Search

Your search keyword '"Jones, K. J."' showing total 606 results
Start Over Author "Jones, K. J."
606 results on '"Jones, K. J."'

1. Mediators of gram-positive septic shock

Author

Jones, K. J.

Subjects
616.944071, Pharmacy
Abstract

Gram-positive bacteria are increasingly becoming more prevalent especially Staphylococcus epidermidis in association with indwelling devices. Lipopolysaccaride (LPS) is the key Gram-negative component involved in this process, but it is not clear which components of Gram-positive bacteria are responsible for progression of this often fatal disease. The aim of this thesis was to investigate the effect of bacterial components on the immune systems. Lipid S, a short chain form of lipoteichoic acid (LTA) found to be excreted from bacteria during growth in culture medium was examined along with other Gram-positive cell wall components: LTA, peptidoglycan (PG) and wall teichoic acids (WTA) and LPS from Gram-negative bacteria. Lipid S, LTA, PG and LPS but not WTA all stimulated murine macrophages and cell lines to produce significant amounts of NO, TNF-a, IL-6 and IL-1 and would induce fever and tissue damage seen in inflammatory diseases. Lipid S proved to be the most potent out of the Gram-positive samples tested. IgG antibodies in patients serum were found to bind to and cross react with lipid S and LTA. Anti-inflammatory antibiotics, platelet activating factor (PAF), PAF receptor antagonists and monoclonal antibodies (mAbs) directed to LTA, CD14 and toll-like receptors were utilised to modulate cytokine and NO production. In cell culture the anti-LTA and the anti-CD14 mAbs failed to markedly attenuate the production of NO, TNF-a, IL-6 or IL-1, the anti-TLR4 antibody did greatly inhibit the ability of LPS to stimulate cytokine production but not lipid S. The tetracyclines proved to be the most effective compounds, many were active at low concentrations and showed efficacy to inhibit both lipid S and LPS stimulated macrophages to produce NO.

Published
2003
Full Text
View/download PDF

2. Making Connections.

Author

JONES, K. J.

Subjects
AUTOMATIC automobile transmissions, FUEL pumps, PRESSURE sensors, TELECOMMUNICATION cables
Abstract

This article discusses the installation of a Holley EFI Terminator X Max system in a project car called the T-top Coupe. The Terminator X Max is an entry-level player in Holley's advanced-EFI lineup and is compatible with various engine types. The article provides a step-by-step guide to installing the system, including connecting the wiring harnesses and sensors. The Terminator X Max system offers self-learning fuel mapping and can control features such as nitrous oxide and turbocharger boost. The article also mentions the availability of additional tuning options and the use of a handheld LCD touchscreen display for monitoring and tuning the system. [Extracted from the article]

Published
2024

3. RUST BUSTED.

Author

JONES, K. J.

Subjects
AUTOMOBILE transmission fluids
Abstract

• In our next report, we will continue detailing our forensic discoveries as Scott Hes-selgrave at Air Flow Research and Greg Dawley at Vortech inspect the Renegade 220cc cylinder heads and Paxton Novi supercharger. Progress on our 1986 Mustang T-top coupe remains on course and at a pace that should have us firing the engine and taking a maiden voyage soon. Since the reveal, KJ and Saul "The Surgeon" Gutierrez have revamped the coupe's fuel system, and during that process, started disassembling the Paxton Novi 2500-supercharged, 350ci Ford engine. [Extracted from the article]

Published
2024

5. THE BIG CLEAN OUT.

Author

JONES, K. J.

Subjects
COBRA automobile, FUEL pumps, PUMPING machinery, FUEL tanks
Abstract

Source Aeromotive; 913.647.7300; aeromotiveinc.com HOT ROD 75TH ANNIVERSARY/2023/DECEMBER We are working on the reintroduction of our unique 1986 Ford Mustang T-top coupe, a former project car that was put into storage for more than 10 years. Here's the latest suggestion from Big Steve, Aeromotive's Eliminator Fox Body Stealth fuel tank. 04 An EFI bypass regulator, 10-Micron post-pump filter, gauge, and fuel rails for '86-'95.5 model year 5.0-liter V8-powered Mustangs are the finishing pieces of our project's fuel system. [Extracted from the article]

Published
2023

6. Assessing the Relationship of Patient Reported Outcome Measures With Functional Status in Dysferlinopathy: A Rasch Analysis Approach

Author

Mayhew, A. G., James, M. K., Moore, U., Sutherland, H., Jacobs, M., Feng, J., Lowes, L. P., Alfano, L. N., Muni Lofra, R., Rufibach, L. E., Rose, K., Duong, T., Bello, L., Pedrosa-Hernandez, I., Holsten, S., Sakamoto, C., Canal, A., Sanchez-Aguilera Praxedes, N., Thiele, S., Siener, C., Vandevelde, B., Dewolf, B., Maron, E., Gordish-Dressman, H., Hilsden, H., Guglieri, M., Hogrel, J. -Y., Blamire, A. M., Carlier, P. G., Spuler, S., Day, J. W., Jones, K. J., Bharucha-Goebel, D. X., Salort-Campana, E., Pestronk, A., Walter, M. C., Paradas, C., Stojkovic, T., Mori-Yoshimura, M., Bravver, E., Diaz-Manera, J., Pegoraro, E., Mendell, J. R., Jain COS Consortium, Straub, V., Jain Foundation, John Walton Centre Muscular Dystrophy Research Centre, and MRC Centre Neuromuscular Biobank (UK)

Subjects
PROMs, Neurology, quality of life, Neurology (clinical), clinical outcome assessments, dysferlinopathy, limb girdle muscular dystrophy, Function and Dysfunction of the Nervous System
Abstract

Dysferlinopathy is a muscular dystrophy with a highly variable functional disease progression in which the relationship of function to some patient reported outcome measures (PROMs) has not been previously reported. This analysis aims to identify the suitability of PROMs and their association with motor performance.Two-hundred and four patients with dysferlinopathy were identified in the Jain Foundation's Clinical Outcome Study in Dysferlinopathy from 14 sites in 8 countries. All patients completed the following PROMs: Individualized Neuromuscular Quality of Life Questionnaire (INQoL), International Physical Activity Questionnaire (IPAQ), and activity limitations for patients with upper and/or lower limb impairments (ACTIVLIMs). In addition, nonambulant patients completed the Egen Klassifikation Scale (EK). Assessments were conducted annually at baseline, years 1, 2, 3, and 4. Data were also collected on the North Star Assessment for Limb Girdle Type Muscular Dystrophies (NSAD) and Performance of Upper Limb (PUL) at these time points from year 2. Data were analyzed using descriptive statistics and Rasch analysis was conducted on ACTIVLIM, EK, INQoL. For associations, graphs (NSAD with ACTIVLIM, IPAQ and INQoL and EK with PUL) were generated from generalized estimating equations (GEE). The ACTIVLIM appeared robust psychometrically and was strongly associated with the NSAD total score (Pseudo R 2 0.68). The INQoL performed less well and was poorly associated with the NSAD total score (Pseudo R 2 0.18). EK scores were strongly associated with PUL (Pseudo R 2 0.69). IPAQ was poorly associated with NSAD scores (Pseudo R 2 0.09). This study showed that several of the chosen PROMs demonstrated change over time and a good association with functional outcomes. An alternative quality of life measure and method of collecting data on physical activity may need to be selected for assessing dysferlinopathy., The estimated US $4 million needed to fund this study was provided by the Jain Foundation (www.jainfoundation.org). The John Walton Muscular Dystrophy Research Centre is part of the MRC Centre for Neuromuscular Diseases (Grant Number MR/K000608/1).

Published
2022

9. Reply

Author

Jones, K. J., primary, Bevilacqua, E., additional, Grati, F. R., additional, Schmid, M., additional, and Jani, J. C., additional

Published
2021
Full Text
View/download PDF

21. Biallelic and monoallelic variants in PLXNA1 cause a syndromic disorder with neurodevelopmental and oculo-cerebral anomalies

Author

Dworschak, G. C., Punetha, J., Kalanithy, J. C., Mingardo, E., Erdem, H. B., Akdemir, Z. C., Karaca, E., Mitani, T., Marafi, D., Fatih, J. M., Jhangiani, S. N., Hunter, J. V., Dakal, T., Dhabhai, B., Dabbagh, O., Alsaif, H. S., Alkuraya, F. S., Maroofian, R., Houlden, H., Efthymiou, S., Dominik, N., Salpietro, V., Sultan, T., Haider, S., Bibi, F., Thiele, H., Hoefele, J., Riedhammer, K. M., Wagner, M., Guella, I., Demos, M., Keren, B., Buratti, J., Charles, P., Nava, C., Valkanas, E., Waddell, L. B., Jones, K. J., Oates, E. C., Cooper, S. T., MacArthur, D., Schnur, R. E., Hoganson, G. E., Burton, J. E., McEntagart, M., Pehlivan, D., Posey, J. E., Lupski, J. R., Reutter, H., Dworschak, G. C., Punetha, J., Kalanithy, J. C., Mingardo, E., Erdem, H. B., Akdemir, Z. C., Karaca, E., Mitani, T., Marafi, D., Fatih, J. M., Jhangiani, S. N., Hunter, J. V., Dakal, T., Dhabhai, B., Dabbagh, O., Alsaif, H. S., Alkuraya, F. S., Maroofian, R., Houlden, H., Efthymiou, S., Dominik, N., Salpietro, V., Sultan, T., Haider, S., Bibi, F., Thiele, H., Hoefele, J., Riedhammer, K. M., Wagner, M., Guella, I., Demos, M., Keren, B., Buratti, J., Charles, P., Nava, C., Valkanas, E., Waddell, L. B., Jones, K. J., Oates, E. C., Cooper, S. T., MacArthur, D., Schnur, R. E., Hoganson, G. E., Burton, J. E., McEntagart, M., Pehlivan, D., Posey, J. E., Lupski, J. R., and Reutter, H.

Published
2020

25. Performance of a targeted cell-free DNA prenatal test for 22q11.2 deletion in a large clinical cohort.

Author

Bevilacqua, E., Jani, J. C., Chaoui, R., Suk, E.‐K. A., Palma‐Dias, R., Ko, T.‐M., Warsof, S., Stokowski, R., Jones, K. J., Grati, F. R., Schmid, M., Suk, E-K A, Palma-Dias, R, Ko, T-M, and Jani, J

Subjects
PRENATAL genetic testing, CELL-free DNA, OBSTETRICS, SENSITIVITY & specificity (Statistics), FETAL abnormalities, INTROGRESSION (Genetics), ULTRASONIC imaging
Abstract

Objective: 22q11.2 deletion is more common than trisomies 18 and 13 combined, yet no routine approach to prenatal screening for this microdeletion has been established. This study evaluated the clinical sensitivity and specificity of a targeted cell-free DNA (cfDNA) test to screen for fetal 22q11.2 deletion in a large cohort, using blinded analysis of prospectively enrolled pregnancies and stored clinical samples.Methods: In order to ensure that the analysis included a meaningful number of cases with fetal 22q11.2 deletion, maternal plasma samples were obtained by prospective, multicenter enrolment of pregnancies with a fetal cardiac abnormality and from stored clinical samples from a research sample bank. Fetal genetic status, as evaluated by microarray analysis, karyotyping with fluorescence in-situ hybridization or a comparable test, was available for all cases. Samples were processed as described previously for the Harmony prenatal test, with the addition of DANSR (Digital Analysis of Selected Regions) assays targeting the 3.0-Mb region of 22q11.2 associated with 22q11.2 deletion syndrome. Operators were blinded to fetal genetic status. Sensitivity and specificity of the cfDNA test for 22q11.2 deletion were calculated based on concordance between the cfDNA result and fetal genotype.Results: The final study group consisted of 735 clinical samples, including 358 from prospectively enrolled pregnancies and 377 stored clinical samples. Of 46 maternal plasma samples from pregnancies with a 22q11.2 deletion, ranging in size from 1.25 to 3.25 Mb, 32 had a cfDNA result indicating a high probability of 22q11.2 deletion (sensitivity, 69.6% (95% CI, 55.2-80.9%)). All 689 maternal plasma samples without a 22q11.2 deletion were classified correctly by the cfDNA test as having no evidence of a 22q11.2 deletion (specificity, 100% (95% CI, 99.5-100%)).Conclusions: The results of this large-scale prospective clinical evaluation of the sensitivity and specificity of a targeted cfDNA test for fetal 22q11.2 deletion demonstrate that this test can detect the common and smaller, nested 22q11.2 deletions with a low (0-0.5%) false-positive rate. Although the positive predictive value (PPV) observed in this study population was 100%, the expected PPV in the general pregnant population is estimated to be 12.2% at 99.5% specificity and 41.1% at 99.9% specificity. The use of this cfDNA test to screen for 22q11.2 deletion could enhance identification of pregnancies at risk for 22q11.2 deletion syndrome without significantly increasing the likelihood of maternal anxiety and unnecessary invasive procedures related to a false-positive result. © 2021 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Published
2021
Full Text
View/download PDF

26. Assessment of disease progression in dysferlinopathy: a 1-year cohort study

Author

Moore, U., Jacobs, Marni, James, Meredith K, Mayhew, A. G., Fernandez-Torron, Roberto, Feng, J., Cnaan, A., Eagle, M., Bettinson, K., Rufibach, L. E., Lofra, R. M., Blamire, A. M., Carlier, P. G., Mittal, P., Lowes, L. P., Alfano, L., Rose, K., Duong, T., Berry, K. M., Montiel-Morillo, E., Pedrosa-Hernández, I., Holsten, S., Sanjak, M., Ashida, A., Sakamoto, C., Tateishi, T., Yajima, H., Canal, A., Ollivier, G., Decostre, V., Mendez, J. B., Praxedes, N. S. A., Thiele, S., Siener, C., Shierbecker, J., Florence, J. M., Vandevelde, B., Dewolf, B., Hutchence, M., Gee, R., Prügel, J., Maron, E., Hilsden, Heather, Lochmüller, H., Grieben, U., Spuler, Simone, Rocha, C. T., Day, J. W., Jones, K. J., Bharucha-Goebel, D. X., Salort-Campana, Emmanuelle, Harms, M., Pestronk, Alan, Krause, S., Schreiber-Katz, Olivia, Walter, M. C., Paradas, C., Hogrel, J. Y., Stojkovic, T., Takeda, S., Mori-Yoshimura, M., Bravver, Elena, Sparks, S., Diaz-Manera, Jordi., Bello, Luca, Semplicini, C., Pegoraro, E., Mendell, J. R., Bushby, Kate, Straub, V., Universitat Autònoma de Barcelona, and Jain Foundation

Subjects
0301 basic medicine, medicine.medical_specialty, Dysferlinopathy, business.industry, Wrist, medicine.disease, 3. Good health, Test (assessment), Clinical trial, Manual Muscle Testing, 03 medical and health sciences, 030104 developmental biology, 0302 clinical medicine, medicine.anatomical_structure, Cardiovascular and Metabolic Diseases, Ambulatory, Physical therapy, medicine, Clinical endpoint, Neurology (clinical), business, 030217 neurology & neurosurgery, Cohort study
Abstract

Jain COS Consortium., [Objective] To assess the ability of functional measures to detect disease progression in dysferlinopathy over 6 months and 1 year., [Methods] One hundred ninety-three patients with dysferlinopathy were recruited to the Jain Foundation's International Clinical Outcome Study for Dysferlinopathy. Baseline, 6-month, and 1-year assessments included adapted North Star Ambulatory Assessment (a-NSAA), Motor Function Measure (MFM-20), timed function tests, 6-minute walk test (6MWT), Brooke scale, Jebsen test, manual muscle testing, and hand-held dynamometry. Patients also completed the ACTIVLIM questionnaire. Change in each measure over 6 months and 1 year was calculated and compared between disease severity (ambulant [mild, moderate, or severe based on a-NSAA score] or nonambulant [unable to complete a 10-meter walk]) and clinical diagnosis., [Results] The functional a-NSAA test was the most sensitive to deterioration for ambulant patients overall. The a-NSAA score was the most sensitive test in the mild and moderate groups, while the 6MWT was most sensitive in the severe group. The 10-meter walk test was the only test showing significant change across all ambulant severity groups. In nonambulant patients, the MFM domain 3, wrist flexion strength, and pinch grip were most sensitive. Progression rates did not differ by clinical diagnosis. Power calculations determined that 46 moderately affected patients are required to determine clinical effectiveness for a hypothetical 1-year clinical trial based on the a-NSAA as a clinical endpoint., [Conclusion] Certain functional outcome measures can detect changes over 6 months and 1 year in dysferlinopathy and potentially be useful in monitoring progression in clinical trials., [ClinicalTrials.gov identifier] NCT01676077., The estimated US $4 million needed to fund this study is being provided by the Jain Foundation. The John Walton Centre Muscular Dystrophy Research Centre is part of the MRC Centre for Neuromuscular Diseases (grant MR/K000608/1).

Published
2019

27. Towards regulatory endorsement of drug development tools to promote the application of model-informed drug development in Duchenne muscular dystrophy

Author

Conrado, D. J., Larkindale, J., Berg, A., Hill, M., Burton, J., Abrams, K. R., Abresch, R. T., Bronson, A., Chapman, D., Crowther, M., Duong, T., Gordish-Dressman, H., Harnisch, L., Henricson, E., Kim, S., Mcdonald, C. M., Schmidt, S., Vong, C., Wang, X., Wong, B. L., Yong, F., Romero, K., Vishwanathan, V., Chidambaranathan, S., Douglas Biggar, W., Mcadam, L. C., Mah, J. K., Tulinius, M., Cnaan, A., Morgenroth, L. P., Leshner, R., Tesi-Rocha, C., Thangarajh, M., Kornberg, A., Ryan, M., Nevo, Y., Dubrovsky, A., Clemens, P. R., Abdel-Hamid, H., Connolly, A. M., Pestronk, A., Teasley, J., Bertorini, T. E., Webster, R., Kolski, H., Kuntz, N., Driscoll, S., Bodensteiner, J. B., Gorni, K., Lotze, T., Day, J. W., Karachunski, P., Henricson, E. K., Joyce, N. C., Campbell, C., Torricelli, R. E., Finkel, R. S., Flanigan, K. M., Goemans, N., Heydemann, P., Kaminska, A., Kirschner, J., Muntoni, F., Osorio, A. N., Schara, U., Sejersen, T., Shieh, P. B., Sweeney, H. L., Topaloglu, H., Vilchez, J. J., Voit, T., Wong, B., Alfano, L. N., Eagle, M., James, M. K., Lowes, L., Mayhew, A., Mazzone, E. S., Nelson, L., Rose, K. J., Abdel-Hamid, H. Z., Apkon, S. D., Barohn, R. J., Bertini, E., Bloetzer, C., Devaud, L. C., Butterfield, R. J., Chabrol, B., Chae, J. H., Jongno-Gu, D. R., Comi, G. P., Darras, B. T., Dastgir, J., Desguerre, I., Escobar, R. G., Finanger, E., Guglieri, M., Hughes, I., Iannaccone, S. T., Jones, K. J., Kudr, M., Mathews, K., Parsons, J., Pereon, Y., de Queiroz Campos Araujo, A. P., Renfroe, J. B., de Resende, M. B. D., Selby, K., Tennekoon, G., Vita, G., Apkon, S., Barohn, R., Belousova, E., Brandsema, J., Bruno, C., Burnette, W., Butterfield, R., Byrne, B., Carlo, J., Chandratre, S., Comi, G., Connolly, A., De Groot I, I., Deconinck, N., Dooley, J., Durigneux, J., Finkel, R., Frank, L. M., Harper, A., Hattori, A., Herguner, O., Iannaccone, S., Janas, J., Jong, Y. J., Komaki, H., Lee, W. T., Leung, E., Mah, J., Mercuri, E., Mcmillan, H., Mueller-Felber, W., de Munain A, L., Nakamura, A., Niks, E., Ogata, K., Pascual, S., Pegoraro, E., Renfroe, B., Sanka, R. B., Schallner, J., Sendra, I. I., Servais, L., Smith, E., Sparks, S., Victor, R., Wicklund, M., Wilichoswki, E., Carter, G. T., and Servais, LJP

Subjects
Orphan Drug Production, Duchenne muscular dystrophy, Pharmacy, Model-informed drug development, 030226 pharmacology & pharmacy, Models, Biological, Food and drug administration, 03 medical and health sciences, 0302 clinical medicine, Models, medicine, Humans, Regulatory science, Computer Simulation, Muscular Dystrophy, Drug development tools, Duchenne muscular dystrophy consortium (D-RSC), Rare diseases, Regulatory endorsement, Clinical Trials as Topic, Muscular Dystrophy, Duchenne, United States, United States Food and Drug Administration, Pharmacology, Protocol (science), business.industry, Duchenne, Biological, medicine.disease, Clinical trial, Risk analysis (engineering), Drug development, 030220 oncology & carcinogenesis, Aggregate data, Business
Abstract

Drug development for rare diseases is challenged by small populations and limited data. This makes development of clinical trial protocols difficult and contributes to the uncertainty around whether or not a potential therapy is efficacious. The use of data standards to aggregate data from multiple sources, and the use of such integrated databases to develop statistical models can inform protocol development and reduce the risks in developing new therapies. Achieving regulatory endorsement of such models through defined pathways at the US Food and Drug Administration and European Medicines Authority allows such tools to be used by the drug development community for defined contexts of use without further need for discussion of the underlying model(s). The Duchenne Regulatory Science Consortium (D-RSC) has brought together multiple stakeholders to develop a clinical trial simulation tool for Duchenne muscular dystrophy using such an approach. Here we describe the work of D-RSC as an example of how such an approach may be effective at reducing uncertainty in drug development for rare diseases, and thus bringing effective therapies to patients faster.

Published
2019

35. Systematic review of the introduction and evaluation of magnetic augmentation of the lower oesophageal sphincter for gastro‐oesophageal reflux disease.

Author

Kirkham, E. N., Main, B. G., Jones, K. J. B., Blazeby, J. M., and Blencowe, N. S.

Subjects
HEARTBURN, META-analysis, SPHINCTERS, PATIENT selection, TRAINING of surgeons
Abstract

Copyright of British Journal of Surgery is the property of Oxford University Press / USA and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Published
2020
Full Text
View/download PDF

36. Ataluren in patients with nonsense mutation Duchenne muscular dystrophy (ACT DMD): a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial

Author

McDonald, C. M., Campbell, C., Torricelli, R. E., Finkel, R. S., Flanigan, K. M., Goemans, N., Heydemann, P., Kaminska, A., Kirschner, J., Muntoni, F., Osorio, A. N., Schara, U., Sejersen, T., Shieh, P. B., Sweeney, H. L., Topaloglu, H., Tulinius, M., Vilchez, J. J., Voit, T., Wong, B., Elfring, G., Kroger, H., Luo, X., McIntosh, J., Ong, T., Riebling, P., Souza, M., Spiegel, R. J., Peltz, S. W., Mercuri, E., Alfano, L. N., Eagle, M., James, M. K., Lowes, L., Mayhew, A., Mazzone, E. S., Nelson, L., Rose, K. J., Abdel-Hamid, H. Z., Apkon, S. D., Barohn, R. J., Bertini, E., Bloetzer, C., de Vaud, L. C., Butterfield, R. J., Chabrol, B., Chae, J. -H., Jongno-gu, D. -R., Comi, G. P., Darras, B. T., Dastgir, J., Desguerre, I., Escobar, R. G., Finanger, E., Guglieri, M., Hughes, I., Iannaccone, S. T., Jones, K. J., Karachunski, P., Kudr, M., Lotze, T., Mah, J. K., Mathews, K., Nevo, Y., Parsons, J., Pereon, Y., de Queiroz Campos Araujo, A. P., Renfroe, J. B., de Resende, M. B. D., Ryan, M., Selby, K., Tennekoon, G., Vita, G., Mercuri E. (ORCID:0000-0002-9851-5365), McDonald, C. M., Campbell, C., Torricelli, R. E., Finkel, R. S., Flanigan, K. M., Goemans, N., Heydemann, P., Kaminska, A., Kirschner, J., Muntoni, F., Osorio, A. N., Schara, U., Sejersen, T., Shieh, P. B., Sweeney, H. L., Topaloglu, H., Tulinius, M., Vilchez, J. J., Voit, T., Wong, B., Elfring, G., Kroger, H., Luo, X., McIntosh, J., Ong, T., Riebling, P., Souza, M., Spiegel, R. J., Peltz, S. W., Mercuri, E., Alfano, L. N., Eagle, M., James, M. K., Lowes, L., Mayhew, A., Mazzone, E. S., Nelson, L., Rose, K. J., Abdel-Hamid, H. Z., Apkon, S. D., Barohn, R. J., Bertini, E., Bloetzer, C., de Vaud, L. C., Butterfield, R. J., Chabrol, B., Chae, J. -H., Jongno-gu, D. -R., Comi, G. P., Darras, B. T., Dastgir, J., Desguerre, I., Escobar, R. G., Finanger, E., Guglieri, M., Hughes, I., Iannaccone, S. T., Jones, K. J., Karachunski, P., Kudr, M., Lotze, T., Mah, J. K., Mathews, K., Nevo, Y., Parsons, J., Pereon, Y., de Queiroz Campos Araujo, A. P., Renfroe, J. B., de Resende, M. B. D., Ryan, M., Selby, K., Tennekoon, G., Vita, G., and Mercuri E. (ORCID:0000-0002-9851-5365)

Abstract

Background Duchenne muscular dystrophy (DMD) is a severe, progressive, and rare neuromuscular, X-linked recessive disease. Dystrophin deficiency is the underlying cause of disease; therefore, mutation-specific therapies aimed at restoring dystrophin protein production are being explored. We aimed to assess the efficacy and safety of ataluren in ambulatory boys with nonsense mutation DMD. Methods We did this multicentre, randomised, double-blind, placebo-controlled, phase 3 trial at 54 sites in 18 countries located in North America, Europe, the Asia-Pacific region, and Latin America. Boys aged 7–16 years with nonsense mutation DMD and a baseline 6-minute walk distance (6MWD) of 150 m or more and 80% or less of the predicted normal value for age and height were randomly assigned (1:1), via permuted block randomisation (block size of four) using an interactive voice-response or web-response system, to receive ataluren orally three times daily (40 mg/kg per day) or matching placebo. Randomisation was stratified by age (<9 years vs ≥9 years), duration of previous corticosteroid use (6 months to <12 months vs ≥12 months), and baseline 6MWD (<350 m vs ≥350 m). Patients, parents and caregivers, investigational site personnel, PTC Therapeutics employees, and all other study personnel were masked to group allocation until after database lock. The primary endpoint was change in 6MWD from baseline to week 48. We additionally did a prespecified subgroup analysis of the primary endpoint, based on baseline 6MWD, which is reflective of anticipated rates of disease progression over 1 year. The primary analysis was by intention to treat. This study is registered with ClinicalTrials.gov, number NCT01826487. Findings Between March 26, 2013, and Aug 26, 2014, we randomly assigned 230 patients to receive ataluren (n=115) or placebo (n=115); 228 patients comprised the intention-to-treat population. The least-squares mean change in 6MWD from baseline to week 48 was −47·7 m (SE 9·3

Published
2017

37. DRAG RACE.

Author

JONES, K. J.

Subjects
DRAG racing
Abstract

As he did in the trailer tow, Richard Coker stormed to the front of the pack in the drag-race segment, leading all qualifiers with a low e.t. of 10.79 seconds at a whopping 135.88 mph. A .127 reaction time helped Richard fend off Steven Lucero's hard-charging '06 Dodge Ram 2500 to take the win with an 11.26 e.t. at 132.21 mph over Steven's 10.91/127.03. "As he did in the trailer tow, Richard Coker stormed to the front of the pack in the drag-race segment, leading all qualifiers with a low e.t. of 10.79 seconds at a whopping 135.88 mph". [Extracted from the article]

Published
2019

38. TRAILER TOW.

Author

JONES, K. J.

Subjects
TOWING, TRUCK trailers, NITROUS oxide, TEAMS in the workplace
Abstract

This mission, in the opening act on the dragstrip at Bandimere Speedway in Morrison, Colorado, was to see how quickly competitors' trucks could drag a 20-foot trailer/Bobcat (11,500 total pounds) combination 660 feet. After suffering catastrophic damage during the Day Three dyno test, Matt and his team worked all night to replace two pistons and head gaskets on the truck's 7.3L Power Stroke engine and came back strong. Andrew McCane's '06 Dodge Ram 2500 finished the segment in the middle of the 10-truck field, pulling the setup in just outside the 11-second range. [Extracted from the article]

Published
2019

39. DYNO DAY.

Author

JONES, K. J.

Subjects
DIESEL trucks, NITROUS oxide, ENERGY consumption, BICYCLE racing
Abstract

After a night of thrashing in the diesel truck Triage Center at ATS, Matt and his team got the engine running and back in action for Day Four's segments at Bandimere Speedway in nearby Morrison, Colorado. Led by Richard Coker's '06 Chevrolet Silverado 2500HD and its 1,198.2 hp, seven trucks posted more than 1,000 hp on the chassis dyno. Prior to the start of the segment, the Mustang chassis dyno at ATS Diesel Performance was used to validate the unit's accuracy, using owner Clint Cannon's Ford F-250. [Extracted from the article]

Published
2019

40. SELECTION COMMITTEE.

Author

JONES, K. J.

Subjects
DODGE trucks, RAM truck
Abstract

The article offers brief information on various model of trucks by FCA US LLC exhibited at the 2019 Diesel Power Challenge including 2006 Dodge Ram 2500, 1997 Dodge Ram 3500 and 2007 Dodge Ram 3500.

Published
2019

41. COOLEST DIESEL RIDES OF SEMA 2018.

Author

JONES, K. J.

Subjects
TRADE shows, AUTOMOBILE customizing equipment, AUTOMOBILE equipment, AUTOMOBILE engine equipment
Abstract

The article focuses the different custom trucks and cars that were featured in the 2018 Specialty Equipment Market Associaiton (SEMA) Show in Las Vegas, Nevada. The event was attended by owners of the entries, designers and builders of the customized vehicles. It also mentions the different vehicle parts, display equipments and accessories that were presented in the show.

Published
2019

42. EXTRAVAGANCE.

Author

LEHENBAUER, JOHN and JONES, K. J.

Subjects
AUTOMOBILE exhibitions, DRAG racing
Abstract

The article offers a review on annual truckfest "Scheid Diesel Extravaganza" presenting "show 'n' shine" hosted by the Purdue Diesel Club; "drag race" by Outlaw Diesel Super Series ; and "Sled Pull" organised by Lucas Oil Champions Tour.

Published
2019

43. PICKUP SIX.

Author

JONES, K. J.

Subjects
AUTOMOTIVE transmission repair, FORD trucks, AUTOMOBILE parts
Abstract

The article offers information on the installation of the Allison 1000 six-speed automatic transmission in a 6.0Litre, 6.4Litre, or 7.3Litre Ford Power Stroke or 5.9Litre Cummins-motivated truck. Topics discussed include the differences between the Allison and Ford transmission, the use of Ford-to-Allison conversion hardware and wiring from Custom Automatic Conversions, and Ford pickup's transmission tunnel.

Published
2018

44. PICK 10.

Author

JONES, K. J.

Subjects
PICKUP trucks, TRUCKS, CUSTOMIZATION, DODGE trucks, TRUCK engines, TRUCK springs & suspensions
Abstract

The article offers information on several customized pickup Dodge trucks taking part in the 14the Diesel Power Challenge 2018 to be held in Denver, Colorado from held June 3 to June 7, 2018. Topics discussed include the '04½ Dodge Ram 2500 owned by Alex Goulet featuring 5.9L Cummins I-6 engine and Rancho shocks suspension, the '07 Dodge Ram 3500 owned by Cody Lambert featuring Fox Factory shocks, and the '05 Dodge Ram 2500 owned by Justin Andres featuring 6.2L Cummins I-6 engine.

Published
2018

46. WHAT A YEAR!

Author

JONES, K. J.

Subjects
DRAG racing, DRAG racers
Abstract

The article offers information on the drag racing event, 2017 Outlaw Diesel Super Series held in Julian, North Carolina.

Published
2018

47. POWER PRINCIPLES.

Author

JONES, K. J. and RIDDLE, TRENT

Subjects
FORD trucks, TRUCKS, CUSTOMIZATION, RACING trucks, FORD automobiles, CONSERVATION & restoration
Abstract

The article offers information related to customization of the Ford's 1995 OBS F-350 truck. Several changes made in the truck includes use of ARP's Ultra-Torque bolt lube and antiseize to get more uniform torque across the head studs; ATS's Aurora 3000 turbo kit; and Performance Injection Systems' 160cc fuel injectors.

Published
2017

48. SPEED AND SPORTSMANSHIP RULE THE DRAGSTRIP.

Author

JONES, K. J.

Subjects
AUTOMOBILE racing, DRAG racing
Abstract

The article presents information and photographs related to the drag racing competition held during the Diesel Power Challenge 2017.

Published
2017

49. LIGHTS, CAMERA, AND PLENTY OF DYNO ACTION.

Author

JONES, K. J.

Subjects
AUTOMOBILE racing, HORSEPOWER, TORQUE, AUTOMOTIVE fuel consumption, AUTOMOBILE engines
Abstract

The article discusses the segment of demonstrating horsepower and torque on the chassis dyno of the racing trucks during the Diesel Power Challenge 2017 event. It mentions the fuel consumption test held during the event organized by ATS Diesel Performance. It also mentions concerns related to superheated engine during the test.

Published
2017

50. LEVI KRECH.

Author

JONES, K. J.

Subjects
TRUCKS, CUSTOMIZATION, RAM truck, TRUCK tires, TRUCK springs & suspensions
Abstract

The article focuses on the customization made in the 2006 Dodge Ram 2500 truck by its owner Levi Krech. It mentions addition of the 305/40 R22 Nitto NT420S/22x12 Fuel Lethal tires and wheels. It also mentions addition of Bilstein shocks and CalTracs traction bars for suspension and 6.4L Cummins I-6 engine.

Published
2017

Catalog

Books, media, physical & digital resources
See catalog results