tbuckley/cabot-search huggingface dataset:
Phenotype and genotype in adult patients with cystic fibrosis in Uruguay
Authors: Alejandra Rey, Gabriela Rutz, Pacheco Edilberto, Gueçaimburú Rosario, María Lόpez Varela
Journal: European Respiratory Journal (2011)

There is insufficient knowledge on Cystic Fibrosis (CF) in many countries; patients are often treated inadequately. Life expectancy is consequently far below than could be attainable with adequate treatment. Therefore it is very important to train Centers to concentrate clinical experience on diagnosis and management of disease. In June 2010, the Uruguayan Parliament created the First National Reference Center for Diagnosis and Treatment of Patients with Cystic Fibrosis.   Objective:  To describe genotypic and phenotypic characteristics of patients at the time of first encounter at a CF Center for adults in Uruguay.   Methods:  CF patients over 14 years were evaluated with medical history, spirometry and sputum studies.   Results:  41 adults entered the center. Mean age was 23.9±8.4, mean age at diagnosis was 14.2±11.3 years. Colonized patients showed differences with noncolonized in: mean age (25.8 vs. 22.9, p 0.01), mean age at diagnosis (13±11 vs. 14±11, p 0.01), lung function: FVC (84% vs. 95%, p 0.01); FEV1 (68% vs. 88%, p 0.00) and history of hemoptysis. Genetic mapping for 30 patients indicated that: 30% had two CFTR mutations, 36% a single mutated allele detected and in 33% no mutations detected. The most common mutation was ΔF508. The remaining mutations were substantially heterogeneous. In all, we had 3 alleles mutation class 1, 13 class 2, 3 class 4, and 1 class 5.   Conclusions:   Colonized patients were diagnosed earlier in life, showed significant deterioration in spirometric measures and more frequent hemoptysis.  Our population had different types of CFTR mutations.  The mutation ΔF508DEL was the most common and present in 16%.  33% of CFTR mutations were not detected.
Abstract 10616: Mir-128 Targets E2f3 to Regulate Cardiomyocyte Cell Cycle Re-entry
Authors: Wei Huang, Yuliang Feng, Jialiang Liang, Wenfeng Cai, Kai Kang, Zhili Wen, Yuhua Wang, Dehua Chang, Guo-Chang Fan, Ronald W Millard, Meifeng Xu, Muhammad Ashraf, Yigang Wang
Journal: Circulation (2013)
Abstract 18727: Atrial Fibrillation is an Independent Predictor of Higher Coronary Atherosclerosis Burden
Authors: Sayf Khaleel Bala, Sama Alchalabi, Belqis Elferjani, Kongkiat Chaikriangkrai, Kassi Mahwash, Faisal Nabi, Su Min Chang
Journal: Circulation (2013)

Introduction: Atrial fibrillation (AFIB) and coronary artery disease (CAD) share common risk factors. It’s unknown if AFIB is an independent predictor of subclinical CAD. Objectives: Evaluate the association of AFIB with subclinical CAD as determined by coronary artery calcium scoring (CACs) Method: 430 consecutive AFIB patients without angina, known CAD, systolic heart failure, valvular heart disease and cardiomyopathy underwent CACs with Multi-detectors CT. Results were compared to 1) Age, gender and race predicted CACs using MESA (Multi-ethnic study of atherosclerosis) calculator 2) Age and gender matched cohort of 430 patients presented with chest pain and suspected CAD. Results: The mean age of the population was 63±10, 65% male and 16% diabetics, 63% hypertensive. Prevalence of CAD (CACs &gt; 0) was 76 % of patients with AFIB and significantly higher than predicted by MESA calculator (Figure).Among AFIB patients, 64% had higher CACs than MESA predicted CACs at 50 th percentile (P &lt;0.001) while 13 % had higher CACs than MESA predicted CACs at 90 th percentile (P =0.05). In 320 AFIB patients with CACS &gt; 0, estimated arterial age was higher than the actual biological age. (68.8± 12 Vs.64.1±8.9, mean difference of 4.7 ±13 years, median of 5. P &lt; 0.001). Compared to the chest pain cohort, prevalence of CAD (CACs &gt; 0) and severe CACS (&gt;400) in patients with AFIB was significantly higher (Figure).Multivariable -analysis adjusted to Framingham 10 years risk showed that the presence of AFIB is independently associated with the presence of CACs (OR 1.81; 95% CI 1.34-2.44; P&lt;0.001) and severe calcification (OR 2.32; 95%CI 1.43-3.74; p0.001) Conclusion: Presence of AFIB without other known cardiac disease appears to have higher prevalence and more advanced CAD when compared to both epidemiological data and symptomatic patients with suspected CAD.
High-Grade Neuroendocrine Carcinoma with Bronchial Intraepithelial Tumor Spread
Authors: Hideaki Kojima, Reiko Watanabe, Mitsuhiro Isaka, Reiko Shimizu, Hiroyuki Kayata, Naoko Miyata, Tomohiro Maniwa, Shoji Takahashi, Ichiro Ito, Toru Kameya, Kazuhito Funai, Yasuhisa Ohde, Takashi Nakajima
Journal: Journal of Thoracic Oncology (2015)
ATP11C targets basolateral bile salt transporter proteins in mouse central hepatocytes
Authors: Dirk R. de Waart, Jyoti Naik, Karina S. Utsunomiya, Suzanne Duijst, Kam Ho‐Mok, A. Ruth Bolier, Johan Hiralall, Laura N. Bull, Piter J. Bosma, Ronald P.J. Oude Elferink, Coen C. Paulusma
Journal: Hepatology (2016)

ATP11C is a homolog of ATP8B1, both of which catalyze the transport of phospholipids in biological membranes. Mutations in ATP8B1 cause progressive familial intrahepatic cholestasis type1 in humans, which is characterized by a canalicular cholestasis. Mice deficient in ATP11C are characterized by a conjugated hyperbilirubinemia and an unconjugated hypercholanemia. Here, we have studied the hypothesis that ATP11C deficiency interferes with basolateral uptake of unconjugated bile salts, a process mediated by organic anion-transporting polypeptide (OATP) 1B2. ATP11C localized to the basolateral membrane of central hepatocytes in the liver lobule of control mice. In ATP11C-deficient mice, plasma total bilirubin levels were 6-fold increased, compared to control, of which ∼65% was conjugated and ∼35% unconjugated. Plasma total bile salts were 10-fold increased and were mostly present as unconjugated species. Functional studies in ATP11C-deficient mice indicated that hepatic uptake of unconjugated bile salts was strongly impaired whereas uptake of conjugated bile salts was unaffected. Western blotting and immunofluorescence analysis demonstrated near absence of basolateral bile salt uptake transporters OATP1B2, OATP1A1, OATP1A4, and Na(+) -taurocholate-cotransporting polypeptide only in central hepatocytes of ATP11C-deficient liver. In vivo application of the proteasome inhibitor, bortezomib, partially restored expression of these proteins, but not their localization. Furthermore, we observed post-translational down-regulation of ATP11C protein in livers from cholestatic mice, which coincided with reduced OATP1B2 levels.ATP11C is essential for basolateral membrane localization of multiple bile salt transport proteins in central hepatocytes and may act as a gatekeeper to prevent hepatic bile salt overload. Conjugated hyperbilirubinemia and unconjugated hypercholanemia and loss of OATP expression in ATP11C-deficient liver strongly resemble the characteristics of Rotor syndrome, suggesting that mutations in ATP11C can predispose to Rotor syndrome. (Hepatology 2016;64:161-174).
Cytologic Correlates of Chemical Carcinogens in Respiratory Tracts of Dogs2
Authors: Kimito Matsumura, Arthur H. Cohen, Edwin C. Shors, John R. Benfield
Journal: JNCI Journal of the National Cancer Institute (1978)

Journal Article Cytologic Correlates of Chemical Carcinogens in Respiratory Tracts of Dogs Get access Kimito Matsumura, M.D., Kimito Matsumura, M.D. 3Department of Surgery, Harbor General Hospital Campus, University of California at Los Angeles School of Medicine. Torrance, Calif. 90502. 4Present address: Chiba University School of Medicine, Institute of Pulmonary Cancer Research, Chiba, Japan. Search for other works by this author on: Oxford Academic PubMed Google Scholar Arthur H. Cohen, M.D., Arthur H. Cohen, M.D. 5Department of Pathology, Harbor General Hospital Campus of the University of California at Los Angeles School of Medicine. Search for other works by this author on: Oxford Academic PubMed Google Scholar Edwin C. Shors, M.S., Edwin C. Shors, M.S. 3Department of Surgery, Harbor General Hospital Campus, University of California at Los Angeles School of Medicine. Torrance, Calif. 90502. Search for other works by this author on: Oxford Academic PubMed Google Scholar John R. Benfield, M.D. John R. Benfield, M.D. 6 3Department of Surgery, Harbor General Hospital Campus, University of California at Los Angeles School of Medicine. Torrance, Calif. 90502. 4Present address: Chiba University School of Medicine, Institute of Pulmonary Cancer Research, Chiba, Japan. 6Address reprint requests to Dr. Benfield. City of Hope National Medical Center, Duarte. Calif. 91010. Search for other works by this author on: Oxford Academic PubMed Google Scholar JNCI: Journal of the National Cancer Institute, Volume 61, Issue 5, November 1978, Pages 1209–1214, https://doi.org/10.1093/jnci/61.5.1209 Published: 01 November 1978 Article history Received: 26 July 1977 Revision received: 14 March 1978 Accepted: 26 May 1978 Published: 01 November 1978
Increasing evidence that Young's syndrome is associated with mercury
Authors: A. Dally, B. Hendry
Journal: BMJ (1996)

students and not therefore classed.This is an exceptionally skewed population and not appropriate as a basis for drawing general conclusions.The authors' selective quotation from the world literature is unfortunate.They could have quoted papers from countries where there has been no decline in semen quality.Their reference to Carlsen et als paper is also selective.2Most serious reanalyses of the data in that paper suggest that no decline has occurred in the past 30 years.What is in dispute is why figures in the 20 years before that seem to have been higher.The factors that influence sperm quality are numerous, and the variability of specimens is considerable.Unless authors base their results on large studies and control for the many confounding variables then their contribution to our understanding of the problem is likely to be modest at best and in some cases misleading.
Sudden Onset of Generalized Pustules in a Newborn
Authors: Ge Qian, Jiang Ji, Jianbo Wu
Journal: JAMA (2016)

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 Podcasts Clinical Reviews Editors' Summary Medical News Author Interviews More 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) 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
RAD001 and zoledronic acid in renal cell carcinoma patients with bone metastases (RAZOR): A randomized phase II trial.
Authors: Reuben James Broom, Vicky Hinder, Katrina Sharples, Janie Proctor, Steven Duffey, Stephanie Pollard, Peter C.C. Fong, Garry Forgeson, Dean Laurence Harris, Michael B. Jameson, Anne O'Donnell, Richard T. North, Sanjeev Deva, Fritha J. Hanning, Andrew Grey, Michael P. N. Findlay
Journal: Journal of Clinical Oncology (2012)

e15054 Background: Bone metastases (BM) from renal cell carcinoma (RCC) are common, cause major morbidity and have been identified as an adverse prognostic feature. Previous trials have not assessed the effects of modern therapies on BM from RCC. Randomized data has demonstrated that zoledronic acid (ZOL) reduces skeletal-related-events (SRE’s) in RCC patients (pts). Bone turnover markers can identify pts at risk of SREs among those receiving ZOL. We sought to evaluate the effect on BM of RAD001 (everolimus) alone compared to RAD001 plus ZOL in the first-line setting. Methods: 30 treatment naïve pts with RCC and ≥ 1 BM were randomized 1:1 to RAD001 10mg daily (Arm A) vs. RAD001 + ZOL 4mg IV 4-weekly (dose adjusted for creatinine clearance [CrCl], Arm B). Key eligibility criteria were ECOG PS ≤ 2, no bisphosphonates or radiotherapy within 4 weeks and CrCl &gt;35ml/min. Bone-specific assessments were performed at baseline, weeks 1,4,8 and12. Treatment was continued on the allocated arm until progression (RECIST 1.1). The primary objective was to assess the difference in bone turnover markers over the first 12-weeks. The primary endpoint was urine N-telopeptide (uNTX) levels with plasma C-telopeptide (CTX) being secondary. Secondary objectives include comparison of quality of life (QoL) and pain (FACT-BP, BPI scores), SREs, safety and efficacy (PFS, RR). Results: Heng prognostic group was poor, intermediate and favorable-risk in; 40.0%, 46.7%, 13.3% respectively in Arm A and 20.0%, 46.7%, 33.3% (Arm B). 53.3% (Arm A) and 60.0% (Arm B) of pts had prior nephrectomy. Over the first 12 weeks, the reduction in mean CTX on Arm B relative to Arm A was 77% (95% CI (68%, 83%); p&lt;0.0001). Median PFS was 7.5mo (95% CI 3.5, 14.7) in Arm B and 5.5mo (95% CI 3.2, 7.2) in Arm A (p=0.11). Data on uNTX, QoL and SREs will be presented. At data cut off, 8 pts in Arm B had stopped ZOL before progression with either a drop in CrCl or hypocalcaemia. No cases of osteonecrosis of the jaw were seen. Conclusions: The addition of ZOL to RAD001 significantly reduced the bone resorption marker CTX in this treatment-naïve RCC population of pts with the adverse prognostic feature of BM.
Protection Against Induced Cirrhosis and Hepatocellular Carcinoma in Rats by Chloramphenicol2
Authors: Ramon Puron, Harlan I. Firminger
Journal: JNCI Journal of the National Cancer Institute (1965)

Two percent chloramphenicol administered simultaneously with 0.05 percent N-2-fluorenyidiacetamide in a semisynthetic diet effectively prevented the development of cirrhosis and hepatocellular carcinoma in 21 of 22 adult male Wistar rats. Hyperplastic nodules, a forerunner of carcinoma, were in the liver of only 3 animals. In a group of 12 rats given the same carcinogen-containing diet without chloramphenicol, by a modified pair-feeding regimen, all developed cirrhosis and 9 had hepatocellular carcinoma within 46 weeks after the start. Of the 3 rats not bearing hepatic tumors, 2 had hyperplastic nodules with varying degrees of atypicality. It is suggested that the chloramphenicol, as an inhibitor of protein synthesis prevented blocked protein-binding of carcinogen in the hepatic cells and thereby prevented the development of cirrhosis and carcinoma.
Counter-regulation of rejection activity against human liver grafts by donor PD-L1 and recipient PD-1 interaction
Authors: Xiao-Lei Shi, Shanta Mancham, Bettina E. Hansen, Robert J. de Knegt, Jeroen de Jonge, Luc J.W. van der Laan, Fernando Rivadeneira, Herold J. Metselaar, Jaap Kwekkeboom
Journal: Journal of Hepatology (2016)
Influenza virus damages the alveolar barrier by disrupting epithelial cell tight junctions
Authors: Kirsty R. Short, Jennifer Kasper, Stijn van der Aa, Arno C. Andeweg, Fatiha Zaaraoui-Boutahar, Marco Goeijenbier, Mathilde Richard, Susanne Herold, Christin Becker, Dana P. Scott, Ronald W.A.L. Limpens, Abraham J. Koster, Montserrat Bárcena, Ron A.M. Fouchier, Charles James Kirkpatrick, Thijs Kuiken
Journal: European Respiratory Journal (2016)

A major cause of respiratory failure during influenza A virus (IAV) infection is damage to the epithelial–endothelial barrier of the pulmonary alveolus. Damage to this barrier results in flooding of the alveolar lumen with proteinaceous oedema fluid, erythrocytes and inflammatory cells. To date, the exact roles of pulmonary epithelial and endothelial cells in this process remain unclear. Here, we used an in vitro co-culture model to understand how IAV damages the pulmonary epithelial–endothelial barrier. Human epithelial cells were seeded on the upper half of a transwell membrane while human endothelial cells were seeded on the lower half. These cells were then grown in co-culture and IAV was added to the upper chamber. We showed that the addition of IAV (H1N1 and H5N1 subtypes) resulted in significant barrier damage. Interestingly, we found that, while endothelial cells mounted a pro-inflammatory/pro-coagulant response to a viral infection in the adjacent epithelial cells, damage to the alveolar epithelial–endothelial barrier occurred independently of endothelial cells. Rather, barrier damage was associated with disruption of tight junctions amongst epithelial cells, and specifically with loss of tight junction protein claudin-4. Taken together, these data suggest that maintaining epithelial cell integrity is key in reducing pulmonary oedema during IAV infection.
Genomic Characterization of Early Stages of Ovarian Cancer with Emphasis in Low-Grade Endometroid and Low-Grade Serous Histologies. a Study By Spanish Group for Ovarian Cancer Research (Geico)
Authors: I. Romero, J.A. Lopez Guerrero, J. Palacios, B. Ojeda, C. Illueca, A. Gutierrez Pecharromán, S. Blanch, E. Cristóbal, M.Z. Garcia Casado, B. Vieites, I. Ruiz Díaz, F.J. Vera Sempere, F. Pastor, E. Andrada, M. Culubret, D. Hardisson, E. Calvo, C. Churruca, A. Santaballa, A.M. Poveda
Journal: Annals of Oncology (2014)
P71 Antiviral treatment of recurrent hepatitis C after liver transplantation—the Newcastle experience
Authors: S McPherson, M Hudson, D Sheridan, C Miller, K Baxter, M Bassendine
Journal: Gut (2010)

Introduction  Recurrence of hepatitis C (HCV) post liver transplantation is universal and may follow a rapidly progressive course, which results in poorer long-term graft survival rates compared with other liver diseases. Sustained virological response (SVR) after anti-viral treatment has recently been shown to significantly improve liver histology and long-term survival1.  Aim  To describe our experience of the treatment of recurrent HCV post liver transplantation.  Method  Retrospective case-note review of all patients transplanted for HCV.  Results  41 patients were transplanted in Newcastle for chronic HCV (10 had HCC) between 1993 and 2008. Up to 2002 our 5 year survival for patients with HCV was 45%. In order to try and improve this antiviral therapy was offered to patients from 2002. 15 patients (median age 50, range 38–68; 11 (73%) male) received individualised treatment with pegylated interferon (PEG-IFN) +/- ribavirin (RBV). All had liver biopsy showing recurrent HCV, 3 had cirrhosis and 1 had cholestatic hepatitis. 10 (66%) patients were infected with HCV genotype 1 (G1) and 5 with genotype 3 (G3). 4 patients (27%) achieved SVR following treatment (4 G3 and 1 G1) and 1 patient is currently on treatment and was HCV RNA negative at 12 weeks (G3). All patients who achieved SVR had =48 weeks treatment with PEG-IFN+RBV and had mild hepatic fibrosis. Of the four patients who had an SVR, 2 were taking ciclosporin and 2 tacrolimus. Adverse events were common and led to cessation of therapy in 6 patients (3 pancytopaenia, 1 refractory anaemia, 1 myocardial infarction, 1 hepatic decompensation). All cirrhotic patients stopped treatment due to adverse events. Severe anaemia was very common and 5 patients were treated with erythropoietin (2 had SVR).  Conclusion  Treatment of recurrent HCV post-liver transplantation can be successful in selected patients, particularly patients with HCV G3 with mild hepatic fibrosis. This experience has led us to discuss anti-viral therapy at 6 months post liver transplant.
Abstract P295: Exercise Capacity and Rate of Progression to Chronic Kidney Disease
Authors: Peter Kokkinos, Apostolos Tsimploulis, Charles Faselis, Jonathan Myers, Jiajia Zhang, Xuemei Sui, Steven Blair, Raya Kheirbek, Puneet Narayan, Carlos Palant
Journal: Circulation (2014)

Introduction: Increased exercise capacity is associated with better health outcomes. It is not known if increased fitness can attenuate the progression to chronic kidney disease (CKD). Hypothesis: We assessed the hypothesis that increased exercise capacity is associated inversely with the rate of progression to CKD. Methods: A routine exercise stress was performed on 6,452 veterans (mean age: 58±12) with normal kidney function at VA Medical Centers in Washington DC. We used Cox proportional hazards model with spline function of MET to define the MET level associated with no increase in rate of progression to CKD (hazard ratio (HR)=1.0). We used this MET level to guide the formation of the following four fitness categories based on intervals of 2 METs achieved above and below this threshold: Least-Fit (&lt;5.5 METs; n=1,392); Low-Fit (5.5-7.5 METs; n=2,270); Moderate-Fit (7.6-9.5 METs; n=2,192) and High-Fit (&gt;9.5 METs; n=714). We then performed Cox proportional hazards analysis adjusted for age, BMI, cardiac risk factors, sleep apnea, alcohol dependence and medications. We used the Least-fit category as the reference group. Results: The MET threshold for the entire cohort was defined at 7.5 METs. During the follow-up period (median 8.8 years; 50,371 person-years of follow-up), 925 individuals developed CKD based on an estimated glomerular filtration rate &lt;60 ml/min/1.73m 2 . Cox proportional hazards analysis revealed that exercise capacity was inversely associated with the rate of progression to CKD. More specifically, the rate of progression was lower by 25% (HR=0.75; CI: 0.64-0.87; p&lt;0.001) for Low-Fit individuals, 40% (HR=0.60; CI: 0.48-0.73; p&lt;0.001) for the Moderate-Fit and 68% (HR=0.42; CI: 0.28-0.64; p&lt;0.001) for High-Fit individuals. Conclusions: Exercise capacity is inversely associated with the rate of progression to CKD.
Phase II trial of carboplatin plus cisplatin in recurrent and advanced squamous cell carcinoma of the head and neck.
Authors: I W Dimery, B J Brooks, R Winn, T Martin, M Shirinian, W K Hong
Journal: Journal of Clinical Oncology (1991)

Cisplatin is one of the most active chemotherapeutic agents for the treatment of squamous carcinoma of the head and neck; however, neurotoxicity and nephrotoxicity are dose-limiting. The analog, carboplatin, is a promising new agent with similar activity but a different spectrum of toxicity. To evaluate if a therapeutic advantage could be achieved with acceptable toxicity, a combination of carboplatin 350 mg/m2 and cisplatin 50 mg/m2 were administered every 28 days to patients with recurrent or metastatic disease who had received no prior chemotherapy. Of 24 patients enrolled in this study, 21 were assessable for response and toxicity. Five partial responses were observed (24%; 95% confidence interval [Cl], 4.9% to 38.6%). No complete response occurred. Two of these patients received definitive radiotherapy and achieved complete responses. The median survival of all patients was 24 weeks. Hematologic toxicity was dose-limiting necessitating a decrease in the starting dose of carboplatin to 300 mg/m2. Nonhematologic toxicity was infrequent and mild. Significant renal impairment occurred in only two patients. Although treatment with the combination of carboplatin and cisplatin is feasible, we found no therapeutic advantage in terms of an increased response or survival.