tbuckley/cabot-search huggingface dataset:
Clinical and healthcare status of patients with neurofibromatosis type 1
Authors: A.K. Langenbruch, M. Augustin, S. Granström, L. Kluwe, V.F. Mautner
Journal: British Journal of Dermatology (2011)

Journal Article Clinical and healthcare status of patients with neurofibromatosis type 1 Get access A.K. Langenbruch, A.K. Langenbruch German Centre for Health Services Research in Dermatology (CVderm), Institute for Health Services Research in Dermatology and Nursing, and Search for other works by this author on: Oxford Academic Google Scholar M. Augustin, M. Augustin German Centre for Health Services Research in Dermatology (CVderm), Institute for Health Services Research in Dermatology and Nursing, and Search for other works by this author on: Oxford Academic Google Scholar S. Granström, S. Granström Department of Maxillofacial Surgery, Section Phakomatosis; University Medical Centre Hamburg‐Eppendorf, Martinistr. 52, 20246 Hamburg, Germany E‐mail: a.langenbruch@uke.de Search for other works by this author on: Oxford Academic Google Scholar L. Kluwe, L. Kluwe Department of Maxillofacial Surgery, Section Phakomatosis; University Medical Centre Hamburg‐Eppendorf, Martinistr. 52, 20246 Hamburg, Germany E‐mail: a.langenbruch@uke.de Search for other works by this author on: Oxford Academic Google Scholar V.F. Mautner V.F. Mautner Department of Maxillofacial Surgery, Section Phakomatosis; University Medical Centre Hamburg‐Eppendorf, Martinistr. 52, 20246 Hamburg, Germany E‐mail: a.langenbruch@uke.de Search for other works by this author on: Oxford Academic Google Scholar British Journal of Dermatology, Volume 165, Issue 1, 1 July 2011, Pages 225–227, https://doi.org/10.1111/j.1365-2133.2011.10296.x Published: 01 July 2011
Unlikely Suspects Identified in Neuroblastoma Conspiracy
Authors: Rene Bernards
Journal: Cancer Discovery (2014)

KIF1B is a candidate tumor-suppressor gene in neuroblastoma whose function is to mediate apoptosis when nerve growth factor becomes limiting in the developing nervous system. Chen and colleagues now provide mechanistic insight into how one of the protein products of this locus, KIF1Bβ, induces apoptosis.
Postremission Predictors of Relapse in Women With Eating Disorders
Authors: Pamela K. Keel, David J. Dorer, Debra L. Franko, Safia C. Jackson, David B. Herzog
Journal: American Journal of Psychiatry (2005)

The authors sought to evaluate patterns and predictors of relapse among women with eating disorders.Interviews were conducted biannually to annually to assess symptoms of eating disorders, axis I disorders, treatment, and psychosocial function on a weekly basis for women diagnosed with anorexia nervosa (N=136) or bulimia nervosa (N=110) and prospectively followed for 9 years. At the last follow-up, 229 (93%) of the subjects had been retained in the study group.Relapse occurred in 36% of the women with anorexia nervosa and 35% of the women with bulimia nervosa. Women with intake diagnoses of anorexia nervosa, restricting subtype, tended to develop bulimic symptoms during relapse, whereas women with intake diagnoses of anorexia nervosa, binge-purge subtype, or bulimia nervosa tended to return to bulimic patterns during relapse. Greater body image disturbance contributed to a risk of relapse in both eating disorders, and worse psychosocial function increased the risk of relapse in bulimia nervosa.These results may explain the long-term efficacy of interpersonal therapy for bulimia nervosa and suggest that focused body image work during relapse prevention may enhance long-term recovery from eating disorders.
Families in Pain: Children, Siblings, Spouses, and Parents of the Mentally Ill Speak Out; The Caring Family: Living With Chronic Mental Illness; The 36-Hour Day: A Family Guide to Caring for Persons With Alzheimer's Disease, Related Dementing Illnesses, and Memory Loss in Later Life
Authors: JOHN A. TALBOTT, SHIRLEY STARR
Journal: American Journal of Psychiatry (1983)
Effectiveness of Bystander Cardiopulmonary Resuscitation and Survival Following Out-of-Hospital Cardiac Arrest
Authors: E. John Gallagher
Journal: JAMA (1995)

To examine the independent relationship between effectiveness of bystander cardiopulmonary resuscitation (CPR) and survival following out-of-hospital cardiac arrest.Prospective observational cohort.New York City.A total of 2071 consecutive out-of-hospital cardiac arrests meeting Utstein criteria.Trained prehospital personnel assessed the quality of bystander CPR on arrival at the scene. Satisfactory execution of CPR required performance of both adequate compressions and ventilations in conformity with current American Heart Association guidelines.Adjusted association between CPR effectiveness and survival. Survival was defined as discharge from hospital to home.Outcome was determined on all members of the inception cohort--none were lost to follow-up. When the association between bystander CPR and survival was adjusted for effectiveness of CPR in the parent data set (N = 2071), only effective CPR was retained in the logistic model (adjusted odds ratio [OR] = 5.7; 95% confidence interval [CI], 2.7 to 12.2; P < .001). Of the subset of 662 individuals (32%) who received bystander CPR, 305 (46%) had it performed effectively. Of these, 4.6% (14/305) survived vs 1.4% (5/357) of those with ineffective CPR (OR = 3.4; 95% CI, 1.1 to 12.1; P < .02). After adjustment for witness status, initial rhythm, interval from collapse to CPR, and interval from collapse to advanced life support, effective CPR remained independently associated with improved survival (adjusted OR = 3.9; 95% CI, 1.1 to 14.0; P < .04).The association between bystander CPR and survival in out-of-hospital cardiac arrest appears to be confounded by CPR quality. Effective CPR is independently associated with a quantitatively and statistically significant improvement in survival.
Fluoroestradiol Positron Emission Tomography Reveals Differences in Pharmacodynamics of Aromatase Inhibitors, Tamoxifen, and Fulvestrant in Patients with Metastatic Breast Cancer
Authors: Hannah M. Linden, Brenda F. Kurland, Lanell M. Peterson, Erin K. Schubert, Julie R. Gralow, Jennifer M. Specht, Georgiana K. Ellis, Thomas J. Lawton, Robert B. Livingston, Philip H. Petra, Jeanne M. Link, Kenneth A. Krohn, David A. Mankoff
Journal: Clinical Cancer Research (2011)

To determine, by molecular imaging, how in vivo pharmacodynamics of estrogen-estrogen receptor (ER) binding differ between types of standard endocrine therapy.
Progress in Screening for Neural Tube Defects
Authors: James N. Macri
Journal: JAMA (1977)

To the Editor.—  Screening of an unselected pregnant population for neural tube defects using maternal serum αfetoprotein (AFP) represents an important step toward the prenatal diagnosis of a majority of these serious congenital anomalies. This concepthas been alluded to by Macri et al (236:1251, 1976).  The first such screening program in the United States has been initiated at the Nassau County Medical Center in October 1976. Normal maternal serum AFP values have now been established in more than 2,500 pregnancies. 1 To date, 1,337 nonrisk pregnancies have been screened. The Table represents a summary of findings subsequent to the initial screening observation of two elevated serum AFP levels between the 15th and 20th weeks of gestation.  Report of Cases.—Case 1.—  Maternal serum was initially obtained during the 18th week of gestation from an 18-year-old primagravida with no history of birth defects. This sample contained 522 ng/ml
Tuberculosis during TNF-α inhibitor therapy, despite screening: Table 1
Authors: Regina W Hofland, Steven F T Thijsen, Marc A M T Verhagen, Yolande Schenk, Ailko W J Bossink
Journal: Thorax (2013)

As part of a prospective study on the safety of TNF-α inhibitor therapy after screening for and treatment of latent tuberculosis infection (LTBI), we report two patients who developed active tuberculosis (TB) infection during TNF-α inhibitor therapy, despite negative screening for LTBI. The clinical history is suggestive of a primary infection acquired during travelling to TB-endemic countries. In this lesson of the month we would like to highlight the risk of travelling to TB-endemic areas in patients treated with TNF-α inhibitor therapy. Screening for latent tuberculosis infection is not enough to prevent tuberculosis in patients treated with TNF-α inhibitor therapy.
Intravenous Immunoglobulin Attenuates Experimental Autoimmune Arthritis by Inducing Reciprocal Regulation of Th17 and Treg Cells in an Interleukin‐10–Dependent Manner
Authors: Seon‐Yeong Lee, Young‐Ok Jung, Jun‐Geol Ryu, Chang‐Min Kang, Eun‐Kyung Kim, Hye‐Jin Son, Eun‐Ji Yang, Ji‐Hyeon Ju, Young‐Sun Kang, Sung‐Hwan Park, Ho‐Youn Kim, Mi‐La Cho
Journal: Arthritis & Rheumatology (2014)

Intravenous immunoglobulin (IVIG) is used as a therapeutic agent in various autoimmune diseases. The aims of this study were to investigate the therapeutic effects of IVIG on collagen-induced arthritis (CIA) and identify the mechanism responsible for any therapeutic effects.IVIG was administered to mice with CIA, and the in vivo effects were determined. Th17 and Treg cell frequencies were analyzed by flow cytometry, and cytokine levels in the supernatant were measured by enzyme-linked immunosorbent assay. Subpopulations of T cells and B cells in the spleen were assessed by confocal microscopy.The arthritis severity score and incidence of arthritis were lower in mice treated with IVIG compared with untreated mice. Histopathologic analysis showed less joint damage in mice treated with IVIG. The expression of proinflammatory cytokines, specific type II collagen antibodies, and osteoclast markers was significantly reduced in mice treated with IVIG. Administration of IVIG induced increased FoxP3 expression and inhibited Th17 cell development. The number of FoxP3+ Treg cells was increased, and the number of Th17 cells was decreased in the spleens of mice treated with IVIG. The number of FoxP3+ follicular helper T cells was increased, and subsequent maturation of germinal center B cells was inhibited by IVIG. In addition, IVIG up-regulated interleukin-10 (IL-10) and Fcγ receptor IIB expression. The treatment effects of IVIG on arthritis were lost in IL-10-knockout mice.These results showed that IVIG has therapeutic effects by modulating CD4+ T cell differentiation. The therapeutic effects of IVIG are dependent on IL-10.
Hypoxia: a critical regulator of early human tendinopathy
Authors: Neal L Millar, James H Reilly, Shauna C Kerr, Abigail L Campbell, Kevin J Little, William J Leach, Brian P Rooney, George A C Murrell, Iain B McInnes
Journal: Annals of the Rheumatic Diseases (2011)

<h3>Objectives</h3> To seek evidence for the role of hypoxia in early human tendinopathy, and thereafter to explore mechanisms whereby tissue hypoxia may regulate apoptosis, inflammatory mediator expression and matrix regulation in human tenocytes. <h3>Methods</h3> Fifteen torn supraspinatus tendon (established pathology) and matched intact subscapularis tendon (representing 'early pathology') biopsies were collected from patients undergoing arthroscopic shoulder surgery. Control samples of the subscapularis tendon were collected from 10 patients undergoing arthroscopic stabilisation surgery. Markers of hypoxia were quantified by immunohistochemical methods. Human tendon-derived primary cells were derived from hamstring tendon tissue obtained during hamstring tendon anterior cruciate ligament reconstruction. The impact of hypoxia upon tenocyte biology ex vivo was measured using quantitative real-time PCR, multiplex cytokine assays, apoptotic proteomic profiling, immunohistochemistry and annexin V fluorescence-activated cell sorter staining. <h3>Results</h3> Increased expression of hypoxia-inducible factor 1α, Bcl-2 and clusterin was detected in subscapularis tendon samples compared with both matched torn samples and non-matched control samples (p&lt;0.01). Hypoxic tenocytes exhibited increased production of proinflammatory cytokines (p&lt;0.001), altered matrix regulation (p&lt;0.01) with increased production of collagen type III operating through a mitogen-activated protein kinase-dependent pathway. Finally, hypoxia increased the expression of several mediators of apoptosis and thereby promoted tenocyte apoptosis. <h3>Conclusion</h3> Hypoxia promotes the expression of proinflammatory cytokines, key apoptotic mediators and drives matrix component synthesis towards a collagen type III profile by human tenocytes. The authors propose hypoxic cell injury as a critical pathophysiological mechanism in early tendinopathy offering novel therapeutic opportunities in the management of tendon disorders.
Chronic obstructive pulmonary disease mortality and prevalence: the associations with smoking and poverty: a BOLD analysis—authors’ reply
Authors: Peter Burney, Bernet Kato, Christer Janson, David Mannino, Michael Studnicka, Wan Tan, Eric Bateman, Ali Koçabas, William M Vollmer, Thorarrin Gislason, Guy Marks, Parvaiz A Koul, Louisa Gnatiuc, Sonia Buist
Journal: Thorax (2014)

Chronic obstructive pulmonary disease mortality and prevalence: the associations with smoking and poverty: a BOLD analysis-authors' replyWe are grateful to Dr Stanojevic and her colleagues 1 for their interest in our paper. 2 On their first point we assume that they mean mortality rates not national prevalence rates.In either case the weak association between diagnosis and spirometric findings is already well known and unsurprising given that very few people have spirometric testing.In the case of death
Prediction of wheeze recurrence and severity in infants by composite epidemiological score
Authors: M.P. van der Schee, S. Hashimoto, A.C. Schuurman, N. Adriaens, A.B. Sprikkelman, E.G. Haarman, W.M.C. van Aalderen, P.J. Sterk
Journal: European Respiratory Journal (2013)

Rationale-Pre-school wheezing is associated with a diagnosis of asthma atschool-age. It is unclear to what extend early life epidemiological markers can predict wheeze severity and recurrence. Hypothesis-A composite score based on epidemiological data collected   Conclusion-A composite score of epidemiological markers at age 1 has a moderate predictive potential for the presence but not severity of recurrent wheeze at age 2.5. Simple epidemiological markers can help to identify infants at increased risk of sustained wheezing, but additional (bio)markers are needed.
Heart Valve Development
Authors: Michelle D. Combs, Katherine E. Yutzey
Journal: Circulation Research (2009)

Abstract : In recent years, significant advances have been made in the definition of regulatory pathways that control normal and abnormal cardiac valve development. Here, we review the cellular and molecular mechanisms underlying the early development of valve progenitors and establishment of normal valve structure and function. Regulatory hierarchies consisting of a variety of signaling pathways, transcription factors, and downstream structural genes are conserved during vertebrate valvulogenesis. Complex intersecting regulatory pathways are required for endocardial cushion formation, valve progenitor cell proliferation, valve cell lineage development, and establishment of extracellular matrix compartments in the stratified valve leaflets. There is increasing evidence that the regulatory mechanisms governing normal valve development also contribute to human valve pathology. In addition, congenital valve malformations are predominant among diseased valves replaced late in life. The understanding of valve developmental mechanisms has important implications in the diagnosis and management of congenital and adult valve disease.
Dacarbazine in Solitary Fibrous Tumor: A Case Series Analysis and Preclinical Evidence vis-à-vis Temozolomide and Antiangiogenics
Authors: S. Stacchiotti, M. Tortoreto, F. Bozzi, E. Tamborini, C. Morosi, A. Messina, M. Libertini, E. Palassini, D. Cominetti, T. Negri, A. Gronchi, S. Pilotti, N. Zaffaroni, P.G. Casali
Journal: Clinical Cancer Research (2013)

To explore the value of triazines in solitary fibrous tumor (SFT).We retrospectively reviewed 8 cases of patients with SFT treated with dacarbazine (1,200 mg/m(2) every 3 weeks) as from January 2012. Then, we studied a dedifferentiated-SFT subcutaneously xenotransplanted into severe combined immunodeficient (SCID) mice. Dacarbazine, temozolomide, sunitinib, bevacizumab, and pazopanib were administered at their reported optimal doses for the mouse model when mean tumor volume (TV) was about 80 mm(3); each experimental groups included 6 mice. Drug activity was assessed as tumor volume inhibition percentage (TVI%). Dacarbazine was tested according to two different schedules of administration. One hunded twenty days after treatment interruption, mouse tumor samples were analyzed.Among the eight patients treated with dacarbazine, best response evaluation criteria in solid tumors responses (RECIST) were three partial responses, 4 stable disease, 1 progression. Two responsive patients had paraneoplastic hypoglycemia that disappeared after 10 days from starting dacarbazine. In the dedifferentiated-SFT xenograft model, dacarbazine and temozolomide showed the highest antitumor activity (about 95% TVI), confirmed pathologically. Sunitinib and pazopanib were only marginally active (52% and 41% TVI, respectively), whereas bevacizumab caused a 78% TVI. No tumor regrowth was observed up to 100 days from end of treatment with temozolomide and dacarbazine, whereas secondary progression followed sunitinib, pazopanib, and bevacizumab interruption.Dacarbazine as single agent has antitumor activity in SFT. Our preclinical results suggest a cytotoxic effect of temozolomide and dacarbazine, as compared with a cytostatic role for sunitinib, pazopanib, and bevacizumab. A phase II study on dacarbazine in advanced SFT is planned.
Side Effects of Adjuvant Endocrine Treatment in Premenopausal Breast Cancer Patients: A Prospective Randomized Study
Authors: Marianne Nystedt, Gunilla Berglund, Christina Bolund, Tommy Fornander, Lars Erik Rutqvist
Journal: Journal of Clinical Oncology (2003)

Purpose: To compare the effect of adjuvant endocrine therapies with and without chemotherapy on physical symptoms, anxiety, and depressive symptoms in premenopausal women with breast cancer in a randomized clinical trial (the Zoladex in Premenopausal Patients trial). Patients and Methods: The patients were randomly assigned to goserelin, goserelin plus tamoxifen, tamoxifen alone, or no endocrine therapy. The duration of the endocrine treatment was 2 years. The groups were observed for 3 years after primary treatment (ie, during 2 years of active treatment as well as 1 year after cessation of the adjuvant endocrine therapy). All patients with node-positive disease received adjuvant chemotherapy with cyclophosphamide, methotrexate, and fluorouracil (CMF), which was given concurrently with the endocrine treatment. Results: Patients treated with CMF typically reported higher levels of physical symptoms than did patients who did not receive CMF. It was only among patients who did not receive chemotherapy that the endocrine treatment had differential effects. Goserelin was most burdensome and resulted in similar symptom levels as those of CMF, whereas the side effects of tamoxifen alone were milder. After cessation of the endocrine treatment, the side effects diminished in patients who had not received CMF, whereas patients treated with CMF reported ongoing problems at the 3-year follow-up. In contrast, anxiety and depressive symptoms were not significantly affected by endocrine treatment or chemotherapy during the 3 years of assessment. Conclusion: Goserelin and tamoxifen resulted in menopausal symptoms, but these symptoms were reversible. However, women treated with CMF experienced physical symptoms throughout the whole study period.
Recovery of plasma corticotrophin and cortisol levels after three-week course of prednisolone.
Authors: J Webb, T J Clark
Journal: Thorax (1981)

Patients with chronic airflow obstruction were given a three-week course of prednisolone 40 mg per day. The basal plasma cortisol level and response to tetracosactrin were depressed after such a course. Basal plasma cortisol and corticotrophin (ACTH) levels were measured on five consecutive days after three weeks of treatment with prednisolone and were found to rise simultaneously to control levels within three days. Pituitary and adrenal functions were depressed for four days after short high dose courses of corticosteroids and patients may be at risk if they encounter stress during this time.
A 3-Plex Methylation Assay Combined with the <i>FGFR3</i> Mutation Assay Sensitively Detects Recurrent Bladder Cancer in Voided Urine
Authors: Raju Kandimalla, Roy Masius, Willemien Beukers, Chris H. Bangma, Torben F. Orntoft, Lars Dyrskjot, Nikki van Leeuwen, Hester Lingsma, Angela A.G. van Tilborg, Ellen C. Zwarthoff
Journal: Clinical Cancer Research (2013)

DNA methylation is associated with bladder cancer and these modifications could serve as useful biomarkers. FGFR3 mutations are present in 60% to 70% of non-muscle invasive bladder cancer (NMIBC). Low-grade bladder cancer recurs in more than 50% of patients. The aim of this study is to determine the sensitivity and specificity of a urine assay for the diagnosis of recurrences in patients with a previous primary NMIBC G1/G2 by using cystoscopy as the reference standard.We selected eight CpG islands (CGI) methylated in bladder cancer from our earlier genome-wide study. Sensitivity of the CGIs for recurrences detection was investigated on a test set of 101 preTUR urines. Specificity was determined on 70 urines from healthy males aged more than 50 years. A 3-plex assay for the best combination was developed and validated on an independent set of 95 preTUR, recurrence free, and nonmalignant urines (n=130).The 3-plex assay identified recurrent bladder cancer in voided urine with a sensitivity of 74% in the validation set. In combination with the FGFR3 mutation assay, a sensitivity of 79% was reached (specificity of 77%). Sensitivity of FGFR3 and cytology was 52% and 57%, respectively.The combination of methylation and FGFR3 assays efficiently detects recurrent bladder cancer without the need for stratification of patients regarding methylation/mutation status of the primary tumor. We conclude that the sensitivity of this combination is in the same range as cystoscopy and paves the way for a subsequent study that investigates a modified surveillance protocol consisting of the urine test followed by cystoscopy only when the urine test is positive.
Gamma secretase inhibition promotes hypoxic necrosis in mouse pancreatic ductal adenocarcinoma
Authors: Natalie Cook, Kristopher K. Frese, Tashinga E. Bapiro, Michael A. Jacobetz, Aarthi Gopinathan, Jodi L. Miller, Sudhir S. Rao, Tim Demuth, William J. Howat, Duncan I. Jodrell, David A. Tuveson
Journal: The Journal of Experimental Medicine (2012)

Pancreatic ductal adenocarcinoma (PDA) is a highly lethal disease that is refractory to medical intervention. Notch pathway antagonism has been shown to prevent pancreatic preneoplasia progression in mouse models, but potential benefits in the setting of an established PDA tumor have not been established. We demonstrate that the gamma secretase inhibitor MRK003 effectively inhibits intratumoral Notch signaling in the KPC mouse model of advanced PDA. Although MRK003 monotherapy fails to extend the lifespan of KPC mice, the combination of MRK003 with the chemotherapeutic gemcitabine prolongs survival. Combination treatment kills tumor endothelial cells and synergistically promotes widespread hypoxic necrosis. These results indicate that the paucivascular nature of PDA can be exploited as a therapeutic vulnerability, and the dual targeting of the tumor endothelium and neoplastic cells by gamma secretase inhibition constitutes a rationale for clinical translation.