was lower than anticipated: in no samples were there both completely resistant and fully sensitive colonies and although zone sizes did vary, these crossed break points in only 12.5% of assessable samples. There was more variability in phage sensitivity. Colonies from two subjects displayed &lt;1 log dilution differences between them and were therefore reasonably consistent. The other 4 subjects displayed &gt;1 log differences between the most and least sensitive; in one, colonies clearly fell into two groups (highly sensitive/less so with a 4 log dilution difference) and on subculture, differences in pigment production were apparent. Susceptibility may not be predicted by testing a mix of colonies. As described previously for antibiotics, individual, apparently morphologically similar colonies of Pa in a CF sputum sample display inconsistent susceptibility profiles to anti-pseudomonal bacteriophages. The potential role of genetic variability to explain this is being explored and numbers increased. The results may help determine the best methodology for assessing isolate susceptibility to a phage mix for inclusion in a future clinical trial.
CT evaluation of small pulmonary vessels area in patients with COPD with severe pulmonary hypertension
Authors: Florence Coste, Gaël Dournes, Claire Dromer, Elodie Blanchard, Véronique Freund-Michel, Pierre-Olivier Girodet, Michel Montaudon, Fabien Baldacci, François Picard, Roger Marthan, Patrick Berger, François Laurent
Journal: Thorax (2016)

<h3>Rationale</h3> Severe pulmonary hypertension (PH) is very uncommon in COPD, and a distinct phenotype has been hypothesised. We aimed to evaluate whether CT can help to recognise this condition non-invasively by measuring small pulmonary vessels. <h3>Material and methods</h3> Patients with COPD who underwent pulmonary function tests, unenhanced CT of the chest and right heart catheterisation (RHC) during a period of stability were included in the study. From 105 included patients, 20 patients with COPD with severe PH (mean pulmonary arterial pressure, mPAP&gt;35 mm Hg) were compared with 20 FEV<sub>1</sub>-matched and age-matched patients with COPD with mild or without PH (mPAP&lt;35 mm Hg). The percentage of total cross-sectional area of vessels less than 5 mm<sup>2</sup> normalised by lung area (%CSA<sub>&lt;5</sub>) and 5–10 mm<sup>2</sup> (%CSA<sub>5–10</sub>), the mean number of cross-sectioned vessels (CSNs) and bronchial wall thickness (WT) were measured on CT examination and compared between groups. Paw scores combining PaO<sub>2</sub> measurement and CT parameters best correlated with mPAP were compared by receiver operating characteristic analysis to predict severe PH in COPD. <h3>Results</h3> Patients with severe PH COPD had higher %CSA and CSN values than those of patients with COPD without severe PH. Using multiple regression analysis, %CSA<sub>&lt;5</sub> and WT were the best predictors of mPAP in patients with and without severe PH, respectively. A score combining %CSA<sub>&lt;5</sub>, PaO<sub>2</sub> and WT best predicted severe PH in patients with COPD. <h3>Conclusions</h3> CT measurements of small vessels support a distinct vessel-related phenotype in patients with COPD with severe PH, and combined with WT and PaO<sub>2</sub> parameters in the paw score, which may offer a non-invasive tool to select patients for RHC.
Identification of High-Risk and Low-Risk Subgroups of Patients with Mitral-Valve Prolapse
Authors: Andrew R. Marks, Christopher Y. Choong, Anthony J. Sanfilippo, Merry Ferré, Arthur E. Weyman
Journal: New England Journal of Medicine (1989)

Mitral-valve prolapse is a common cardiac valvular disorder with a wide range of severity and diverse clinical outcomes. The lack of a standard definition of mitral-valve prolapse may explain the variation in reported complication rates. To identify high-risk and low-risk subgroups, we retrospectively analyzed clinical and two-dimensional echocardiographic data from 456 patients with mitral-valve prolapse. Mitral-valve prolapse was defined on the basis of echocardiographic findings as systolic displacement into the left atrium of one or both leaflets beyond the plane of the mitral annulus in the parasternal long-axis view. Two groups of patients were compared: those with thickening of the mitral-valve leaflets and redundancy (designated the classic form; n = 319) and those without leaflet thickening (designated the nonclassic form; n = 137). The two groups were similar in age and sex ratio. Complications or a history of complications was more prevalent in the classic than the nonclassic form: infective endocarditis, 3.5 percent and 0 percent, respectively (P less than 0.02); moderate-to-severe mitral regurgitation, 12 percent and 0 percent (P less than 0.001); and the need for mitral-valve replacement, 6.6 percent and 0.7 percent (P less than 0.02). However, the frequency of stroke was similar in the two groups: 7.5 percent and 5.8 percent (P not significant). We conclude that in a selected population of patients with mitral-valve prolapse, those with the classic form (leaflet thickening and redundancy) are at higher risk than those without these features for the infectious and hemodynamic complications of mitral-valve prolapse, but not for stroke.
Abstract IA25: Prevention in survivors: Reducing the “cost” of surviving childhood cancer
Authors: Lisa R. Diller
Journal: Cancer Research (2016)

Abstract Childhood cancer survivors are at significantly increased risk of treatment-related chronic disease and subsequent malignancy. Preventive intervention and screening can hopefully reduce the morbidity or mortality of these late-occurring illnesses. Screening for secondary cancer based upon prior exposures has been implemented for detection of early radiogenic breast cancers, skin cancers and GI malignancies. In adults without a history of childhood cancer, prevention strategies for diseases such as breast cancer and coronary artery disease have been investigated, but there is little evidence for the use of population-based strategies in childhood cancer survivors. We will review the role of primary and secondary prevention in childhood cancer patients and survivors. How have reduction of therapy studies reduced risk of late sequelae? What prevention interventions have been studied in survivors and what is the ongoing research in survivors? Data from studies of breast cancer screening with MRI and prevention with tamoxifen will be presented. Can we draw experience in prevention in other high-risk groups to apply to childhood cancer survivors? We will present results of our unique survivorship Markov model, which can help estimate the impact of screening strategies on outcome. Finally, prevention strategies carry potential risk – how do we help patients make decisions and help them understand risk? Citation Format: Lisa R. Diller. Prevention in survivors: Reducing the “cost” of surviving childhood cancer. [abstract]. In: Proceedings of the AACR Special Conference on Advances in Pediatric Cancer Research: From Mechanisms and Models to Treatment and Survivorship; 2015 Nov 9-12; Fort Lauderdale, FL. Philadelphia (PA): AACR; Cancer Res 2016;76(5 Suppl):Abstract nr IA25.
The Treatment of Subacute Bacterial Endocarditis with Aureomycin and Chloromycetin
Authors: Lewis W. Kane, John J. Finn
Journal: New England Journal of Medicine (1951)

PRIOR to the introduction of penicillin subacute bacterial endocarditis was nearly always fatal. In the past five years the many reports that have appeared on penicillin therapy of this infection indicate that bacteriologic cure may be expected in the majority of cases. A review of 817 cases treated with penicillin during the past five years has revealed a rate of cure of 72 per cent.1 Those cases of subacute bacterial endocarditis in which the etiologic bacterium was Streptococcus viridans have yielded most readily to penicillin.2 , 3 However, Streptococcus faecalis (enterococcus), being more resistant to penicillin, has been responsible for infections that . . .
Survival Improvement in Myelodysplastic Syndrome with Refractory Anemia after Treatment with Selective-Serotonin-Reuptake-Inhibitors
Authors: Gabriela Sanchez, Ang Li, Sarvari Venkata Yellapragada, Martha P. Mims, Anna Frolov, Effie Rahman, Gustavo A. Rivero
Journal: Blood (2014)
Report of the National Heart, Lung, and Blood Institute Working Group
Authors: Sara K. Pasquali, Jeffrey P. Jacobs, Gregory K. Farber, David Bertoch, Elizabeth D. Blume, Kristin M. Burns, Robert Campbell, Anthony C. Chang, Wendy K. Chung, Tiffany Riehle-Colarusso, Lesley H. Curtis, Christopher B. Forrest, William J. Gaynor, Michael G. Gaies, Alan S. Go, Paul Henchey, Gerard R. Martin, Gail Pearson, Victoria L. Pemberton, Steven M. Schwartz, Robert Vincent, Jonathan R. Kaltman
Journal: Circulation (2016)

The National Heart, Lung, and Blood Institute convened a working group in January 2015 to explore issues related to an integrated data network for congenital heart disease research. The overall goal was to develop a common vision for how the rapidly increasing volumes of data captured across numerous sources can be managed, integrated, and analyzed to improve care and outcomes. This report summarizes the current landscape of congenital heart disease data, data integration methodologies used across other fields, key considerations for data integration models in congenital heart disease, and the short- and long-term vision and recommendations made by the working group.