tbuckley/cabot-search huggingface dataset:
Abstract 18101: EFFICAS I Results - Are Low Contact Force Parameters Predictors for Gap Formation after Pulmonary Vein Isolation by Radiofrequency Catheter Ablation?
Authors: Petr Neuzil, Dan Wichterle, Robert Cihak, Jan Skoda, Erik Wissner, Olivier Fremont, aude Yulzari, vivek Y Reddy, Josef Kautzner, Karl-Heinz Kuck
Journal: Circulation (2011)

Objectives Electrical reconnection of pulmonary veins (PVs) results in recurrences of atrial fibrillation (AF) following PV isolation (PVI). Gaps in the isolation lines may occur due to suboptimal catheter tip-to-tissue contact force (CF) during ablation. EFFICAS I, a multi-center prospective study, evaluated the relationship between CF parameters and gap formation during follow-up. Methods Pts with paroxysmal AF underwent PVI using an irrigated RF ablation catheter that provides tip-to-tissue CF information (TactiCath®, Endosense, Switzerland). The operator was blinded to the CF, but CF and Force Time Integral (FTI) were recorded for later analysis. PV antra were divided into 8 segments of interest (SEG). Patients underwent a re-mapping procedure at 3 months to evaluate gap occurrence. Incidence of gaps per SEG at 3 months was correlated with CF and FTI during initial PVI. Results Forty pts (59 ± 10 yrs, 30 males) were treated by 10 operators at 3 centers with 2519 RF ablations. Average CF at the right PVs was higher compared to the left PVs (26.0 ± 16.6 g vs. 17.9 ± 13.0 g, p&lt;0.0001). Greater CF was applied to the posterior wall than the left anterior wall (21.9 ± 14.5 g vs. 13.3 ± 9.9 g, p&lt;0.0001). At re-mapping, 26 pts (65%), including 6 pts (15%) with AF recurrence, had 52 gaps out of 318 SEGs (gap ratio 16%). CF was lower in the SEGs with gaps compared to no-gap SEGs (19.5 ± 14.4 g vs. 22.4 ± 14.8 g, p=0.04), but the strongest gap predictor was the minimum FTI applied at each SEG with 213 ± 325 gs for gap vs 434 ± 599 gs for no-gap SEGs (p&lt;0.001). Likelihood for gap considerably increased with minimum FTI &lt; 400 gs (21% vs 5%, p&lt;0.001, Odd Ratio = 4.8) with a significant inverse relationship between minimum FTI and incidence of gaps (Figure). Conclusions Low minimum FTI, as an indicator of catheter stability, is a strong predictor of gap occurrence following previous PVI. This endpoint also correlates with low CF. Optimization of minimum FTI threshold will be determined in ongoing EFFICAS II study.
Sudden Death in Patients With Cardiac Implantable Electronic Devices
Authors: Zian H. Tseng, Robert M. Hayward, Nina M. Clark, Christopher G. Mulvanny, Benjamin J. Colburn, Philip C. Ursell, Jeffrey E. Olgin, Amy P. Hart, Ellen Moffatt
Journal: JAMA Internal Medicine (2015)

<h3>Importance</h3> Interrogations and autopsies of sudden deaths with cardiac implantable electronic devices (CIEDs) are rarely performed. Therefore, causes of sudden deaths with these devices and the incidence of device failure are unknown. <h3>Objective</h3> To determine causes of death in individuals with CIEDs in a prospective autopsy study of all sudden deaths over 35 months as part of the San Francisco, California, Postmortem Systematic Investigation of Sudden Cardiac Death (POST SCD) study. <h3>Design, Setting, and Participants</h3> Full autopsy, toxicology, histology, and device interrogation were performed on incident sudden cardiac deaths with pacemakers or implantable cardioverter defibrillators (ICDs). The setting was the Office of the Chief Medical Examiner, City and County of San Francisco. Participants included all sudden deaths captured through active surveillance of all deaths reported to the medical examiner and San Francisco residents with an ICD (January 1, 2011, to November 30, 2013). <h3>Main Outcomes and Measures</h3> Identification of a device concern in sudden deaths with CIEDs, including hardware failures, device algorithm issues, device programming issues, and improper device selection. For the ICD population, outcomes were the cumulative incidence of death and sudden cardiac death and the proportion of deaths with an ICD concern. <h3>Results</h3> Twenty-two of 517 sudden deaths (4.3%) had CIEDs, and autopsy revealed a noncardiac cause of death in 6. Six of 14 pacemaker sudden deaths and 7 of 8 ICD sudden deaths died of ventricular tachycardia or ventricular fibrillation. Device concerns were identified in half (4 pacemakers and 7 ICDs), including 3 hardware failures contributing directly to death (1 rapid battery depletion with a sudden drop in pacing output and 2 lead fractures), 5 ICDs with ventricular fibrillation undersensing, 1 ICD with ventricular tachycardia missed due to programming, 1 improper device selection, and a pacemaker-dependent patient with pneumonia and concern about lead fracture. Of 712 San Francisco residents with an ICD during the study period, 109 died (15.3% cumulative 35-month incidence of death), and the 7 ICD concerns represent 6.4% of all ICD deaths. <h3>Conclusions and Relevance</h3> Systematic interrogation and autopsy of sudden deaths in one city identified concerns about CIED function that might otherwise not have been observed. Current passive surveillance efforts may underestimate device malfunction. These methods can provide unbiased data regarding causes of sudden death in individuals with CIEDs and improve surveillance for CIED problems.
Domiciliary oxygen--by liquid or concentrator? Working Group on Oxygen Therapy of IUATLD
Authors: P Howard, R de Haller
Journal: European Respiratory Journal (1991)

A few hypoxaemic and even less non-hypoxaemic patients with chronic obstructive pulmonary disease (COPD) benefit from portable oxygen. For these few, selected by double-blind tests against compressed air, liquid oxygen would seem more convenient and efficient. The extra cost of treating all hypoxaemic COPD patients with liquid oxygen by displacing the oxygen concentrator is not justified on the basis of current knowledge. The application of differing oxygen delivery systems to COPD patients requires more evaluation. The selection of patients for long-term oxygen therapy (LTOT) still presents major difficulties, which reflects on the numbers of patients likely to benefit from portable oxygen. No studies have yet shown the benefit of LTOT to chronic respiratory failure in other disorders such as lung fibrosis. As the pathophysiology is quite different, extension of the use of LTOT and portable oxygen to non-COPD patients must be treated with great caution.
CHEMICAL STUDIES IN THE EPILEPTIC SYNDROME
Authors: Helen Hopkins
Journal: American Journal of Psychiatry (1935)

The results of the present investigation, dealing with the rhythmic diurnal and nocturnal changes in blood chemistry and water balance, indicate that the quality of these changes may be correlated with the clinical observation of increased susceptibility to seizures during sleep, particularly after midnight. From the literature may be gained evidence for a shift of the blood and urinary titre toward the alkaline side during the early morning hours, as well as rising intracranial pressure and brain volume during sleep. In addition, the cholesterol content of the blood has been shown to fall consistently during sleep, reaching minimal values at 3 a. m. Each of these changes has been considered by others to be definitely related to the precipitation of seizures in susceptible individuals. The acid-base, water-shifting, and ionic blood changes of the present experiments are qualitatively of the type to encourage the development of seizures during the latter half of the period of sleep. These rhythmic changes are observed to occur in the normal as well as in the epileptic subjects, and may be interpreted as the normal rhythm for blood chemistry.
Linking superinhibitory PLN mutations to CaMKII activation: a new arrhythmogenic mechanism in genetic DCM?
Authors: Michael Wagner, Silvio Weber, Ali El-Armouche
Journal: Cardiovascular Research (2015)

This editorial refers to ‘A novel human R25C-phospholamban mutation is associated with super-inhibition of calcium cycling and ventricular arrhythmia’ by G.-S. Liu et al. , pp. 164–174. 

Genetically caused dilated cardiomyopathy (DCM) is typically diagnosed between 40 and 60 years and patients often present with heart failure or advanced arrhythmia, which may possibly lead to stroke or sudden cardiac death.1 While potentially lethal arrhythmias are commonly found in end-stage heart failure independent of its aetiology, there is growing evidence that in certain cases of DCM, arrhythmia and sudden cardiac death are present already in early stages and may precede the development of overt heart failure. So far, this subset of cases has been linked to mutations in LMNA , coding for lamin A/C, and SCN5A , coding for the cardiac fast Na+ channel α-subunit NaV1.5.1 In the recent years, also a mutation in PLN , coding for phospholamban (PLB), R14del, has been connected to this subset of DCM.2–4

PLB is the main regulator of SERCA …
Abstract 17252: Texas Adolescent Athlete Heart Screening Registry (TAAHSR): a Statewide Screening Study
Authors: Silvana M Lawrence, Arnold Fenrich, Harold W Kohl, Patrick W Austin, Jennifer Y Hutchings, Michelle Garcia, Michael Guerrero, George Rodgers
Journal: Circulation (2011)

Background: Sudden cardiac death (SCD) in athletes, though a rare event, continues to claim lives every year. Optimal athlete screening strategies have proved to be elusive. Hypothesis: Statewide screening of high school student athletes (SA) for hypertrophic cardiomyopathy (HCM) and other conditions associated with SCD is feasible and establishing a registry may lead to reduction in SCD and improved quality of life. Objective: Identify high school SA at risk for SCD due to presence of HCM and/or other conditions associated with SCD through screening events in the state of Texas, and establish a statewide data bank (registry). Methods: The target population in this observational screening study included high school age SA who voluntarily presented at screening venues in Texas from March 2010-April 2011. Demographic and cardiac history data, 12-lead ECG and limited two-dimensional echocardiogram (L-ECHO) were obtained on all SA. Studies were read either on site or remotely subsequent to data acquisition. Athletes with positive screenings (SCD-related or other abnormal findings) were referred for complete cardiology evaluation. Follow up data were compared to screening results. Results: Of 1002 SA screened, 840 agreed to participate in TAAHSR (71% male). Most SA were in grades 9 and 10 (63%), mostly White (55%) or Hispanic (34%). Fifty one percent reported positive at least once in the history. A total of 94 SA (11.2%) had positive screening on ECG (9.6%) and/or L-ECHO (2.1%) and referral was made. Of the 94 SA referred, follow up data were obtained in 91 (98%): Data were still pending at the time in 44 (48%) and complete in 47 (52%). Of the 94, 22 were referred for SCD-related findings: 19 ECG (15 HCM, 3 WPW and 1 Brugada) and 3 L-ECHO (1 HCM, 2 aortic root dilation). Of the 47 SA, screening findings were confirmed in 12 (25%), 3 with WPW (2 underwent ablation) and 1 with LVH considered ‘benign’ but not HCM. Conclusion: Establishing a registry with statewide screenings in SA for SCD-related conditions is feasible and longitudinal data to assess effectiveness is highly needed at the present time. To date, 3 SA (0.36%) were identified with SCD-related condition (WPW). Future work on cost-effectiveness analysis is imperative to determine value of widespread SA screening for SCD.
Current perspectives on radiation-induced breast cancer.
Authors: P E Goss, S Sierra
Journal: Journal of Clinical Oncology (1998)

PURPOSE An approach to screening and detection of radiation-induced breast cancer is offered. Primary and secondary prevention strategies are suggested and the need for prospective clinical trials is emphasized. METHODS Data are reviewed from published evidence of radiation-induced breast cancer secondary to atomic bomb radiation, occupational, and therapeutic exposure, especially that incurred during successful treatment of Hodgkin's disease (HD). Preclinical studies are reviewed to explore potential risk factors. RESULTS Risk factors evident in the link between radiation and breast cancer include the differentiation of breast tissue as mediated by age and hormonal influence. Evidence is presented exploring the link between genetics and breast cancer, including specific genes such as the BRCA1 and BRCA2 genes, the p53 gene, the ataxia telangiectasia (AT) gene, and other nonspecific alterations in DNA repair proficiency. In light of these findings, steps toward primary prevention are discussed, including avoiding radiation exposure, genetic screening, and manipulation of the hormonal milieu. Secondary prevention may also be possible with the use of tamoxifen, low-fat diets, and/or the consumption of flaxseed. CONCLUSION Our current recommendations for patients irradiated before 30 years of age for Hodgkin's disease include breast self-examination (BSE) monthly, yearly mammography 8 years postirradiation, and regular physical examinations every 6 months. Given the clear link between radiation exposure and breast cancer, we strongly recommend a prospective trial randomize patients to different levels of intensity of surveillance to monitor the efficacy of such screening efforts.
A case of primary tracheal B cell lymphoma leading to obstruction of central airway
Authors: Seda Tural Onur, Levent Dalar, Sinem Nedime Sokucu, Ahmet Levent Karasulu, Ramazan Celik, Aysegul Akbas, Nurdan Simsek, Sedat Altin
Journal: European Respiratory Journal (2012)

Primary malignant tumors of the trachea are rare; and they follow up such as asthma, chronic obstructive pulmonary disease incorrect diagnoses. Tracheal tumors are rare and the incidence less than 0,01% and the majority of squamous cell carcinoma. Very few cases of primary lymphoma of the trachea have been reported so far. 72 years old male patient admitted to our clinic with complaints of shortness of breath, bronchoscopy showed a mass obstructing the tracheal lumen of the al)most complete.  Diode laser and coring was performed for emergency desobstruction.  There was no another focus of the patient9ssystemic screening whose pathological result is B-cell lymphoma. Reported due to a rare tracheal tumor.
Prospective evaluation of carcinoembryonic antigen levels and alternating chemotherapeutic regimens in metastatic breast cancer.
Authors: C L Loprinzi, D C Tormey, P Rasmussen, G Falkson, T E Davis, H C Falkson, A Y Chang
Journal: Journal of Clinical Oncology (1986)

Ninety-seven eligible and evaluable women with metastatic breast cancer were placed on a prospective clinical protocol to evaluate the use of continuous cyclic therapy with dibromodulcitol, doxorubicin, vincristine, tamoxifen, and fluoxymesterone (DAVTH) v DAVTH alternating with cyclophosphamide, methotrexate, 5-fluorouracil, and prednisone (CMFP); and the use of pretreatment and serial carcinoembryonic antigen (CEA) levels in these patients. Continuous DAVTH and DAVTH/CMFP were equivalent therapies with respect to response rates, time to treatment failure (TTF), and survival. Pretreatment CEA levels were elevated (greater than 5 ng/mL) in 42/97 patients and less than 5 ng/mL in the remaining patients. Patients with elevated pretreatment CEA levels were more likely to be estrogen receptor (ER) positive (P = .006), to have prolonged disease-free intervals (P = .017), to have hepatic (P = .004) and/or osseous (P = .01) metastases, and to have multiple sites of metastatic disease (P = .004). Pretreatment CEA levels did not significantly predict for overall response rates, TTF, or survival; nonetheless, those patients with low pretreatment CEA levels had more complete responses (CRs) (16/55 v 4/42; P = .02). Serial CEA levels during therapy revealed a number of interesting patterns. During the first 4 months of treatment, serial CEA levels in responding patients either (1) progressively declined (15/29 women with elevated pretreatment CEA levels), or (2) initially rose significantly (mean, 243% of pretreatment value) and then declined (14/29 women with elevated pretreatment CEA levels). Peak CEA levels in the latter patients were seen 27 to 135 days following initiation of cytotoxic therapy. In some patients the initial increase in the CEA level was incorrectly interpreted as evidence of impending disease progression. CEA levels frequently increased around the time of clinical disease progression. However, rising CEA levels rarely provided a clinically meaningful lead time before the appearance of other clinical evidence of disease progression. These data suggest that routine pretreatment and monthly serial CEA levels in metastatic breast cancer patients have minimal use in clinical practice. Two further noteworthy findings were observed in this prospective study. First, patients with an unknown ER status had a prolonged median survival when compared with patients with ER positive or negative tumors; this appeared to be related to prolonged disease-free intervals in ER unknown patients. Second, two case of secondary acute leukemia were seen in patients treated with continuous DAVTH therapy.
Peripheral primitive neuroectodermal tumors in adults: documentation by molecular analysis.
Authors: E R Lawlor, J A Mathers, T Bainbridge, D E Horsman, A Kawai, J H Healey, A G Huvos, J A Bridge, M Ladanyi, P H Sorensen
Journal: Journal of Clinical Oncology (1998)

PURPOSE The Ewing tumor (ET) family of peripheral primitive neuroectodermal tumors (pPNETs) are primitive small round-cell tumors (SRCTs) of the bone and soft tissue that occur predominantly in children and adolescents. However, pPNETs only rarely enter the differential diagnosis of bone and soft tissue SRCTs in adults. Recently, gene fusions between the EWS gene and different members of the ETS transcription factor family have been shown to occur in virtually all pPNETs and thus constitute a pathognomonic marker for this tumor subclass. The aim of the present study was to document EWS/ETS fusion gene expression in suspected pPNETs of adults as objective evidence for the existence of this tumor family in older patients. PATIENTS AND METHODS The three contributing molecular diagnostic laboratories retrospectively compiled a cohort of all SRCT cases in which EWS/ETS gene fusions had been shown by molecular analysis. This cohort was surveyed for cases that occurred in patients aged 40 years or older, which were then analyzed for their clinical and pathologic features. RESULTS Nine patients between 40 and 65 years of age were found to have tumors positive for EWS/ETS gene fusions. Standard histopathologic and clinical features of these cases, other than age, were similar to those of childhood pPNETs. Patients were initiated on appropriate therapy after molecular analysis confirmed the diagnosis of pPNET. CONCLUSION Identification of an EWS/ETS gene fusion is useful in providing objective evidence of the diagnosis of pPNET in patients over the age of 40 years. This diagnosis should be considered in adults who present with bone and soft tissue SRCTs and appropriate biopsy specimens should be collected for molecular analysis at the time of diagnosis.
Incidence and survival of cancers among 1,054 hemophilia patients: A nationwide and 14‐year cohort study
Authors: Yung‐Chieh Huang, Yu‐Tse Tsan, Wei‐Cheng Chan, Jiaan‐Der Wang, Wei‐Min Chu, Yun‐Ching Fu, Kwok‐Man Tong, Ching‐Heng Lin, Shin‐Tsu Chang, Wen‐Li Hwang
Journal: American Journal of Hematology (2015)

As life expectancy increases in persons with hemophilia (PWH), more age-related diseases such as cancer emerge among this patient group. The aim of this study was to investigate incidence and survival of cancers among PWH in Taiwan. We analyzed data of 1,054 PWH retrieved from Taiwan's National Health Insurance Research Database between 1997 and 2010, by comparing variables to 10540 age- and gender-matched healthy individuals from the general population. There were 43 PWH and 178 individuals of general population with newly diagnosed cancer (RR 2.42, 95% CI 1.74-3.35). The cumulative incidences of cancer in PWH and the general population were 4.7 and 1.9%, respectively. Hepatocellular carcinoma (HCC) was the major type of cancer (17 cases) in PWH; cancer rate was still increased when HCC and HIV-related cancers were excluded (RR 1.66, 95% CI 1.06-2.59). There was no significant difference observed in lung, colorectal, or prostate cancer occurrence. Compared to the general population, PWH were younger at the time of cancer diagnosis (45.1 vs. 57.2 years old, P value < 0.001), and had fewer co-morbidities. Nineteen PWH with cancers died during the study period, and no bleeding-related death was recorded among these patients. The survival rate was not different between PWH and the general population, P = 0.86. In conclusion, the cumulative incidence of cancer among PWH was higher than the general population. PWH with cancer were younger and had fewer comorbidities, but the survival rates were similar in the two groups.
Association of Vitamin D Levels With Multiple Sclerosis Activity and Progression in Patients Receiving Interferon Beta-1b
Authors: Kathryn C. Fitzgerald, Kassandra L. Munger, Karl Köchert, Barry G. W. Arnason, Giancarlo Comi, Stuart Cook, Douglas S. Goodin, Massimo Filippi, Hans-Peter Hartung, Douglas R. Jeffery, Paul O’Connor, Gustavo Suarez, Rupert Sandbrink, Ludwig Kappos, Christoph Pohl, Alberto Ascherio
Journal: JAMA Neurology (2015)

<h3>Importance</h3> Low serum 25-hydroxyvitamin D (25[OH]D) levels are associated with an increased risk of multiple sclerosis (MS) as well as with increased disease activity and rate of progression in clinically isolated syndromes and early MS. <h3>Objective</h3> To assess the association between 25(OH)D and disease course and prognosis in patients with relapsing-remitting MS treated with interferon beta-1b. <h3>Design, Setting, and Participants</h3> We conducted a prospective cohort study assessing 25(OH)D levels and subsequent MS disease course and progression characterized by magnetic resonance imaging (MRI) and clinical end points. The study took place between November 2003 and June 2005; data analysis was performed between June 2013 and December 2014. The study was conducted among participants in the Betaferon Efficacy Yielding Outcomes of a New Dose (BEYOND) study, a large, phase 3, prospective, multicenter, blinded, randomized clinical trial. Patients were monitored for at least 2 years. Clinic visits were scheduled every 3 months, and MRI was performed at baseline and annually thereafter. Eligible patients included 1482 participants randomized to receive 250 μg or 500 μg of interferon-1b with at least 2 measurements of 25(OH)D obtained 6 months apart. <h3>Exposures</h3> Serum 25(OH)D measurements were performed at baseline, 6 months, and 12 months. <h3>Main Outcomes and Measures</h3> Main outcomes included cumulative number of new active lesions (T2 lesions and gadolinium acetate–enhancing lesions), change in normalized brain volume, relapse rate, and progression determined by the Expanded Disability Status Scale (EDSS). Statistical analyses were adjusted for age, sex, randomized treatment, region, disease duration, and baseline EDSS score. <h3>Results</h3> Overall, average 25(OH)D levels in 1482 patients were significantly inversely correlated with the cumulative number of new active lesions between baseline and the last MRI, with a 50.0-nmol/L increase in serum 25(OH)D levels associated with a 31% lower rate of new lesions (relative rate [RR], 0.69; 95% CI, 0.55-0.86;<i>P</i> = .001). The lowest rate of new lesions was observed among patients with 25(OH)D levels greater than 100.0 nmol/L (RR, 0.53; 95% CI, 0.37-0.78;<i>P</i> = .002). No significant associations were found between 25(OH)D levels and change in brain volume, relapse rates, or EDSS scores. Results were consistent following adjustment for HLA-DRB1*15 or vitamin D–binding protein status. <h3>Conclusions and Relevance</h3> Among patients with MS treated with interferon beta-1b, higher 25(OH)D levels were associated with lower rates of MS activity observed on MRI. Results for brain atrophy and clinical progression were more equivocal.