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https://www.readbyqxmd.com/read/28426258/improvements-in-out-of-hospital-cardiac-arrest-survival-from-1998-to-2013
#1
Yutaka Yamaguchi, Jeff A Woodin, Koichiro Gibo, Dana M Zive, Mohamud R Daya
OBJECTIVES: Out-of-hospital cardiac arrest (OHCA) remains a major public health burden. Aggregate OHCA survival to hospital discharge has reportedly remained unchanged at 7.6% for almost 30 years from 1970 to 2008. We examined the trends in adult OHCA survival over a 16-year period from 1998 to 2013 within a single EMS agency. METHODS: Observational cohort study of adult OHCA patients treated by Tualatin Valley Fire & Rescue (TVF&R) from 1998 to 2013. This is an ALS first response fire agency that maintains an active Utstein style cardiac arrest registry and serves a population of approximately 450,000 in 9 incorporated cities in Oregon...
April 20, 2017: Prehospital Emergency Care
https://www.readbyqxmd.com/read/28421301/multiple-sclerosis-and-amyotrophic-lateral-sclerosis-a-human-leukocyte-antigen-challenge
#2
Vincenzo Dattola, Fausto Famà, Margherita Russo, Rocco Salvatore Calabrò, Anna Lisa Logiudice, Maria Grazia Grasso, Francesco Patti, Maria Buccafusca
Multiple sclerosis (MS) and amyotrophic lateral sclerosis (ALS) are two different central nervous system pathology that, according to the most accredited hypotheses, recognize a different etiopathogenesis. The simultaneous occurrence of MS and ALS is quite unusual. To our knowledge, only three cases have been so far described by clinical, laboratory, and post-mortem studies. We report four new cases of this peculiar combination that have been observed and are herein described, included their human leukocyte antigen (HLA) profile studies...
April 18, 2017: Neurological Sciences
https://www.readbyqxmd.com/read/28414559/evaluating-the-cost-and-utility-of-mandating-schools-to-stock-epinephrine-auto-injectors
#3
Chelsea Steffens, Benjamin Clement, William Fales, Ahel El Haj Chehade, Kevin Putman, Robert Swor
BACKGROUND: The Michigan Legislature mandated that all public schools stock epinephrine auto-injectors (EAIs). A minimal amount is known regarding the incremental value of EAIs in schools. Our primary objective was to describe the frequency of administration of epinephrine for EMS patients with acute allergic reactions in public schools. Our secondary objective was to estimate the cost of mandating public schools to stock EAIs. METHODS: We performed a retrospective cohort study of EMS cases with an impression of allergic reaction and who received epinephrine recorded in the 2014 Michigan EMS Information System (MI-EMSIS)...
April 17, 2017: Prehospital Emergency Care
https://www.readbyqxmd.com/read/28387141/nationwide-incidence-of-motor-neuron-disease-using-the-french-health-insurance-information-system-database
#4
Sofiane Kab, Frédéric Moisan, Pierre-Marie Preux, Benoît Marin, Alexis Elbaz
OBJECTIVE: There are no estimates of the nationwide incidence of motor neuron disease (MND) in France. We used the French health insurance information system to identify incident MND cases (2012-2014), and compared incidence figures to those from three external sources. METHODS: We identified incident MND cases (2012-2014) based on three data sources (riluzole claims, hospitalisation records, long-term chronic disease benefits), and computed MND incidence by age, gender, and geographic region...
April 7, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28381746/syndrome-of-inappropriate-antidiuretic-hormone-secretion-associated-with-amyotrophic-lateral-sclerosis-in-a-patient-developing-carbon-dioxide-narcosis
#5
Yui Inoue, Takaaki Murakami, Takeshi Nakamura, Kyohei Morita, Daita Kaneda, Ichizo Nishino, Tetsuya Hayashi, Yuya Shinoto, Tomonobu Hatoko, Tomoko Kato, Shin Yonemitsu, Seiji Muro, Shogo Oki
We report a rare case of syndrome of inappropriate antidiuretic hormone secretion (SIADH) associated with amyotrophic lateral sclerosis (ALS). A 69-year-old man was admitted to our hospital with sustained hyponatremia. Hyposmolality with elevated urinary osmolality and sodium excretion was observed, which indicated SIADH. The treatment for SIADH was challenging; the patient developed carbon dioxide narcosis, which led to the diagnosis of ALS. After the initiation of noninvasive positive-pressure ventilation, the patient's serum sodium concentration normalized and became stable...
2017: Internal Medicine
https://www.readbyqxmd.com/read/28371266/autism-spectrum-disorders-and-the-amplitude-of-auditory-brainstem-response-wave-i
#6
Mariline Santos, Cristina Marques, Ana Nóbrega Pinto, Raquel Fernandes, Miguel Bebiano Coutinho, Cecília Almeida E Sousa
To determine whether children with autism spectrum disorders (ASDs) have an increased number of wave I abnormal amplitudes in auditory brainstem responses (ABRs) than age- and sex-matched typically developing children. This analytical case-control study compared patients with ASDs between the ages of 2 and 6 years and children who had a language delay not associated with any other pathology. Amplitudes of ABR waves I and V; absolute latencies (ALs) of waves I, III, and V; and interpeak latencies (IPLs) I-III, III-IV, and I-V at 90 dB were compared between ASD patients and normally developing children...
April 1, 2017: Autism Research: Official Journal of the International Society for Autism Research
https://www.readbyqxmd.com/read/28367949/an-early-history-of-japanese-amyotrophic-lateral-sclerosis-als-and-the-current-significance
#7
Koji Abe
The present review focuses an early history of Japanese amyotrophic lateral sclerosis (ALS) and the current significance in comparison to previously known and newly found reports on Japanese ALS. After a preliminary case report of ALS by Masamichi Hirai on 1890, 2 completed reports were simultaneously published within 2 weeks of 1891 by Momojiro Nakamura and Zenjiro Inoue, followed by Eikichi Watanabe's report on 1892. After Shonosuke Hasegawa's and Hiroshi Kawahara's case reports on 1894-1896, Aihiko Sata first reported an autopsy case of ALS on 1897...
March 30, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/28358904/neurotrophic-effects-of-progranulin-in-vivo-in-reversing-motor-neuron-defects-caused-by-over-or-under-expression-of-tdp-43-or-fus
#8
Babykumari P Chitramuthu, Denis G Kay, Andrew Bateman, Hugh P J Bennett
Progranulin (PGRN) is a glycoprotein with multiple roles in normal and disease states. Mutations within the GRN gene cause frontotemporal lobar degeneration (FTLD). The affected neurons display distinctive TAR DNA binding protein 43 (TDP-43) inclusions. How partial loss of PGRN causes TDP-43 neuropathology is poorly understood. TDP-43 inclusions are also found in affected neurons of patients with other neurodegenerative diseases including amyotrophic lateral sclerosis (ALS) and Alzheimer's disease. In ALS, TDP-43 inclusions are typically also immunoreactive for fused in sarcoma (FUS)...
2017: PloS One
https://www.readbyqxmd.com/read/28356084/conjugal-amyotrophic-lateral-sclerosis-a-case-report-from-scotland
#9
P M Fernandes, M R Macleod, A Bateman, S Abrahams, S Pal
BACKGROUND: Conjugal amyotrophic lateral sclerosis is rare, with significant effects on psychological and care needs. We report a case of conjugal amyotrophic lateral sclerosis disease from central Scotland. This case is particularly unusual as both patients were diagnosed within an 18-month period and experienced the disease simultaneously, with similar symptomatology and progression. CASE PRESENTATION: Patient A was a 71-year-old man who presented with unilateral arm weakness and wasting...
March 29, 2017: BMC Neurology
https://www.readbyqxmd.com/read/28355414/retrospective-assessment-of-occupational-exposures-for-the-geneva-study-of-als-among-military-veterans
#10
Anila Bello, Susan R Woskie, Rebecca Gore, Dale P Sandler, Silke Schmidt, Freya Kamel
Objective: This paper describes the retrospective exposure assessment conducted to assess occupational exposures for the Genes and Environmental Exposures in Veterans (GENEVA) study, a case-control study investigating the joint contribution of genetics and environmental exposures to the risk of amyotrophic lateral sclerosis (ALS) among military veterans. Methods: Occupational histories for 1597 study participants collected as part of the GENEVA study were the basis for this retrospective exposure assessment...
January 1, 2017: Annals of Work Exposures and Health
https://www.readbyqxmd.com/read/28301478/retrotransposon-activation-contributes-to-neurodegeneration-in-a-drosophila-tdp-43-model-of-als
#11
Lisa Krug, Nabanita Chatterjee, Rebeca Borges-Monroy, Stephen Hearn, Wen-Wei Liao, Kathleen Morrill, Lisa Prazak, Nikolay Rozhkov, Delphine Theodorou, Molly Hammell, Josh Dubnau
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are two incurable neurodegenerative disorders that exist on a symptomological spectrum and share both genetic underpinnings and pathophysiological hallmarks. Functional abnormality of TAR DNA-binding protein 43 (TDP-43), an aggregation-prone RNA and DNA binding protein, is observed in the vast majority of both familial and sporadic ALS cases and in ~40% of FTLD cases, but the cascade of events leading to cell death are not understood...
March 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28293919/-extracorporeal-liver-support-of-liver-failure
#12
Hans Ulrich Gerth, Michele Pohlen, Hermann Pavenstädt, Hartmut Schmidt
Extracorporeal liver support can be classified into cell-free, artificial methods (artificial liver support, ALS) and cell-based bioartificial methods (bioartificial liver support, BLS). ALS improves biochemical parameters of liver failure by the simultaneous removal of protein-bound and water-soluble substances. Here, the MARS therapy belongs to the most studied methods with a proved beneficial effect on hepatic encephalopathy (HE), hepatorenal syndrome (HRS) or hyperbilirubinemia. However, a general survival advantage of any liver support for liver failure has not been shown yet and is restricted to meta-analyses or patient subgroups...
April 2017: Zeitschrift Für Gastroenterologie
https://www.readbyqxmd.com/read/28291468/navy-en-route-care-a-3-year-review-of-428-navy-air-evacuations
#13
Benjamin Walrath, Alejandra Mora, Victoria Ganem, Stephen Harper, Elliot Ross, Chetan Kharod, Gerard Demers, Vikhyat S Bebarta
BACKGROUND: Navy medical personnel have been recording en route care (ERC) missions through Search and Rescue (SAR) reports since the 1970's. Our objective was to report clinical ERC cases treated by Navy operational assets from January 2012 to January 2015. METHODS: The Search and Rescue Model Manager office collects SAR reports for all patient transports involving Navy personnel and equipment. From these reports, descriptive statistics to include total number of patients transported, percentages of Advanced Life Support versus Basic Life Support transports, time of transport, and type of ERC provider for the transport were collected...
March 2017: Military Medicine
https://www.readbyqxmd.com/read/28291249/analysis-of-sod1-mutations-in-a-chinese-population-with-amyotrophic-lateral-sclerosis-a-case-control-study-and-literature-review
#14
QianQian Wei, QingQing Zhou, YongPing Chen, RuWei Ou, Bei Cao, YaQian Xu, Jing Yang, Hui-Fang Shang
Although the copper/zinc superoxide dismutase-1 (SOD1) gene has been identified in both familial ALS (FALS) and sporadic ALS (SALS), it has rarely been studied in Chinese patients with ALS, and there are few studies with large samples. This study sought to assess the prevalence of SOD1 mutations in Chinese ALS patients. We screened a cohort of 499 ALS patients (487 SALS and 12 FALS) from the Department of Neurology at the West China Hospital of Sichuan University and analyzed all coding exons of SOD1 by Sanger sequencing...
March 14, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28288521/young-onset-rapidly-progressive-als-associated-with-heterozygous-fus-mutation
#15
Marta Gromicho, Miguel Oliveira Santos, Anabela Pinto, Ana Pronto-Laborinho, Mamede De Carvalho
We report a 36-years-old Cape Verdean man who presented with respiratory insufficiency due to rapidly progressive sporadic amyotrophic lateral sclerosis (ALS), in whom FUS mutation c.1551C > G (p.Hist517Gln) in heterozygosity was identified, a finding previously described as non-pathogenic. The only previous report on this mutation was in a family from Cape Verde in which four members developed ALS; all were homozygous for the mutation. This case shows that this FUS mutation presents a highly variable penetrance and expressivity...
March 13, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28267691/vorapaxar-and-amyotrophic-lateral-sclerosis-coincidence-or-adverse-association
#16
REVIEW
Victor L Serebruany, Seth D Fortmann, Daniel F Hanley, Moo Hyun Kim
BACKGROUND: Vorapaxar, a novel antiplatelet thrombin PAR-1 inhibitor, is currently approved for post myocardial infarction and peripheral artery disease indications with concomitant use of clopidogrel and/or aspirin. The vorapaxar safety profile was acceptable. However, aside from heightened bleeding risks, excesses of solid cancers and diplopia, there were more amyotrophic lateral sclerosis (ALS) diagnoses after vorapaxar. STUDY QUESTION: To assess the Food and Drug Administration (FDA) reviews on the potential association of vorapaxar with ALS...
March 2017: American Journal of Therapeutics
https://www.readbyqxmd.com/read/28247171/genetic-counseling-dilemmas-for-a-patient-with-sporadic-amyotrophic-lateral-sclerosis-frontotemporal-degeneration-parkinson-s-disease
#17
Vittorio Mantero, Claudia Tarlarini, Angelo Aliprandi, Giuseppe Lauria, Andrea Rigamonti, Lucia Abate, Paola Origone, Paola Mandich, Silvana Penco, Andrea Salmaggi
Amyotrophic lateral sclerosis (ALS), frontotemporal degeneration and Parkinson's disease may be different expressions of the same neurodegenerative disease. However, association between ALS and parkinsonism-dementia complex (ALS-PDC) has only rarely been reported apart from the cluster detected in Guam. We report a patient presenting with ALS-PDC in whom pathological mutations/expansions were investigated. No other family members were reported to have any symptoms of a neurological condition. Our case demonstrates that ALS-PDC can occur as a sporadic disorder, even though the coexistence of the three clinical features in one patient suggests a single underlying genetic cause...
March 1, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/28241758/clinical-trials-from-the-patient-perspective-survey-in-an-online-patient-community
#18
Pronabesh DasMahapatra, Priya Raja, Jeremy Gilbert, Paul Wicks
BACKGROUND: Developing new medicines relies on the successful conduct of clinical trials. As trial protocols become more arduous, it becomes harder to recruit and retain patient volunteers, although recent efforts such as OMERACT and I-SPY2 show that partnering with patients can be beneficial. We sought to describe drivers and barriers to trial participation, as well as condition-specific trial preferences. METHODS: An online survey was fielded via the patient-powered research network PatientsLikeMe to 1,621 members living with nine selected chronic health conditions...
February 27, 2017: BMC Health Services Research
https://www.readbyqxmd.com/read/28220290/epidemiology-of-amyotrophic-lateral-sclerosis-in-southern-germany
#19
Angela Rosenbohm, Raphael S Peter, Siegfried Erhardt, Dorothée Lulé, Dietrich Rothenbacher, Albert C Ludolph, Gabriele Nagel
The objective of this study is to determine the current distribution of clinical phenotypes and to estimate future trends of ALS incidence in Western societies. We report on a clinical-epidemiological registry with a capture-recapture rate of >80% and population-based case-control study in ALS patients in South Western Germany. 1163 incidents of ALS were registered. Clinical and neuropsychological data were prospectively collected from 699 cases. The mean age at onset was 66.6 (SD = 11.6) years in prospective cases (N = 699)...
February 20, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28195573/whole-exome-sequencing-of-individuals-from-an-isolated-population-implicates-rare-risk-variants-in-bipolar-disorder
#20
F Lescai, T D Als, Q Li, M Nyegaard, G Andorsdottir, M Biskopstø, A Hedemand, A Fiorentino, N O'Brien, A Jarram, J Liang, J Grove, J Pallesen, E Eickhardt, M Mattheisen, L Bolund, D Demontis, A G Wang, A McQuillin, O Mors, J Wang, A D Børglum
Bipolar disorder affects about 1% of the world's population, and its estimated heritability is about 75%. Only few whole genome or whole-exome sequencing studies in bipolar disorder have been reported, and no rare coding variants have yet been robustly identified. The use of isolated populations might help finding variants with a recent origin, more likely to have drifted to higher frequency by chance. Following this approach, we investigated 28 bipolar cases and 214 controls from the Faroe Islands by whole exome sequencing, and the results were followed-up in a British sample of 2025 cases and 1358 controls...
February 14, 2017: Translational Psychiatry
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