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https://www.readbyqxmd.com/read/28433478/investigation-on-acute-effects-of-enzyme-replacement-therapy-and-influence-of-clinical-severity-on-physiological-variables-related-to-exercise-tolerance-in-patients-with-late-onset-pompe-disease
#1
Annalisa Sechi, Desy Salvadego, Alessandro Da Ponte, Nicole Bertin, Andrea Dardis, Silvia Cattarossi, Grazia Devigili, Federico Reccardini, Bruno Bembi, Bruno Grassi
Exercise intolerance is one of the clinical hallmarks of late-onset Pompe disease (LOPD). We studied the acute effects of ERT on the physiological variables associated with exercise tolerance in patients chronically ERT treated. Moreover, we assessed the influence of clinical severity on the investigated variables. The day before (B) and the day after (A) ERT injection, 11 LOPD patients performed on a cycle-ergometer an exercise tolerance test to voluntary exhaustion; VO2, HR, RPE, and GAA activity were determined in B and A...
March 10, 2017: Neuromuscular Disorders: NMD
https://www.readbyqxmd.com/read/28430960/triple-site-pacing-for-cardiac-resynchronization-in-permanent-atrial-fibrillation-follow-up-results-from-a-prospective-observational-study
#2
Pedro Marques, Miguel Nobre Menezes, Gustavo Lima da Silva, Tatiana Guimarães, Ana Bernardes, Nuno Cortez-Dias, Luis Carpinteiro, João de Sousa, Fausto J Pinto
Aims: Cardiac Resynchronization Therapy (CRT) is associated with a particularly high non-response rate in patients with atrial fibrillation (AF). We aimed to assess the effectiveness of triple-site (Tri-V) pacing CRT in this population. Methods and results: Prospective observational study of patients with permanent AF who underwent CRT implantation with an additional right ventricle lead in the outflow tract septal wall. After implantation, programming mode (Tri-V or biventricular pacing) was selected based on cardiac output determination...
April 19, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28427470/pulmonary-hypertension-in-lymphangioleiomyomatosis-prevalence-severity-and-the-role-of-carbon-monoxide-diffusion-capacity-as-a-screening-method
#3
Carolina S G Freitas, Bruno G Baldi, Carlos Jardim, Mariana S Araujo, Juliana Barbosa Sobral, Gláucia I Heiden, Ronaldo A Kairalla, Rogério Souza, Carlos R R Carvalho
BACKGROUND: Lymphangioleiomyomatosis (LAM) is included within group 5 of the current PH classification (unclear multifactorial mechanisms). However, data regarding the occurrence of PH in LAM are scarce. The aims of the study were to describe the prevalence and characteristics of PH in a large cohort of LAM patients with different levels of severity, and to evaluate the role of echocardiography and carbon monoxide diffusion capacity (DLCO) as screening methods for PH in LAM. METHODS: One hundred five LAM patients underwent transthoracic echocardiography, pulmonary function tests (PFTs) and 6-min walk test (6MWT)...
April 20, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28421409/six-minute-walk-test-for-assessing-physical-functional-capacity-in-chronic-heart-failure
#4
REVIEW
Huiyun Du, Parichat Wonggom, Jintana Tongpeth, Robyn A Clark
PURPOSE OF THE REVIEW: The six-minute walk test (6MWT) is a submaximal exercise test for evaluating physical functional capacity. This review aims to report the research on the use of the 6MWT in chronic heart failure (CHF) that has been published in the past 5 years. RECENT FINDINGS: The 6MWT distance does not accurately reflect peak VO2. Minimal clinically important difference in the 6MWT distance, and additional measurements, such as heart rate recovery, can assist in the interpretation of the 6MWT distance, so management decisions can be made...
April 18, 2017: Current Heart Failure Reports
https://www.readbyqxmd.com/read/28417609/action-plans-and-coping-strategies-in-elderly-copd-patients-influence-the-result-of-pulmonary-rehabilitation-an-observational-study
#5
Patrizia Russo, Giulia Prinzi, Aliaksei Kisialou, Vittorio Cardaci, Emanuele Stirpe, Vittoria Conti, Massimo Fini, Stefano Bonassi
BACKGROUND: COPD management needs a comprehensive assessment of clinical features (symptoms severity, co-morbidities) together with life-style, behavioural, socio-economic and multi-omics parameters. Among the other issues, psycho-cognitive assessment plays a critical role. Coping strategies are used to manage psychological stress. AIM: To evaluate the association between coping strategies and outcome of Pulmonary Rehabilitation (PR). DESIGN: Observational study...
April 14, 2017: European Journal of Physical and Rehabilitation Medicine
https://www.readbyqxmd.com/read/28417202/cardiorespiratory-fitness-and-muscle-strength-in-pancreatic-cancer-patients
#6
Dorothea Clauss, Christine Tjaden, Thilo Hackert, Lutz Schneider, Cornelia M Ulrich, Joachim Wiskemann, Karen Steindorf
PURPOSE: Cancer patients frequently experience reduced physical fitness due to the disease itself as well as treatment-related side effects. However, studies on physical fitness in pancreatic cancer patients are missing. Therefore, we assessed cardiorespiratory fitness and muscle strength of pancreatic cancer patients. METHODS: We included 65 pancreatic cancer patients, mostly after surgical resection. Cardiorespiratory fitness was assessed using cardiopulmonary exercise testing (CPET) and 6-min walk test (6MWT)...
April 17, 2017: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/28410878/efficacy-of-laronidase-therapy-in-patients-with-mucopolysaccharidosis-type-i-who-initiated-enzyme-replacement-therapy-in-adult-age-a-systematic-review-and-meta-analysis
#7
Jordi Pérez-López, Montserrat Morales-Conejo, Mónica López-Rodríguez, Álvaro Hermida-Ameijeiras, Marc Moltó-Abad
BACKGROUND: The efficacy of starting enzyme replacement therapy (ERT) in adults with Muchopolysaccharidosis Type I (MPS-I) is controversial. Evaluating the benefits reported by patients initiating ERT with laronidase at adult age might help physicians decide whether the use of ERT in these patients is worthwhile from a clinical point of view. OBJECTIVE: To assess every effectiveness variable modified in MPS-I patients who initiated laronidase at adult age. METHODS: A systematic search of the literature, from inception to July 2016, was conducted using MEDLINE, EMBASE, CENTRAL and LILACS to identify randomized trials or observational studies including ≥1 MPS-I patients with ERT initiated in adult age (≥18years) and evaluating ERT efficacy...
April 9, 2017: Molecular Genetics and Metabolism
https://www.readbyqxmd.com/read/28385308/early-feasibility-study-of-a-transcatheter%C3%A2-tricuspid-valve-annuloplasty-scout-trial-30-day%C3%A2-results
#8
Rebecca T Hahn, Christopher U Meduri, Charles J Davidson, Scott Lim, Tamim M Nazif, Mark J Ricciardi, Vivek Rajagopal, Gorav Ailawadi, Mani A Vannan, James D Thomas, Dale Fowler, Stuart Rich, Randy Martin, Geraldine Ong, Adam Groothuis, Susheel Kodali
BACKGROUND: The SCOUT (Percutaneous Tricuspid Valve Annuloplasty System for Symptomatic Chronic Functional Tricuspid Regurgitation) trial is a prospective, single-arm, multicenter, early feasibility study of a novel transcatheter device to plicate the tricuspid annulus (TA) and reduce tricuspid regurgitation (TR). OBJECTIVES: This study tested the feasibility and safety of a novel transcatheter device and assessed its early performance and functional outcomes. METHODS: Between November 2015 and June 2016, 15 patients with New York Heart Association (NYHA) functional class ≥II and moderate or greater functional TR were enrolled...
April 11, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28384908/cardiac-effect-of-interstitial-lung-disease-correlated-with-spirometry-and-six-minute-walk-test
#9
Mitali Bharat Agrawal, Nilkanth Tukaram Awad
INTRODUCTION: The cardiac effect of different pulmonary functions, six minute walk distance, arterial blood gases and saturation in Interstitial Lung Disease (ILD) is not much known. So this study, a tertiary care hospital experience that entails to know the various factors in Pulmonary Hypertension (PH) mentioned above causing PH and their correlation with PH. AIM: To study the correlation of PH in patients with ILD with spirometry and six minute walk test (6MWT)...
February 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28384378/functional-outcome-after-surgery-in-patients-with-bone-sarcoma-around-the-knee-results-from-a-long-term-prospective-study
#10
Janneke C van Egmond-van Dam, W Peter Bekkering, Jos A M Bramer, Auke Beishuizen, Marta Fiocco, P D Sander Dijkstra
BACKGROUND AND OBJECTIVES: In a previous conducted study functional outcome of young patients with bone sarcoma located around the knee was longitudinally evaluated during the first 2 years postoperatively. Functional outcome improved significantly over the first 2 years. The purpose of this descriptive study was to evaluate the functional outcome of these patients at long-term follow-up of 7 years. METHODS: Functional outcome was assessed with the TESS, MSTS, Baecke questionnaire, and three functional performance tests: time up and down stairs (TUDS), various walking activities (VWA), and the 6-min walking test (6MWT)...
April 6, 2017: Journal of Surgical Oncology
https://www.readbyqxmd.com/read/28382660/the-effects-of-threshold-inspiratory-muscle-training-in-patients-with-chronic-obstructive-pulmonary-copd-disease-a-randomized-experimental-study
#11
Han-Yu Chuang, Hsiao Yun Chang, Yueh-Yen Fang, Su-Er Guo
AIMS: To investigate the effects of threshold inspiratory muscle training (IMT) in patients with stages II through IV chronic obstructive pulmonary disease (COPD) using maximum inspiratory pressure (PImax), baseline dyspnoea index (BDI), 6-minute walk test (6MWT), and quality of life (QoL). BACKGROUND: A threshold inspiratory muscle training device provides pressure for inspiratory muscle strength, but there is limited information on the effects of threshold inspiratory muscle training starting at low pressure training...
April 5, 2017: Journal of Clinical Nursing
https://www.readbyqxmd.com/read/28382609/metabolic-syndrome-as-a-factor-affecting-systemic-inflammation-in-patients-with-chronic-obstructive-pulmonary-disease
#12
R Rubinsztajn, T Przybyłowski, M Maskey-Warzęchowska, M Paplińska-Goryca, P Nejman-Gryz, K Karwat, R Chazan
Chronic obstructive pulmonary disease (COPD) is a systemic disease which may be associated with other comorbidities. The aim of the study was to estimate the incidence of metabolic syndrome (MS) in COPD patients and to assess its impact on systemic inflammation and lung function. MS was diagnosed in accordance with the recommendations of the Polish Forum for the Prevention of Cardiovascular Diseases. The study group consisted of 267 patients with stable COPD in all stages of severity. All patients underwent spirometry with bronchial reversibility testing and 6 min walk test (6MWT)...
April 6, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28377186/reliability-construct-validity-and-determinants-of-6-minute-walk-test-performance-in-patients-with-chronic-heart-failure
#13
Nicole H M K Uszko-Lencer, Rafael Mesquita, Eefje Janssen, Christ Werter, Hans-Peter Brunner-La Rocca, Fabio Pitta, Emiel F M Wouters, Martijn A Spruit
BACKGROUND: In-depth analyses of the measurement properties of the 6-minute walk test (6MWT) in patients with chronic heart failure (CHF) are lacking. We investigated the reliability, construct validity, and determinants of the distance covered in the 6MWT (6MWD) in CHF patients. METHODS: 337 patients were studied (median age 65years, 70% male, ejection fraction 35%). Participants performed two 6MWTs on subsequent days. Demographics, anthropometrics, clinical data, ejection fraction, maximal exercise capacity, body composition, lung function, and symptoms of anxiety and depression were also assessed...
February 28, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28375682/active-video-gaming-for-children-with-cerebral-palsy-does-a-clinic-based-virtual-reality-component-offer-an-additive-benefit-a-pilot-study
#14
Danielle Levac, Anna McCormick, Mindy F Levin, Marie Brien, Richard Mills, Elka Miller, Heidi Sveistrup
AIMS: To compare changes in gross motor skills and functional mobility between ambulatory children with cerebral palsy who underwent a 1-week clinic-based virtual reality intervention (VR) followed by a 6-week, therapist-monitored home active video gaming (AVG) program and children who completed only the 6-week home AVG program. METHODS: Pilot non-randomized controlled trial. Five children received 1 hour of VR training for 5 days followed by a 6-week home AVG program, supervised online by a physical therapist...
April 4, 2017: Physical & Occupational Therapy in Pediatrics
https://www.readbyqxmd.com/read/28367422/adipose-derived-mesenchymal-stem-cells-for-treatment-of-airway-injuries-in-a-patient-after-long-term-exposure-to-sulfur-mustard
#15
Amir Nejad-Moghaddam, Soheila Ajdari, Eisa Tahmasbpour, Hassan Goodarzi, Yunes Panahi, Mostafa Ghanei
OBJECTIVE: Sulfur mustard (SM) is a potent mutagenic agent that targets several organs, particularly lung tissue. Changes in morphological structure of the airway system are associated with chronic obstructive pulmonary deficiency following exposure to SM. Although numerous studies have demonstrated pathological effects of SM on respiratory organs, unfortunately there is no effective treatment to inhibit further respiratory injuries or induce repair in these patients. Due to the extensive progress and achievements in stem cell therapy, we have aimed to evaluate safety and potential efficacy of systemic mesenchymal stem cell (MSC) administration on a SM-exposed patient with chronic lung injuries...
April 2017: Cell Journal
https://www.readbyqxmd.com/read/28365577/submaximal-exercise-capacity-in-juvenile-dermatomyositis-after-longterm-disease-the-contribution-of-muscle-lung-and-heart-involvement
#16
Kristin Schjander Berntsen, Anita Tollisen, Thomas Schwartz, Eva Kirkhus, Trond Mogens Aaløkken, May Brit Lund, Berit Flatø, Ivar Sjaastad, Helga Sanner
OBJECTIVE: To compare submaximal exercise capacity in patients with juvenile dermatomyositis (JDM) with controls, and analyze contributions of muscle, heart, and lung impairment in patients. METHODS: Fifty-nine patients with JDM, with a mean 16.9 years after symptom onset, and 59 sex- and age-matched controls completed a 6-min walk test (6MWT) and a timed up and go (TUG) test. Muscle function, disease activity/damage, and health-related quality of life (HRQOL) were assessed by validated tools; heart function by echocardiography and electrocardiography; and lung function by spirometry, DLCO, and body plethysmography...
April 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28353374/prediction-of-poor-short-term-prognosis-and-unmet-needs-in-advanced-chronic-obstructive-pulmonary-disease-use-of-the-two-minute-walking-distance-extracted-from-a-six-minute-walk-test
#17
Han-Yee Neo, Hui-Ying Xu, Huei-Yaw Wu, Allyn Hum
OBJECTIVES: Prognostic challenges hinder the identification of patients with advanced chronic obstructive pulmonary disease (COPD) for timely palliative interventions. We postulate that a two-minute derivative (two-minute walking distance [2MWD]) of a standard six-minute walk test (6MWT) can identify frail subjects with poorer survival for early palliative intervention. The primary outcome of interest is mortality at 18 months. Secondary objectives include evaluation of the relationship between the 2MWD and ability to self-care, dyspnea-related disabilities, nutrition, forced expiratory volume in first second (FEV1), quality of life (QoL), and comorbidity burden...
March 29, 2017: Journal of Palliative Medicine
https://www.readbyqxmd.com/read/28344613/anp-and-bnp-plasma-levels-in-patients-with-rheumatic-mitral-stenosis-after-percutaneous-balloon-mitral-valvuloplasty
#18
Łukasz Mazurkiewicz, Witold Rużyłło, Zbigniew Chmielak, Ewa Opalińska-Ciszek, Jadwiga Janas, Piotr Hoffman, Tomasz Hryniewiecki, Jacek Grzybowski
INTRODUCTION: Atrial (ANP) and B-type (BNP) natriuretic peptides are hormones secreted by the heart as a response to volume expansion and pressure overload. AIM: To assess the changes of ANP and BNP after percutaneous balloon mitral valvuloplasty (PBMV) and to investigate factors associated with endpoints. MATERIAL AND METHODS: The study included 96 patients (90.7% females, age 51.6 ±12.2 years) with rheumatic mitral valve stenosis (mitral valve area (MVA) 1...
2017: Postępy W Kardiologii Interwencyjnej, Advances in Interventional Cardiology
https://www.readbyqxmd.com/read/28340285/the-six-minute-walk-test-in-patients-with-al-amyloidosis-a-single-centre-case-series
#19
Vina Pulido, Gheorghe Doros, John L Berk, Vaishali Sanchorawala
The six-minute walk test (6MWT) has been widely used as an objective evaluation of functional exercise capacity and response to medical intervention in cardiopulmonary diseases. However, little is known about the 6MWT in evaluating patients with AL amyloidosis. We performed a retrospective study of 120 adults with systemic AL amyloidosis (60 with cardiac involvement and 60 without cardiac involvement) who had their initial evaluation at the Amyloidosis Center between 2013 and 2015 and had undergone 6MWT as a measure of functional exercise capacity...
March 24, 2017: British Journal of Haematology
https://www.readbyqxmd.com/read/28329121/very-low-calorie-diets-for-weight-loss-in-obese-older-adults-a-randomized-trial
#20
Cilla J Haywood, Luke A Prendergast, Katrina Purcell, Lauren Le Fevre, Wen Kwang Lim, Mary Galea, Joseph Proietto
Background: Obesity contributes to disability in older adults, and this is offset by weight loss and exercise. Very Low Calorie Diets (VLCDs) achieve rapid weight loss; however, these have not been rigorously evaluated in older people. Methods: A randomized trial was conducted from August 2012 through December 2015. The intervention was 12 weeks of thrice weekly exercise combined with either healthy eating advice (Ex/HE), hypocaloric diet (Ex/Diet), or VLCD (Ex/VLCD)...
February 20, 2017: Journals of Gerontology. Series A, Biological Sciences and Medical Sciences
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