tbuckley/cabot-search huggingface dataset:
Effects of an Estrogen Receptor α Variant
Authors: Deborah J. Clegg, Biff F. Palmer
Journal: New England Journal of Medicine (2013)

Quaynor et al. (July 11 issue) 1 describe a young woman with delayed puberty and estrogen resistance who had a mutation in the gene encoding estrogen receptor α (ESR1).The findings in this patient are similar to those observed in transgenic mice generated by introducing a transcriptional block in the ability of estrogen receptor α to bind and activate estrogen-response elements in DNA (classical regulation of estrogen receptor α) to alter target gene transcription.These mice have intact estrogen-response element-independent activity (nonclassical, or membrane-associated, estrogen receptor α activity) and resemble the phenotype of the patient, in that they have hypoplastic mammary glands, an-ovulation, and altered steroidogenesis. 2,3In addition, the mice have normal body weight, adiposity, locomotor activity, and glucose homeostasis, similar to the findings in the patient.The data from these transgenic mice support the authors' supposition that nonclassical regulation of estrogen receptor α is sufficient to protect against the obesity-metabolic syndrome phenotype associated with a total loss of activity of estrogen receptor α but is not sufficient to rescue the infertility phenotype.We offer the murine findings as support of the authors' initial claims, and we believe these findings further implicate a differential role for classical versus nonclassical activity of estrogen receptor α in the regulation of energy and reproductive homeostasis.
Malignant Progenitors From Patients With Acute Myelogenous Leukemia Are Sensitive to a Diphtheria Toxin-Granulocyte-Macrophage Colony-Stimulating Factor Fusion Protein
Authors: Donna E. Hogge, Cheryl L. Willman, Robert J. Kreitman, Marc Berger, Philip D. Hall, Kenneth J. Kopecky, Chris McLain, Edward P. Tagge, Connie J. Eaves, Arthur E. Frankel
Journal: Blood (1998)
Using forceps biopsy during endobronchial ultra sound is feasible in routine practise
Authors: Bruno Escarguel, Georges Thomas, Cecile Tchouhdjian, Alexandre Chollat Namy, Cyril Foa, Jean Baptiste Paoli, Alain Poisson
Journal: European Respiratory Journal (2012)

Introduction:

EBUS is becoming a gold standard in exploring mediastinum abnormality. Especialy during a staging in lung cancer, big tissue become more necessary.

Forceps biopsy have been used in biopsy of subcarinal masses with interesting results for lymphomas or sarcoidosis, without major complications. What happend in routine practise and using forceps for other mediastinal sites ?

Material and methods:

In the thoracic endoscopic unit of the hopital St Joseph (Marseille, France) during June 2011-decembrer 2011, we used a pediatric forceps biopsy after each puncture by EBUS TBNA. 13 patients were analysed, 16 mediastinal sites (7, 4R, 10R, 11R, 12R, 4L, 11L) were biopsied (3 histological specimens) after using a 21 or 22G needle. All the procedures were doing under general anesthesia using a laryngeal mask.

Results:

The average diameter of lymph nodes was 15,3 mm. In only two samples, the results were non significant. Concerning cancerous results (50%), the forceps biopsy did not increase diagnostic yield versus TBNA (Slides or cytolit). Biomarker analyses were possible for metastasis patients. In one case, sarcoidosis diagnosis was made only with forceps biopsy. There were no immediate complications.

Conclusion:

Using forceps biopsy during EBUS is feasible and safe. We can perform this procedure for the different sites of the mediastinum. The result doesn't show an increasing diagnostic value in lung cancer but could be interresting for lymphoma or non malignant disorders as sarcoidosis.
Alisporivir plus ribavirin, interferon free or in combination with pegylated interferon, for hepatitis C virus genotype 2 or 3 infection
Authors: Jean‐Michel Pawlotsky, Robert Flisiak, Shiv K. Sarin, Jens Rasenack, Teerha Piratvisuth, Wan‐Long Chuang, Cheng‐Yuan Peng, Graham R. Foster, Samir Shah, Heiner Wedemeyer, Christophe Hézode, Wei Zhang, Kelly A. Wong, Bin Li, Claudio Avila, Nikolai V. Naoumov
Journal: Hepatology (2015)

Alisporivir is a cyclophilin inhibitor with pan‐genotypic anti–hepatitis C virus (HCV) activity and a high barrier to viral resistance. The VITAL‐1 study assessed alisporivir as interferon (IFN)‐free therapy in treatment‐naïve patients infected with HCV genotype 2 or 3. Three hundred forty patients without cirrhosis were randomized to: arm 1, alisporivir (ALV) 1,000 mg once‐daily (QD); arm 2, ALV 600 mg QD and ribavirin (RBV); arm 3, ALV 800 mg QD and RBV; arm 4, ALV 600 mg QD and pegylated IFN (Peg‐IFN); or arm 5, Peg‐IFN and RBV. Patients receiving IFN‐free ALV regimens who achieved rapid virological response (RVR) continued the same treatment throughout, whereas those with detectable HCV RNA at week 4 received ALV, RBV, and Peg‐IFN from weeks 6 to 24. Overall, 300 patients received ALV‐based regimens. In arm 1 to arm 4, the intent‐to‐treat rates of sustained virological response (SVR) 24 weeks after treatment (SVR24) were from 80% to 85%, compared with 58% (n = 23 of 40) with Peg‐IFN/RBV. Per‐protocol analysis showed higher SVR24 rates in patients who received ALV/RBV, IFN‐free after RVR (92%; n = 56 of 61) than with ALV alone after RVR (72%; n = 13 of 18) or with Peg‐IFN/RBV (70%; n = 23 of 33). Both RVRs and SVRs to ALV IFN‐free regimens were numerically higher in genotype 3– than in genotype 2–infected patients. Viral breakthrough was infrequent (3%; n = 7 of 258). IFN‐free ALV treatment showed markedly better safety/tolerability than IFN‐containing regimens. Conclusions : ALV plus RBV represents an effective IFN‐free option for a proportion of patients with HCV genotype 2 or 3 infections, with high SVR rates for patients with early viral clearance. Further investigations of ALV in IFN‐free combination regimens with direct‐acting antiviral drugs deserve exploration in future trials. (H epatology 2015;62:1013‐1023)
Genetic polymorphisms in TNF genes and tuberculosis in cystic fibrosis patients
Authors: Galina Shmarina, Alexander Pukhalsky, Nikolai Kapranov, Vladimir Alioshkin
Journal: European Respiratory Journal (2011)

The genes for tumor necrosis factor-α (TNF-α;  TNF ) and lymphotoxin-α (LT-α;  LTA ) are arranged in tandem within MHC III region of chromosome 6 in the same transcriptional orientation. This formation is conserved, even in marsupials, suggesting that there may be some functional advantage to this arrangement. The proteins encoded by  TNF  and  LTA  are known to interact as complementary factors in various cell signaling networks. Single nucleotide polymorphism sites for  TNF  (-308 G>A) and  LTA  (252 A>G) were investigated with TaqMan allelic discrimination assay. 190 cystic fibrosis (CF) subjects were enrolled into the study. The distribution of allelic variants of  TNF  and  LTA  genes in CF patients did not differ from those in health subjects of Moscow and European populations. We did not find any association between  TNF  genotypes and CF lung disease progression. In the same time the carriers of -308A allele more frequently had asthma and other atopic disorders as compared to patients homozygous for -308G allele (14.9  vs  5.3%, p vs  32.4 pg/ml; p TNF  allele for asthma development and provide robust evidence that  LTA  gene variants are involved in tuberculosis etiology.
A Novel Prion Disease Presenting with Diarrhea and Autonomic Neuropathy
Authors: S Mead, S Gandhi, J Beck, D Caine, D Gallujipali, C Carswell, H Hyare, S Joiner, H Ayling, T Lashley, JM Linehan, H Al-Doujaily, B Sharps, T Revesz, M Sandberg, M Reilly, M Koltzenburg, A Forbes, P Rudge, S Brandner, JD Warren, Jdf Wadsworth, NW Wood, JL Holton, J Collinge
Journal: New England Journal of Medicine (2013)

Background. Human prion diseases, although variable in clinicopathological phenotype, generally present as neurologic or neuropsychiatric conditions associated with rapid multifocal central nervous system degeneration that is usually dominated by dementia and cerebellar ataxia. Approximately 15% of cases of recognized prion disease are inherited and associated with coding mutations in the gene encoding prion protein (PRNP). The availability of genetic diagnosis has led to a progressive broadening of the recognized spectrum of disease.
Methods. We used longitudinal clinical assessments over a period of 20 years at one hospital combined with genealogical, neuropsychological, neurophysiological, neuroimaging, pathological, molecular genetic, and biochemical studies, as well as studies of animal transmission, to characterize a novel prion disease in a large British kindred. We studied 6 of 11 affected family members in detail, along with autopsy or biopsy samples obtained from 5 family members.
Results. We identified a PRNP Y163X truncation mutation and describe a distinct and consistent phenotype of chronic diarrhea with autonomic failure and a length-dependent axonal, predominantly sensory, peripheral polyneuropathy with an onset in early adulthood. Cognitive decline and seizures occurred when the patients were in their 40s or 50s. The deposition of prion protein amyloid was seen throughout peripheral organs, including the bowel and peripheral nerves. Neuropathological examination during end-stage disease showed the deposition of prion protein in the form of frequent cortical amyloid plaques, cerebral amyloid angiopathy, and tauopathy. A unique pattern of abnormal prion protein fragments was seen in brain tissue. Transmission studies in laboratory mice were negative.
Conclusions. Abnormal forms of prion protein that were found in multiple peripheral tissues were associated with diarrhea, autonomic failure, and neuropathy. (Funded by the U.K. Medical Research Council and others.)
Abstract 4725: Comparative Analysis of Vascular Responses Following Stent Implantation in the Lesion Wth Acute Coronary Syndrome Between Paclitaxel-Eluting and Bare-Metal Stent Using Optical Coherence Tomography
Authors: Maoto Habara, Mitsuyasu Terashima, Tsuyoshi Ito, Nobuyoshi Tanaka, Kenya Nasu, Tatsuya Ito, Masashi Kimura, Mariko Ehara, Yoshihisa Kinoshita, Etsuo Tsuchikane, Yasushi Asakura, Osamu Katoh, Takahiko Suzuki
Journal: Circulation (2009)
Patients with no cystic fibrosis bronchiectasis colonized by pseudomonas aeruginosa: Role of inhaled colistin
Authors: Abel Trujillano Ruiz, María Jesús Avilés Inglés, María Teresa Antequera Lardón, Juan José Franco Miguel, María Carmen Abellán Martínez, Pedro Méndez Martínez, Jose Carlos Vicente López
Journal: European Respiratory Journal (2014)

Objective 

To describe the results of the use of IC in patients with non-cystic fibrosis(CF)bronchiectasis colonized by Pseudomonas aeruginosa in terms of efficiency and quality of life(QOL)

Material and Method 

Retrospective observational study.Patients who started treatment with IC within a period of 4 years were selected.For each patient we collected demographic,clinical and microbiological data evaluating whether the colonization was initial,intermittent or chronic.At the end of the treatment,efficacy was determined by examining the negative cultures and QOL according to the use of antibiotics and hospitalizations pre and post treatment as well as the adverse effects

Results 

58 patients who began treatment with IC with an average age of 70±15,4 years were included,43,1% were women.Regarding the type of colonization in 20 patients was initial,in 5 was intermittent and in 24 was chronic.At the time of data collection,32 patients had completed the treatment of which 11 defaulted(9 by adverse reactions and 2 for difficulty in handling the device).The average duration of treatment was 4,5±4 months and 17 negativized the cultures(9 with initial colonization,1 with intermittent and 7 with chronic).The average number of admissions before treatment was 2,5±2,2 versus 0,6±0,9 post-treatment.The average number of antibiotics that took previously to IC was 4,4±3,8 being 1,3±1,6 post-treatment

Conclusions 

The proportion of patients who negativized cultures with initial colonization is relevant(45%),thus concluding that early initiation improves efficiency

The reduction of antibiotic use and hospital admissions along with few adverse reactions,demonstrate the improvement in QOL of patients.
Vitamin D Deficiency In Asthma Patients With Allergic Bronchopulmonary Aspergillosis (ABPA)
Authors: Livingstone Chishimba, Weijuan Zhang, Andrew Mochridge Andrew Mochridge, Robert M. Niven, Khaled Al-Shar, David Denning
Journal: European Respiratory Journal (2013)

Background : Vitamin D deficiency is being increasingly recognized in chronic lung disease. Recent studies suggest that ABPA correlated with vitamin D deficiency among Cystic Fibrosis (CF) patients, and that heightened Th2 reactivity in ABPA correlated with lower mean serum vitamin D levels. However, this correlation remains unknown in asthma patients with ABPA.   Methods:  144 asthma patients (with ABPA, n=80), (without ABPA, n=64) who attended our hospital between January 2011 and May 2012 were prospectively evaluated for 25-hydroxyvitamin D (25-OHD3), lung function and IgE levels. Patients were retrospectively reviewed for clinical characteristics and prognosis compared with patients with normal VitD levels.   Results:  VitD deficiency ( (    CONCLUSION : Our data suggests that asthmatic ABPA patients may have higher vitamin D status than non-ABPA patients and that there may be an association between lung function and Vitamin D status. However, the causal effect relationship needs to be established.
Abstract 16881: Intracoronary Delivery of Allogeneic Mesenchymal Precursor Cells Directly after Acute Myocardial Infarction Improves Cardiac Function and Myocardial Perfusion and Decreases Adverse Left Ventricular Remodeling in Sheep
Authors: Jaco Houtgraaf, Renate de Jong, Daphne de Groot, Imo Hoeffer, Gerard Pasterkamp, Henricus Duckers
Journal: Circulation (2010)

Rationale Mesenchymal precursor cells (MPC) hold great promise for the treatment of cardiovascular disease. These multipotent cells produce a vast array of angiotrophic factors and immune-modulator...
The Potential Hazards of Hospital Consolidation
Authors: Tim Xu, Albert W. Wu, Martin A. Makary
Journal: JAMA (2015)

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
Evaluating the risk of lower respiratory tract infection (LRTI) hospitalizations due to respiratory distress syndrome (RDS) in late preterm
Authors: Katharina Buesch, Hoa Khong, Katherine Gooch
Journal: European Respiratory Journal (2011)

Background:  Premature birth results in underdeveloped lung function that increases their risk for respiratory infection and morbidity. The prevalence of RDS requiring intervention in premature births is significant, including those considered late preterm. RSV is the most prevalent cause for LRTI hospitalizations in infants. This study sought to determine what the independent risk of RDS diagnosis at birth has on LRTI hospitalisations in late preterm infants during the first year of life.   Methods:  Late premature infants (33-36 weeks gestational age) hospitalized within the first year of life for a LRTI were extracted from the Medical Claims I3 database (2000-2008). Univariate analyses were conducted to compare the differences between infants with and without an RDS event at birth. A multivariate model was developed to determine the independent association of RDS and LRTI hospitalization. Statistical significance was set at 0.05.   Results:  31178 late preterm infants (52% male) were identified during the study timeline. 2297 (7.4%) late preterm infants were hospitalized due to a LRTI; a hyaline membrane disease or RDS diagnosis (ICD-9 code: 769) was observed in 962 late preterms (3%). Multiple logistic regression analysis showed a strong and significant relationship between RDS and LRTI hospitalization (OR 5.2, 95% CI 4.4–6.2).   Conclusion:  Late preterm infants with RDS event at birth are 5 times more likely to be hospitalized due to LRTI during the first year of life compared to late preterm without RDS event at birth. This risk factor should be considered when evaluating a premature infants risk for respiratory hospitalization.
Objective measurement of compliance with nasal CPAP treatment for obstructive sleep apnoea syndrome
Authors: J Krieger, D Kurtz
Journal: European Respiratory Journal (1988)

Compliance with nasal continuous positive airway pressure (CPAP) has become a major concern, since this treatment is efficacious, but constraining. In 46 consecutive obstructive sleep apnoea (OSA) patients, we measured compliance with nasal CPAP by establishing a mean rate of use, with a built-in time counter read at three-month intervals, over a mean follow-up period of 232 +/- 27 days. The mean rate of use in the whole group was 5.14 +/- 0.31 hours per day. The acceptance rate was 90.9-93.2%, showing that patient acceptance is not a limitation in the use of nasal CPAP.
The prevalence and determinants of solar keratoses at a subtropical latitude (Queensland, Australia)
Authors: None Frost, None Green, None Williams
Journal: British Journal of Dermatology (1998)

We report the association between skin pigmentation and individual sun exposure, and the occurrence of solar keratoses (SKs) in an unselected population, quantified for the first time. SKs were examined in a representative sample of 197 residents of the community of Nambour in Queensland, Australia. Estimates of sun exposure were combined with a measure of ultraviolet (UV) flux to estimate actual UV exposure, both occupational and recreational, during childhood and adult life. The number of episodes of painful sunburn was used as a measure of intermittent, intense UV exposure. Eighty‐three participants (43%) had at least one SK, while 35 (18%) had more than 10 SKs diagnosed. The age‐ and sex‐adjusted odds ratios (ORs) for the development of SKs were higher in individuals with fair (OR = 14.1) or medium skin (OR = 6.5), compared with olive‐skinned individuals. Individuals with poor ability to develop a suntan were similarly at increased risk compared with others. High levels of occupational UV exposure during adult life were confirmed as being strongly associated with prevalent SKs (OR = 2.4 for heavy/maximal adult exposure), with an even stronger association seen in those individuals with multiple SKs (OR = 4.3 for maximal adult exposure). Although no clear association was demonstrated between SK prevalence and accumulated childhood sun exposure, a history of even one episode of sunburn in childhood was strongly associated with SK prevalence (peak OR of 5.9 for one sunburn).
Abstract 11561: Cardiac Perivascular Fibrosis is Associated with Impaired Coronary Blood Flow in Patients with Non-ischemic Heart Failure
Authors: Tatsuo Aoki, Zhehao Dai, Yoshihiro Fukumoto, Hiroaki Shimokawa
Journal: Circulation (2012)

Background: Although myocardial interstitial fibrosis has been demonstrated to play an important role in the pathogenesis of chronic heart failure, the role of perivascular fibrosis, another form of fibrosis, remains to be clarified. Methods: We examined 64 consecutive patients with non-ischemic heart failure (HF) in our hospital between January 2001 and April 2009. The underlying cause of HF included hypertrophic cardiomyopathy (HCM, n=16), hypertensive heart disease (HHD, n=11) and dilated cardiomyopathy (DCM, n=37) based on the findings by echocardiography, cardiac catheterization and endomyocardial biopsy (right ventricular side of the interventricular septum). We calculated the collagen volume fraction (CVF) and perivascular fibrosis ratio (PFR) in biopsy samples as follows; PFR = the area of perivascular fibrosis/the area of the vascular wall. We also examined Thrombolysis in Myocardial Infarction (TIMI) frame count in the left anterior descending coronary artery (LAD) to evaluate coronary blood flo...
Randomized Controlled Trials of Rheumatoid Arthritis Registered at ClinicalTrials.gov: What Gets Published and When
Authors: Nasim A. Khan, Manisha Singh, Horace J. Spencer, Karina D. Torralba
Journal: Arthritis & Rheumatology (2014)

Objective To examine characteristics associated with the publication and timeliness of publication of randomized controlled trials (RCTs) of treatment of rheumatoid arthritis (RA). Methods RA RCTs (phases II–IV) registered at ClinicalTrials.gov and completed by December 31, 2009 were identified. A standardized strategy was used to determine publication status and outcome assessment. The association of RCT characteristics recorded at ClinicalTrials.gov and study outcome with publication and time to publication were assessed. Results A search conducted at least 30 months after trial completion revealed that among 143 eligible RCTs, 95 (64.4%) were published. The 48 unpublished RCTs had enrolled &gt;10,000 patients. Efficacy outcomes could be ascertained for 127 of the RCTs. RCT publication was associated with positive outcome, with an adjusted odds ratio [OR] of 4.3 (95% confidence interval [95% CI] 1.8–10.2) ( P = 0.001), and marginally with RCT registration before completion (adjusted OR 0.4 [95% CI 0.1–1.0], P = 0.06). The estimated median time to publication was 38 months. Positive outcome was associated with earlier publication (adjusted hazard ratio 1.9 [95% CI 1.2–2.9], P = 0.006). RCTs completed in 2006–2007 or 2008–2009 were likely to be published sooner than RCTs completed in or before 2005. Sensitivity analyses to assess the impact of 16 RCTs with unknown outcome did not alter these findings, except in the highly implausible scenario of all such trials being positive. Conclusion Positive study outcome was associated with publication and timeliness of publication despite registration in a publicly available registry. A substantial minority of RA RCTs remained unpublished. Efforts to improve transparency in reporting of clinical trials need to continue.