tbuckley/cabot-search huggingface dataset:
The Spectrum of Renal Disease in Laurence–Moon–Biedl Syndrome
Authors: John D. Harnett, Jane S. Green, Benvon C. Cramer, Gordon Johnson, Linda Chafe, Patrick McManamon, Nadir R. Farid, William Pryse-Phillips, Patrick S. Parfrey
Journal: New England Journal of Medicine (1988)

To determine the nature, extent, and severity of renal involvement in Laurence-Moon-Biedl syndrome (obesity, mental retardation, polydactyly, hypogonadism, and pigmented retinal dystrophy), we evaluated 20 of 30 patients with the disorder identified from ophthalmologic records in Newfoundland. The mean age was 31 years, and seven were male. All 20 patients had structural or functional abnormalities of the kidneys or both. Three had end-stage renal disease, with two requiring maintenance hemodialysis. The remaining 17 patients had normal serum creatinine values and estimated creatinine clearances. Half the subjects had hypertension. Fourteen of 17 patients could not concentrate urine above 750 mOsm per kilogram of body weight even after vasopressin, whereas all 10 normal controls could. Urinary pH decreased below 5.3 after ammonium chloride administration in all 15 normal controls, but in only 13 of 18 patients. Calyceal clubbing or blunting was evident in 18 of 19 patients studied by intravenous pyelography; 13 patients had calyceal cysts or diverticula. Seventeen of 19 patients had lobulated renal outlines of the fetal type. Four patients had diffuse renal cortical loss, but only two of these had renal insufficiency. We conclude that Laurence-Moon-Biedl syndrome includes the presence of renal abnormalities.
The Shortfalls of “Obamacare”
Authors: Gail R. Wilensky
Journal: New England Journal of Medicine (2012)

Interview with Drs. Gail Wilensky and John McDonough on the health care platforms of the two presidential candidates. (27:07)Download Since the Affordable Care Act ignores the structural problems in the organization and reimbursement of care, even if it succeeds in providing insurance coverage to more Americans, it won't solve the problems of unaffordable health care costs and inadequate quality of care.
Abstract P4-14-13: Therapeutic mammaplasty does not cause a delay in the delivery of chemotherapy in high risk breast cancer patients
Authors: J Khan, S Barrett, S Stallard, C Forte, E Weiler-Mithoff, I Reid, A Winter, J Doughty, L Romics
Journal: Cancer Research (2012)

Abstract Introduction: oncosurgical safety of therapeutic mammaplasty (TM) is widely investigated. The interval between surgery and delivery of adjuvant chemotherapy is an integral part of overall oncological safety. Therefore, we examined the time between TM and AC, and compared it to wide local excision (WLE) and mastectomy (Mx) with or without immediate breast reconstruction (IBR), respectively. Methods: data of 174 patients who underwent TM, WLE and Mx±IBR was analyzed retrospectively. All patients were operated within three breast units of Glasgow during a period of 48 months. Time between decision to offer adjuvant chemotherpay and delivery of the first cycle of chemotherapy was analyzed. Significance was calculated with Mann-Whitney and Kruskal-Wallis tests (two and four groups compared, respectively). Results: median time to adjuvant chemotherapy after TM (n = 36) was 29 [16–58] days, WLE (n = 66) was 29.5 [15–105], Mx only (n = 56) was 29 [15–57], and Mx and IBR (n = 16) was 31 [15–58] days. No significant difference was found in terms of time to adjuvant chemotherpay in patients treated with TM compared to WLE (p = 0.384), Mx only (p = 0.828) or Mx and IBR (p = 0.366). Further, there was no significant difference when a cumulative comparison of the four groups was carried out (p = 0.507). Conclusions: our data indicate that oncosurgical safety of TM in terms of time to chemotherapy is similar to other high risk breast cancer patients treated WLE and Mx with or without IBR. This also suggests that there is no significant difference in postoperative complication rates after these four ways of surgical treatment of breast cancer, which would possibly be the primary cause for a delay in delivering adjuvant chemotherapy. Citation Information: Cancer Res 2012;72(24 Suppl):Abstract nr P4-14-13.
Quantitative Graphical Analysis of Simultaneous Dynamic PET/MRI For Assessment of Prostate Cancer
Authors: Andrew B. Rosenkrantz, Thomas Koesters, Anne-Kristin Vahle, Kent Friedman, Rachel M. Bartlett, Samir S. Taneja, Yu-Shin Ding, Jean Logan
Journal: Clinical Nuclear Medicine (2015)

Dynamic FDG imaging for prostate cancer characterization is limited by generally small size and low uptake in prostate tumors. Our aim in this pilot study was to explore feasibility of simultaneous PET/MRI to guide localization of prostate lesions for dynamic FDG analysis using a graphical approach.Three patients with biopsy-proven prostate cancer underwent simultaneous FDG PET/MRI, incorporating dynamic prostate imaging. Histology and multiparametric MRI findings were used to localize tumors, which in turn guided identification of tumors on FDG images. Regions of interest were manually placed on tumor and benign prostate tissue. Blood activity was extracted from a region of interest placed on the femoral artery on PET images. FDG data were analyzed by graphical analysis using the influx constant Ki (Patlak analysis) when FDG binding seemed irreversible and distribution volume VT (reversible graphical analysis) when FDG binding seemed reversible given the presence of washout.Given inherent coregistration, simultaneous acquisition facilitated use of MRI data to localize small lesions on PET and subsequent graphical analysis in all cases. In 2 cases with irreversible binding, tumor had higher Ki than benign using Patlak analysis (0.023 vs 0.006 and 0.019 vs 0.008 mL/cm3 per minute). In 1 case appearing reversible, tumor had higher VT than benign using reversible graphical analysis (0.68 vs 0.52 mL/cm3).Simultaneous PET/MRI allows localization of small prostate tumors for dynamic PET analysis. By taking advantage of inclusion of the femoral arteries in the FOV, we applied advanced PET data analysis methods beyond conventional static measures and without blood sampling.
Electromagnetic Fields and Cancerl--Media and Public Attention Affect Research
Authors: M. E. Newman
Journal: JNCI Journal of the National Cancer Institute (1991)

Journal Article Electromagnetic Fields and Cancerl—Media and Public Attention Affect Research Get access Michael E. Newman Michael E. Newman Search for other works by this author on: Oxford Academic PubMed Google Scholar JNCI: Journal of the National Cancer Institute, Volume 83, Issue 3, 6 February 1991, Pages 164–166, https://doi.org/10.1093/jnci/83.3.164 Published: 06 February 1991
Early Dynamic SPECT Acquisition for the Imaging of Hepatic Hemangiomas Utilizing Tc-99m Labeled Red Blood Cells
Authors: ALAN SIEGEL, RICHARD MAZUREK
Journal: Clinical Nuclear Medicine (1997)

Purpose To determine whether rapid, dynamic SPECT imaging with Tc-99m labeled red blood cells was technically feasible and of value in the detection of cavernous hemangiomas of the liver. Materials and Methods The acquisition protocol for Tc-99m labeled red blood cell imaging of hepatic hemangiomas was modified with six sequential 3-minute SPECT images replacing the planar flow phase. Delayed 20-minute SPECT of the liver was then performed 2 hours later. Results The authors present examples of pathologically proven positive and negative cases and retrospectively review their patient series. Examination of 19 patients with 41 lesions revealed no evidence of a false-positive examination results and only one false-negative result. Only one lesion seen to be positive with a 2-hour delayed study was not seen within 18 minutes. Lesions as small as 1.0 cm could be detected with a 3-minute SPECT. Conclusions Rapid, dynamic early SPECT hemangioma imaging is feasible, provides images with relatively good quality, and can replace the planar flow phase.
AB0613 Clinical and Laboratory Differences between SSC Patients with and without Diastolic Dysfunction
Authors: Z. Jurisic, D. Marasović Krstulović, D. Perkovic, K. Boric, M. Radic, D. Martinovic Kaliterna
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> Left ventricular diastolic dysfunction (LVDD) is the most common cardiac abnormality in systemic sclerosis (SSc). It is known that aging is a primary determinant of LVDD in SSc patients and many of the clinical factors associated with LVDD were no longer associated after simply adjusting for age. <h3>Objectives</h3> The aim of this study was to determine clinical and laboratory parameters of SSc patients with and without LVDD matched for age. <h3>Methods</h3> We studied 13 SSc patients with preserved left ventricular ejection fraction (LVEF) and LVDD, and 13 SSc patients without LVDD matched for age, sex, body mass index and smoking status. LVDD was defined and classified according to the American Society of Echocardiography recommendations. Serum IL-6 and NT-proBNP levels were measured and subjects were evaluated by conventional and pulsed-wave tissue Doppler echocardiography and by the six minute walk test (6MWT). <h3>Results</h3> There was no difference between SSc patients with and without LVDD according to heart rate, systolic and diastolic blood pressure, left ventricular mass index, prevalence of pulmonary artery hypertension, and antibody status. SSc patients with LVDD had longer duration of SSc than SSc patients without LVDD (16.0 <i>vs</i> 6.65 yrs; <i>P</i>=0.004), and had higher EUSTAR score (5.77 <i>vs</i> 3.12; <i>P</i>=0.001). The serum level of IL-6 (6.39 <i>vs</i> 2.94 pg/ml; <i>P</i>=0.021) and NT-proBNP (24.78 <i>vs</i> 10.12 pmol/L; P=0.003) were significantly increased in SSc patients with LVDD. The 6MWT distance was shorter in SSc patients with LVDD as compared to those without LVDD (326.46 <i>vs</i> 398.69 m; <i>P</i>=0.034). Also, desaturation during the 6MWT was more significant in SSc patients with LVDD (-3.38% <i>vs</i> -1.92%; <i>P</i>=0.022). <h3>Conclusions</h3> SSc patients with LVDD had longer disease duration and higher disease activity (EUSTAR) scores. SSc patients with LVDD showed significantly increased serum IL-6 and NT-proBNP levels in comparison with those without LVDD. LVDD is associated with decreased exercise capacity in SSc patients. <h3>References</h3> Hinchcliff M, Desai CS, Varga J, Shah SJ. Prevalence, prognosis, and factors associated with left ventricular diastolic dysfunction in systemic sclerosis. Clin Exp Rheumatol. 2012;30:30-7. Scala E, Pallotta S, Frezzolini A et al. Cytokine and chemokine levels in systemic sclerosis: relationship with cutaneous and internal organ involvement. Clin Exp Immunol 2004;138:540-6. Nagueh SF, Appleton CP, Gillebert TC et al. Recommendations for the evaluation of left ventricular diastolic function by echocardiography. J Am Soc Echocardiogr 2009;22:107-33. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.5652
Case 42-1992
Authors: Richard C. Cabot, Robert E. Scully, Eugene J. Mark, William F. McNeely, Betty U. McNeely, B.L. Fanburg, E.J. Mark
Journal: New England Journal of Medicine (1992)

Presentation of CaseA 59-year-old woman was admitted to the hospital because of progressive dyspnea.The patient was in excellent health until four or five years earlier, when she began to experience exertional dyspnea that gradually worsened. Seven months before admission she discontinued a 50—pack-year habit of cigarette smoking, with transitory improvement. Three or four months later the dyspnea began to worsen progressively and was accompanied by fatigue, and she entered another hospital. The results of cardiopulmonary laboratory studies are presented in Table 1. An x-ray film of the chest showed a diffuse, bilateral interstitial infiltrate. A ventilation—perfusion scan indicated a . . .
A Familial Disturbance of Histidine Metabolism
Authors: H. Ghadimi, M. W. Partington, A. Hunter
Journal: New England Journal of Medicine (1961)

THIS communication describes our preliminary findings on 2 children who have what appears to be a hitherto undescribed inborn error of metabolism.Case ReportsCase 1. M.M., a 3-year-old girl, was first seen in February, 1960, because of retardation of speech. The mother had had some vaginal bleeding in the 3d and an attack of rubella in the 7th month of pregnancy. The child was born normally at term and weighed 3.3 kg. (7 pounds, 2 ounces). She suffered several respiratory infections in infancy and, at the age of 1 year, had an attack of diarrhea lasting for 3 weeks. . . .
Kayser-Fleischer Ring in a Patient with Wilson's Disease
Authors: Kim Eagle, Albert W. Biglan, Sharon L. Taylor
Journal: New England Journal of Medicine (1993)

Figure 1. Kayser-Fleischer Ring in a Patient with Wilson's Disease. Slit-lamp photography shows a dense deposit of copper in Descemet's membrane (a Kayser-Fleischer ring) in a 17-year-old girl with Wilson's disease (Panel A). After eight years of therapy with dimercaprol and penicillamine, the Kayser-Fleischer ring had completely resolved (Panel B). The left eye is shown; the right eye had a similar appearance. (The other line is formed by the beam of the slit lamp).
EFFECT OF NATIVE DESCENDING THORACIC AORTA GROWTH AFTER ACUTE AORTIC DISSECTION TYPE A ON EVENT FREE SURVIVAL
Authors: Madelien Veronique Regeer, Vasileios Kamperidis, Michael Versteegh, Robert Klautz, Martin Schalij, Jeroen Bax, Nina Ajmone Marsan, Victoria Delgado
Journal: Journal of the American College of Cardiology (2015)
AB0216 Novel self-organized criticality theory of autoimmunity explaining the cause of SLE
Authors: S. Shiozawa, K. Tsumiyama, Y. Miyazaki, K. Uto
Journal: Annals of the Rheumatic Diseases (2013)

<h3>Background</h3> Since the discovery of LE cell by Haargraves, reactivity against self has been considered the cause of autoimmune diseases. However, recent immunology tells that autoreactive clones are not forbidden and autoantibodies are seen rather ubiquitously. <h3>Objectives</h3> Thus, apart from Mackey's autoimmune disease theory, we have studied the cause of SLE from a different angle, i.e., a view of systems biology, and shown that systemic autoimmunity necessarily takes place when host's immune system is over-stimulated by repeated exposure to antigen to levels that surpass system's self-organized criticality (1,2). Here we characterize the essential component, <i>ai</i>CD4 Tcell, that induces both varieties of autoantibodies and immunopathology, and also clarify the molecular mechanism of antigen cross-presentation responsible for inducing the tissue immunopathology identical to human SLE. <h3>Methods</h3> Mice not prone to autoimmune diseases, Balb/c or B57BL/6, were repeatedly immunized without adjuvant more than x8 with OVA, KLH or SEB. Activated CD4 T cells generated were sub-fractionated using cell sorter or were transferred into naïve mice. Antigen cross-presentation was studied in dendritic cell (DC) under confocal laser microscopy and <i>in vivo</i> in transgenic mice. <h3>Results</h3> After repeated immunization x12 with antigen, all mice developed varieties of autoantibodies including anti-dsDNA antibody (Ab) and typical immunopathology of SLE. Autoantibody inducing CD4 T cell (<i>ai</i>CD4 T cell) that has passed through <i>de novo</i> TCR revision i.e., V(D)J recombination, was newly generated in mice, where TCRalpha but not TCRbeta was revised. Autoantibodies and immunopathology were completely transferrable into naïve recipients <i>via</i> CD4 and CD8 T cells, respectively. For tissue immunopathology including lupus nephritis, (1) help by <i>ai</i>CD4 T cell and (2) antigen cross-presentation were required. The <i>ai</i>CD4 T cell belonged to CD45RB<sup>lo</sup> CD122<sup>lo</sup> subpopulation. In DC, antigen was transfered from endosome to cytoplasm and then cross-presented to CD8 T cells mediated by Sec61. <h3>Conclusions</h3> Systemic autoimmunity necessarily takes place when host's immune system is overstimulated by repeated exposure to antigen to levels that surpass system's self-organized criticality. Generation of <i>ai</i>CD4 T cell and antigen cross-presentation are essential for causing systemic autoimmunity, i.e., SLE. <h3>References</h3> Tsumiyama K, Miyazaki Y, Shiozawa S. Self-organized criticality theory of autoimmunity. PLoS ONE 4(12):e8382, 2009. Hiozawa S. Cause of systemic lupus erythematosus: a novel self-organized criticality theory of autoimmunity. Exp Rev Clin Immunol 7:715-717, 2011. <h3>Disclosure of Interest</h3> None Declared
Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in an Adult
Authors: Jack M. George, Donald M. Knowlan
Journal: New England Journal of Medicine (1959)

ANOMALOUS origin of the left coronary artery from the pulmonary artery has been reported in 13 adult patients.1 2 3 4 5 6 7 8 9 10 11 12 The condition, well described in infants,13 14 15 is the most frequently discussed anomaly of origin of the coronary vessels in the literature. Anomalous origin of the right coronary artery is far less frequent, and the origin of both coronary arteries from the pulmonary artery has been reported in a few cases. The left coronary artery has also been reported to originate from the right coronary artery and from the right aortic sinus. Minor anomalies of left coronary branches have also been reported. The . . .
OP0230 Systemic Vasculitis and Pregnancy: A Multicenter Study on Maternal and Neonatal Outcome of 66 Prospectively Followed Pregnancies
Authors: M.G. Lazzaroni, M. Fredi, L. Andreoli, A. Brucato, R. Caporali, P. Caramaschi, A. Doria, M. Gerosa, L. Guillevin, F. Inverardi, A. Lojacono, P.L. Meroni, C. Montecucco, M. Mosca, M. Motta, V. Ramoni, P. Sfriso, R.A. Sinico, F. Strigini, C. Tani, A. Tincani
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> Systemic Vasculitis (SV) are rare diseases and unlike other rheumatic diseases they do not preferentially affect women during reproductive age. A few data are available in literature, mostly from case reports with retrospective design [1,2]. <h3>Objectives</h3> Aim of our study is to prospectively evaluate maternal/neonatal outcome and disease course before, during and after pregnancy in patients with SV. <h3>Methods</h3> 66 pregnancies in 44 women with diagnosis of SV (according to CHCC and or ACR criteria) were prospectively followed by a multispecialistic team composed by Rheumatologists, Gynecologists and Neonatologists in one of the 8 Institutions involved in a period comprised between years 1995 and 2013. Data were retrospectively collected from clinical charts using a common database. Data about a control population of 3939 healthy women in one center during one year (General Obstetric Population, GOP) were considered to compare the rate of pregnancy-related complications. <h3>Results</h3> Outcome of pregnancy was 55 live births (85,9%), 8 miscarriages before 10° week (12,5%) and 1 fetal death after 10° week (1,5%); 2 pregnancies are still ongoing. Data about pregnancy-related complications were compared to GOP: in SV patients preterm deliveries (20,0% vs 11,9%), severe (&lt;34°weeks) preterm deliveries (10,1% vs 5,0%) and cesarean section (47,3% vs 31,0%) were significantly more frequent than in GOP. Disease-related complications occurred in 24 pregnancies during the three trimesters (36,4%), with 8 severe events (12,1%) including 3 cases of transient ischemic attack. Data about the postpartum period were available for 51 pregnancies: 11 flares (21,6%) occurred, with 1 severe event (1,9%). <h3>Conclusions</h3> SV patients can have successful pregnancies, but with increased risk of preterm delivery. Pregnancy-related modifications (immunological, cardiovascular and thrombophilic) can negatively affect the course of disease, with severe complications. A tight control by a multispecialistic team should be a standard in pregnancies of women with SV. <h3>References</h3> Gatto M et al. Pregnancy and vasculitis: a systematic review of the literature. Autoimmunity reviews 2012;11(6):A447-A459 Pagnoux C, Mahendira D, and Laskin CA. Fertility and pregnancy in vasculitis. Best Practice &amp; Research Clinical Rheumatology 2013;27(1):79-94 <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.2312
SP0001 The Value of Health Professionnals in Europe: A Personal View and Vision for the Future
Authors: M. Dougados
Journal: Annals of the Rheumatic Diseases (2014)

<h3></h3> A lot of studies have demonstrated or suggested the interest of health professionnals led management/therapeutical programs in the field of rheumatic diseases. However, there is a huge room of improvement in this area with regard to the following items. <i>The list of health professionnals</i> useful in the management of rheumatic and musculoskeletal disorders. In different countries, it is clear that the physiotherapists and the nurses are working closely either alone or in collaboration with general practitioners and rheumatologists when educating, monitoring or treating patients suffering from Rheumatic and Musculoskeleta diseases (RMDs). This list is important to consider when elaborating multidisciplinary team program. For example, should such program include systematically a pharmacist, a psychotherapist? ... Even more importantly is to consider the patient her/himself as part of this team either as an individual with a self-assessment of her/his disease or as a patient partner in charge of educational programs for other patients Moreover, for each of the member of the team, the question is whether we could refer to a <i>qualified person</i> with a "general" (non-specific) qualification. For example, do the nurses need a specific training permitting them to be considered as "nurse specialist"to allow them to participate at a rheumatological initiative? Finally, which are <i>the frontiers</i> of such collaboration? For example, a part from rheumatologists and radiologists, what is the role of the other health professionnals in the ultrasonographic evaluation of rheumatism and musculoskeletal disorders. In conclusion, the value of the health professionals in the management of patients with RMDs is obvious but a standardization and an harmonization at least at the European level and why not under the umbrella of EULAR is required. Such standardization will be the guarantee of the quality of the proposed initiatives which will result in benefit for the patients. <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.6262