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https://www.readbyqxmd.com/read/28933017/present-uses-future-applications-and-technical-underpinnings-of-electrical-impedance-myography
#1
REVIEW
Benjamin Sanchez, Seward B Rutkove
PURPOSE OF REVIEW: In this article, we provide an overview of electrical impedance myography (EIM), including its technical and theoretical basis, a summary of its varied applications, and ongoing developments. RECENT FINDINGS: EIM has been used as a disease severity biomarker in a variety of disorders affecting the muscle, ranging from amyotrophic lateral sclerosis (ALS) to muscular dystrophies to disuse atrophy due to the weightlessness of space. In ALS, studies have demonstrated that major reductions in sample size in clinical trials can be achieved...
September 20, 2017: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/28911903/the-vaccines-and-antibodies-associated-with-als3p-for-treatment-of-candida-albicans-infections
#2
REVIEW
Xue Sui, Lan Yan, Yuan-Ying Jiang
Candida albicans is the most common fungal microorganism in healthy individuals, as well as the cause of high mortality infections in high-risk hosts such as immunocompromised patients. Antifungal vaccines and monoclonal antibodies useful for active or passive immunizations have recently generated considerable excitement for the treatment of fungal infections. The cell wall proteins of C. albicans, which are crucial for virulence and pathogenicity, are attractive target antigens. Als3p, a member of the C. albicans agglutinin-like sequence (ALS) family, is a hyphal-specific glycophosphatidylinositol cell wall protein that plays a key role in the interaction with host cells...
September 11, 2017: Vaccine
https://www.readbyqxmd.com/read/28872919/a-safety-analysis-of-edaravone-mci-186-during-the-first-six-cycles-24-weeks-of-amyotrophic-lateral-sclerosis-als-therapy-from-the-double-blind-period-in-three-randomized-placebo-controlled-studies
#3
Alexander Kalin, Elvia Medina-Paraiso, Kaoru Ishizaki, Alex Kim, Yannong Zhang, Takanori Saita, Masahiko Wasaki
BACKGROUND: There continues to be a need for new therapies to treat ALS. OBJECTIVE: Provide an overview of safety for edaravone in ALS patients during the first six cycles of treatment. METHODS: Analysis was based on three randomised, placebo-controlled clinical trials. Endpoints included treatment-emergent adverse events (TEAEs), including AEs leading to discontinuation, serious adverse events (SAEs), and deaths. RESULTS: The analysis included a total of 368 patients (184 in the edaravone group and placebo group, respectively)...
October 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28872912/an-assessment-of-treatment-guidelines-clinical-practices-demographics-and-progression-of-disease-among-patients-with-amyotrophic-lateral-sclerosis-in-japan-the-united-states-and-europe
#4
Koji Takei, Kikumi Tsuda, Fumihiro Takahashi, Manabu Hirai, Joseph Palumbo
BACKGROUND: There is an increasing clinical research focus on neuroprotective agents in amyotrophic lateral sclerosis (ALS). However, it is unclear how generalisable clinical study trial results are between different countries and regions. OBJECTIVE: To assess similarities and differences in clinical practice and treatment guidelines for ALS, and also to compare the demographics and rate of progression of disease in patients with ALS enrolled in clinical trials in Japan, the US, and Europe...
October 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28872908/pharmacokinetic-profile-of-edaravone-a-comparison-between-japanese-and-caucasian-populations
#5
Yoshinobu Nakamaru, Shuji Kinoshita, Atsuhiro Kawaguchi, Koji Takei, Joseph Palumbo, Masayuki Suzuki
BACKGROUND: Amyotrophic lateral sclerosis (ALS) affects persons of all races, and there continues to be a need for effective therapies to treat the disease. OBJECTIVE: To compare the pharmacokinetics (PK) of edaravone between Japanese and Caucasian populations. METHODS: Data from five PK studies among Japanese and Caucasian healthy volunteers were pooled and evaluated. In population PK (PPK) modelling, compartment models and other models with linear elimination were evaluated for appropriateness...
October 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28869393/ethics-of-clinical-research-in-patients-with-als-is-there-a-risk-of-exploitation
#6
Carmel Armon
Amyotrophic lateral sclerosis is the most common degenerative disease of the motor neuron network. Due to its inevitably fatal outcome, numerous attempts have been made to ameliorate its course. The emergence of every new technology has spurred research to see if it might benefit patients with ALS (PALS). This paper suggests a detailed road map to be followed when performing clinical research in PALS to satisfy the ethics requirements of the Declaration of Helsinki, expressed using the terminology of Beauchamp and Childress...
September 4, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28869278/cerebral-near-infrared-spectroscopy-monitoring-for-prevention-of-brain-injury-in-very-preterm-infants
#7
REVIEW
Simon Hyttel-Sorensen, Gorm Greisen, Bodil Als-Nielsen, Christian Gluud
BACKGROUND: Cerebral injury and long-term neurodevelopmental impairment is common in extremely preterm infants. Cerebral near-infrared spectroscopy (NIRS) enables continuous estimation of cerebral oxygenation. This diagnostic method coupled with appropriate interventions if NIRS is out of normal range (that is cerebral oxygenation within the 55% to 85% range) may offer benefits without causing more harms. Therefore, NIRS coupled with appropriate responses to abnormal findings on NIRS needs assessment in a systematic review of randomised clinical trials and quasi-randomised studies...
September 4, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28864675/causal-inference-methods-to-study-gastric-tube-use-in-amyotrophic-lateral-sclerosis
#8
Erin McDonnell, David Schoenfeld, Sabrina Paganoni, Nazem Atassi
OBJECTIVE: To estimate effects of gastric tube (G-tube) on survival and quality of life (QOL) in people with amyotrophic lateral sclerosis (ALS) correcting for confounding by indication inherent in nonrandomized observational data. METHODS: To complement a recent causal inference analysis, which concluded that G-tube placement increases the hazard of death, permanent assisted ventilation, or tracheostomy by 28%, we fit causal inference models on a different sample of 481 patients with ALS enrolled in a recent clinical trial of ceftriaxone...
September 1, 2017: Neurology
https://www.readbyqxmd.com/read/28857199/urate-levels-predict-survival-in-als-analysis-of-the-expanded-pro-act-database
#9
Sabrina Paganoni, Katharine Nicholson, James Chan, Amy Shui, David Schoenfeld, Alexander Sherman, James Berry, Merit Cudkowicz, Nazem Atassi
INTRODUCTION: Urate has been identified as a predictor of ALS survival in some, but not all studies. Here we leverage the recent expansion of the PRO-ACT database to study the association between urate levels and ALS survival. METHODS: Pooled data of 1,736 ALS participants from the PRO-ACT database were analyzed. Cox proportional hazards regression models were used to evaluate associations between urate levels at trial entry and survival. RESULTS: After adjustment for potential confounders (i...
August 31, 2017: Muscle & Nerve
https://www.readbyqxmd.com/read/28828608/eaat2-and-the-molecular-signature-of-amyotrophic-lateral-sclerosis
#10
Lauren Taylor Rosenblum, Davide Trotti
Amyotrophic lateral sclerosis (ALS) is a rapid and fatal neurodegenerative disease, primarily affecting upper and lower motor neurons. It is an extremely heterogeneous disease in both cause and symptom development, and its mechanisms of pathogenesis remain largely unknown. Excitotoxicity, a process caused by excessive glutamate signaling, is believed to play a substantial role, however. Excessive glutamate release, changes in postsynaptic glutamate receptors, and reduction of functional astrocytic glutamate transporters contribute to excitotoxicity in ALS...
2017: Advances in Neurobiology
https://www.readbyqxmd.com/read/28805578/nonmotor-symptoms-in-amyotrophic-lateral-sclerosis-a-systematic-review
#11
Ton Fang, Felix Jozsa, Ammar Al-Chalabi
BACKGROUND: ALS is a progressive neurodegenerative disease with no curative treatment. Nonmotor symptoms presenting in ALS may cause significant distress, worsen prognosis, and affect survival. OBJECTIVE: To systematically review evidence for the prevalence of nonmotor ALS symptoms, and treatment options. METHODS: Multiple medical literature databases were searched and studies screened using predefined inclusion criteria. Of 4580 studies, 44 were eligible for inclusion with 25 relating to treatment and 19 to the prevalence of nonmotor symptoms in ALS...
2017: International Review of Neurobiology
https://www.readbyqxmd.com/read/28801400/protein-misfolding-amyotrophic-lateral-sclerosis-and-guanabenz-protocol-for-a-phase-ii-rct-with-futility-design-promise-trial
#12
Eleonora Dalla Bella, Irene Tramacere, Giovanni Antonini, Giuseppe Borghero, Margherita Capasso, Claudia Caponnetto, Adriano Chiò, Massimo Corbo, Roberto Eleopra, Massimiliano Filosto, Fabio Giannini, Enrico Granieri, Vincenzo La Bella, Christian Lunetta, Jessica Mandrioli, Letizia Mazzini, Sonia Messina, Maria Rosaria Monsurrò, Gabriele Mora, Nilo Riva, Romana Rizzi, Gabriele Siciliano, Vincenzo Silani, Isabella Simone, Gianni Sorarù, Paolo Volanti, Giuseppe Lauria
INTRODUCTION: Recent studies suggest that endoplasmic reticulum stress may play a critical role in the pathogenesis of amyotrophic lateral sclerosis (ALS) through an altered regulation of the proteostasis, the cellular pathway-balancing protein synthesis and degradation. A key mechanism is thought to be the dephosphorylation of eIF2α, a factor involved in the initiation of protein translation. Guanabenz is an alpha-2-adrenergic receptor agonist safely used in past to treat mild hypertension and is now an orphan drug...
August 11, 2017: BMJ Open
https://www.readbyqxmd.com/read/28799809/nutrition-management-methods-effective-in-increasing-weight-survival-time-and-functional-status-in-als-patients-a-systematic-review
#13
Jaylin Kellogg, Lindsey Bottman, Erin J Arra, Stephen M Selkirk, Frances Kozlowski
Poor prognosis and decreased survival time correlate with the nutritional status of patients with amyotrophic lateral sclerosis (ALS). Various studies were reviewed which assessed weight, body mass index (BMI), survival time and ALS functional rating scale revised (ALSFRS-R) in order to determine the best nutrition management methods for this patient population. A systematic review was conducted using CINAHL, Medline, and PubMed, and various search terms in order to determine the most recent clinical trials and observational studies that have been conducted concerning nutrition and ALS...
August 11, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28762856/exploring-the-diagnosis-delay-and-als-functional-impairment-at-diagnosis-as-relevant-criteria-for-clinical-trial-enrolment
#14
Bello Hamidou, Benoit Marin, Geraldine Lautrette, Marie Nicol, William Camu, Philippe Corcia, Marie-Christine Arnes-Bes, Christine Tranchant, Pierre Clavelou, Didier Hannequin, Giroud Maurice, Katell Beauvais, Jean-Christophe Antoine, Véronique Danel-Brunaud, Fausto Viader, Pierre-Marie Preux, Philippe Couratier
Objectives were: i) to describe the phenotypic heterogeneity of incident amyotrophic lateral sclerosis (ALS) patients diagnosed in 2012 in French ALS centres; ii) to look at the associations between ALSFRS-R score and ALSFRS-R slope (ΔFS) at time of diagnosis with diagnosis delay, ALS phenotypes and Airlie House diagnosis criteria (AHDC); iii) to describe the rate of progression on ΔFS, according to diagnosis delay. METHODS: Incident ALS cases diagnosed in French ALS centres were included...
August 1, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28742871/comprehensive-immune-profiling-reveals-substantial-immune-system-alterations-in-a-subset-of-patients-with-amyotrophic-lateral-sclerosis
#15
Michael P Gustafson, Nathan P Staff, Svetlana Bornschlegl, Greg W Butler, Mary L Maas, Mohamed Kazamel, Adeel Zubair, Dennis A Gastineau, Anthony J Windebank, Allan B Dietz
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with a median lifespan of 2-3 years after diagnosis. There are few meaningful treatments that alter progression in this disease. Preclinical and clinical studies have demonstrated that neuroinflammation may play a key role in the progression rate of ALS. Despite this, there are no validated biomarkers of neuroinflammation for use in clinical practice or clinical trials. Biomarkers of neuroinflammation could improve patient management, provide new therapeutic targets, and possibly help stratify clinical trial selection and monitoring...
2017: PloS One
https://www.readbyqxmd.com/read/28710685/pericytes-extend-survival-of-als-sod1-mice-and-induce-the-expression-of-antioxidant-enzymes-in-the-murine-model-and-in-ipscs-derived-neuronal-cells-from-an-als-patient
#16
Giuliana Castello Coatti, Miriam Frangini, Marcos C Valadares, Juliana Plat Gomes, Natalia O Lima, Natale Cavaçana, Amanda F Assoni, Mayra V Pelatti, Alexander Birbrair, Antonio Carlos Pedroso de Lima, Julio M Singer, Francisco Marcelo M Rocha, Giovani Loiola Da Silva, Mario Sergio Mantovani, Lucia Inês Macedo-Souza, Merari F R Ferrari, Mayana Zatz
Amyotrophic Lateral Sclerosis (ALS) is one of the most common adult-onset motor neuron disease causing a progressive, rapid and irreversible degeneration of motor neurons in the cortex, brain stem and spinal cord. No effective treatment is available and cell therapy clinical trials are currently being tested in ALS affected patients. It is well known that in ALS patients, approximately 50% of pericytes from the spinal cord barrier are lost. In the central nervous system, pericytes act in the formation and maintenance of the blood-brain barrier, a natural defense that slows the progression of symptoms in neurodegenerative diseases...
July 14, 2017: Stem Cell Reviews
https://www.readbyqxmd.com/read/28705085/international-survey-of-als-experts-about-critical-questions-for-assessing-patients-with-als
#17
Mamede De Carvalho, Adam Ryczkowski, Peter Andersen, Marta Gromicho, Julian Grosskreutz, Magdalena Kuźma-Kozakiewicz, Susanne Petri, Maria Piotrkiewicz, Gabriel Miltenberger Miltenyi
OBJECTIVE: To define an applicable dataset for ALS patient registries we weighted specific clinical items as scored by worldwide ALS experts. METHODS: Sixty participants were invited based on relevant clinical work, publications and personal acquaintance. They rated 160 clinical items consensually agreed by the members of our project, incorporating specialists from five European Centres. Scoring scheme was defined as: 1 - essential; 2 - important; 3 - not very important...
July 13, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28674995/biomarkers-in-neurodegenerative-diseases
#18
Andreas Jeromin, Robert Bowser
The past decade has seen tremendous efforts in biomarker discovery and validation for neurodegenerative diseases. The source and type of biomarkers has continued to grow for central nervous system diseases, from biofluid-based biomarkers (blood or cerebrospinal fluid (CSF)), to nucleic acids, tissue, and imaging. While DNA remains a predominant biomarker used to identify familial forms of neurodegenerative diseases, various types of RNA have more recently been linked to familial and sporadic forms of neurodegenerative diseases during the past few years...
2017: Advances in Neurobiology
https://www.readbyqxmd.com/read/28674982/motoneuron-disease-clinical
#19
Hristelina Ilieva, Nicholas J Maragakis
ALS is a neurodegenerative disease in which the primary symptoms result in progressive neuromuscular weakness. Recent studies have highlighted that there is significant heterogeneity with regard to anatomical and temporal disease progression. Importantly, more recent advances in genetics have revealed new causative genes to the disease. New efforts have focused on the development of biomarkers that could aid in diagnosis, prognosis, and serve as pharmacodynamics markers. Although traditional pharmaceuticals continue to undergo trials for ALS, new therapeutic strategies including stem cell transplantation studies, gene therapies, and antisense therapies targeting some of the familial forms of ALS are gaining momentum...
2017: Advances in Neurobiology
https://www.readbyqxmd.com/read/28664036/virtual-brain-biopsies-in-amyotrophic-lateral-sclerosis-diagnostic-classification-based-on-in-vivo-pathological-patterns
#20
Peter Bede, Parameswaran M Iyer, Eoin Finegan, Taha Omer, Orla Hardiman
BACKGROUND: Diagnostic uncertainty in ALS has serious management implications and delays recruitment into clinical trials. Emerging evidence of presymptomatic disease-burden provides the rationale to develop diagnostic applications based on the evaluation of in-vivo pathological patterns early in the disease. OBJECTIVES: To outline and test a diagnostic classification approach based on an array of complementary imaging metrics in key disease-associated anatomical structures...
2017: NeuroImage: Clinical
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