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https://www.readbyqxmd.com/read/29444099/airway-ciliary-dysfunction-and-respiratory-symptoms-in-patients-with-transposition-of-the-great-arteries
#1
Maliha Zahid, Abha Bais, Xin Tian, William Devine, Dong Ming Lee, Cyrus Yau, Daniel Sonnenberg, Lee Beerman, Omar Khalifa, Cecilia W Lo
BACKGROUND: Our prior work on congenital heart disease (CHD) with heterotaxy, a birth defect involving randomized left-right patterning, has shown an association of a high prevalence of airway ciliary dysfunction (CD; 18/43 or 42%) with increased respiratory symptoms. Furthermore, heterotaxy patients with ciliary dysfunction were shown to have more postsurgical pulmonary morbidities. These findings are likely a reflection of the common role of motile cilia in both airway clearance and left-right patterning...
2018: PloS One
https://www.readbyqxmd.com/read/29411252/the-fertility-management-experiences-of-australian-women-with-a-non-communicable-chronic-disease-findings-from-the-understanding-fertility-management-in-contemporary-australia-survey
#2
Sara Holton, Aberaami Thananjeyan, Heather Rowe, Maggie Kirkman, Lynne Jordan, Kathleen McNamee, Christine Bayly, John McBain, Vikki Sinnott, Jane Fisher
Introduction Despite the considerable and increasing proportion of women of reproductive age with a chronic non-communicable disease (NCD) and the potential adverse implications of many NCDs for childbearing, little is known about the fertility management experiences of women with an NCD, including their contraceptive use, pregnancy experiences and outcomes, and reproductive health care utilisation. The aim of this study was to investigate the fertility management experiences of women with an NCD and draw comparisons with women without an NCD...
February 6, 2018: Maternal and Child Health Journal
https://www.readbyqxmd.com/read/29143574/investigating-the-preventive-effects-of-baicalin-and-gallocatechin-aginst-glyoxal-induced-cystatin-aggregation
#3
Aamir Sohail, Waseem Feeroze Bhat, Mohammad Furkan, Aaliya Shah, Bilqees Bano
Several mammalian proteins form pathological deposits under nonphysiological conditions that are associated with many degenerative diseases. Protein aggregation is associated with aging, as well as a variety of diseases, including cystic fibrosis, amyotrophic lateral sclerosis (ALS), and hypertrophic cardiomyopathy. There is a lack of any potential anti-amyloidogenic agents and therapeutics till date. Polyphenols have been accredited with myriad biological effects. An analysis of the effects of natural agents like baicalin (BC) and gallocatechin (GC) on aggregation process can open new avenues for the treatment of protein misfolding diseases...
November 16, 2017: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/28828392/thermal-stability-as-a-determinant-of-aav-serotype-identity
#4
Antonette Bennett, Saajan Patel, Mario Mietzsch, Ariana Jose, Bridget Lins-Austin, Jennifer C Yu, Brian Bothner, Robert McKenna, Mavis Agbandje-McKenna
Currently, there are over 150 ongoing clinical trials utilizing adeno-associated viruses (AAVs) to target various genetic diseases, including hemophilia (AAV2 and AAV8), congenital heart failure (AAV1 and AAV6), cystic fibrosis (AAV2), rheumatoid arthritis (AAV2), and Batten disease (AAVrh.10). Prior to patient administration, AAV vectors must have their serotype, concentration, purity, and stability confirmed. Here, we report the application of differential scanning fluorimetry (DSF) as a good manufacturing practice (GMP) capable of determining the melting temperature (Tm) for AAV serotype identification...
September 15, 2017: Molecular Therapy. Methods & Clinical Development
https://www.readbyqxmd.com/read/28653300/defining-the-incidence-and-associated-morbidity-and-mortality-of-severe-respiratory-syncytial-virus-infection-among-children-with-chronic-diseases
#5
REVIEW
Paolo Manzoni, Josep Figueras-Aloy, Eric A F Simões, Paul A Checchia, Brigitte Fauroux, Louis Bont, Bosco Paes, Xavier Carbonell-Estrany
INTRODUCTION: REGAL (RSV Evidence-a Geographical Archive of the Literature) has provided a comprehensive review of the published evidence in the field of respiratory syncytial virus (RSV) in Western countries over the last 20 years. This review covers the risk and burden of RSV infection in children with underlying medical conditions or chronic diseases (excluding prematurity and congenital heart disease). METHODS: A systematic review of publications between January 1, 1995 and December 31, 2015 across PubMed, Embase, The Cochrane Library, and Clinicaltrials...
September 2017: Infectious Diseases and Therapy
https://www.readbyqxmd.com/read/28649564/primary-ciliary-dyskinesia-an-update-on-clinical-aspects-genetics-diagnosis-and-future-treatment-strategies
#6
REVIEW
Virginia Mirra, Claudius Werner, Francesca Santamaria
Primary ciliary dyskinesia (PCD) is an orphan disease (MIM 244400), autosomal recessive inherited, characterized by motile ciliary dysfunction. The estimated prevalence of PCD is 1:10,000 to 1:20,000 live-born children, but true prevalence could be even higher. PCD is characterized by chronic upper and lower respiratory tract disease, infertility/ectopic pregnancy, and situs anomalies, that occur in ≈50% of PCD patients (Kartagener syndrome), and these may be associated with congenital heart abnormalities...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28540046/evaluation-of-a-novel-technique-in-airway-clearance-therapy-specific-cough-technique-sct-in-cystic-fibrosis-a-pilot-study-of-a-series-of-n-of-1-randomised-controlled-trials
#7
Sandra Gursli, Leiv Sandvik, Egil Bakkeheim, Bjørn Skrede, Britt Stuge
OBJECTIVES: The aim of this pilot study was to evaluate the efficacy, safety and participants' perception of a novel technique in airway clearance therapy - specific cough technique in cystic fibrosis. METHODS: We conducted randomised controlled individual trials (N-of-1 randomised controlled trials) in six adults. Each trial included 8 weeks of treatment with two interventions each week, one with specific cough technique and one with forced expiration technique...
2017: SAGE Open Medicine
https://www.readbyqxmd.com/read/28456611/increased-risk-of-ptld-in-lung-transplant-recipients-with-cystic-fibrosis
#8
Erin M Lowery, William Adams, Shellee A Grim, Nina M Clark, Leah Edwards, Jennifer E Layden
BACKGROUND: Post-transplant lymphoproliferative disease (PTLD) is an important cause of morbidity and mortality following lung transplantation. Recipients with cystic fibrosis (CF) may have an increased risk of PTLD although the literature is limited to single center cohorts. Our primary aim is to examine PTLD in an adult lung transplant population by utilizing the International Society for Heart and Lung Transplantation Registry. METHODS: We studied 30,598 adult recipients of lung transplants performed between 1999 and 2011...
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28452739/-experimental-model-and-currency-of-experiment-of-distant-results-of-lead-exposition
#9
G Pataraia, T Bagashvili, G Andronikashvili, T Gurashvili, K Gogeshvili, M Avalishvili
In order to explore the distant results of exposition of little doses of lead, for the objective of the experiment model we have selected 32 mongral rats, of different age, but aged of both sex. Experimental animals were divided in two groups. During first two month from the beginning of the experiment, together with permissible food, animals were given the water, in which was open Pb(NO3)2 - to first group 1.5 mg on kg/weight and to II group 15 mg on kg/weight during the day and night. Before the beginning of the experiment, in the time of process and after it, observation was conducted, description and collection of photo-video materials about the behavior of animals, physiological parameters, possible change of weight, clear-sighted changes in appearance...
February 2017: Georgian Medical News
https://www.readbyqxmd.com/read/28449285/research-review-childhood-chronic-physical-illness-and-adult-emotional-health-a-systematic-review-and-meta-analysis
#10
REVIEW
Ekin Secinti, Ellen J Thompson, Marcus Richards, Darya Gaysina
BACKGROUND: Childhood chronic physical illness is associated with a greater vulnerability for emotional problems (i.e. depression and anxiety) in childhood. However, little is known about life-long effects of childhood chronic physical illness on mental health. The present study aims to systematically review evidence for associations between eight chronic physical illnesses with childhood onset (arthritis, asthma, cancer, chronic renal failure, congenital heart disease, cystic fibrosis, type 1 diabetes, and epilepsy) and adult emotional problems...
July 2017: Journal of Child Psychology and Psychiatry, and Allied Disciplines
https://www.readbyqxmd.com/read/28443234/plastic-bronchitis-an-unusual-complication-of-acute-chest-syndrome-in-adult
#11
Sarah Feray, Pierre Mora, Maxens Decavele, Tài Pham, El Mahdi Hafiani, Muriel Fartoukh
Plastic bronchitis is used to designate endobronchial plugs of rubber-like consistency that form into bronchial trees. It has been described in several diseases like asthma, cystic fibrosis, pulmonary infection, cyanotic congenital heart disease and in few young children with homozygous sickle cell disease. We report the first sickle cell adult case of plastic bronchitis during acute chest syndrome. He developed severe acute respiratory distress syndrome. This unusual presentation related to obstruction by voluminous casts may alert physicians to focus more on the bronchi in sickle cell patients...
2017: Respiratory Medicine Case Reports
https://www.readbyqxmd.com/read/28387447/withdrawn-interventions-for-fatigue-and-weight-loss-in-adults-with-advanced-progressive-illness
#12
REVIEW
Cathy Payne, Philip J Wiffen, Suzanne Martin
BACKGROUND: Fatigue and unintentional weight loss are two of the commonest symptoms experienced by people with advanced progressive illness. Appropriate interventions may bring considerable improvements in function and quality of life to seriously ill people and their families, reducing physical, psychological and spiritual distress. OBJECTIVES: To conduct an overview of the evidence available on the efficacy of interventions used in the management of fatigue and/or unintentional weight loss in adults with advanced progressive illness by reviewing the evidence contained within Cochrane reviews...
April 7, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28366682/indications-for-lung-resection-surgery-and-lung-transplant-in-south-american-children-with-cystic-fibrosis
#13
REVIEW
Fabíola Villac Adde, Sílvia Vidal Campos, Ricardo Henrique de Oliveira Braga Teixeira, Joaquim Carlos Rodrigues
The current available literature evaluating lung resection surgery and lung transplantation in children with cystic fibrosis (CF) was reviewed through a PubMed search and references from selected studies were additionally included. Pulmonary resections, i.e. lobectomy, segmentectomy, and pneumonectomy, are seldom performed in CF. The main indications, in patients with a forced expiratory volume in 1second (FEV 1 ) that is greater than 30% predicted, are localized bronchiectasis/atelectasis, severe hemoptysis, and bronchopleural fistula refractory to medical management...
January 2018: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28364441/survival-after-lung-transplantation-for-cystic-fibrosis-in-sweden
#14
MULTICENTER STUDY
Marita Gilljam, Ulla Nyström, Göran Dellgren, Ingrid Skog, Lennart Hansson
Objectives: In Sweden, lung transplantation has been performed in patients with end-stage lung disease since 1990. We assessed survival after lung transplantation for cystic fibrosis (CF) with focus on early mortality and outcome for patients infected with certain multiresistant bacteria, considered a relative contraindication for lung transplantation. Methods: Review of CF and transplant databases and patient charts. The Kaplan-Meier method and log-rank test were used for survival analysis and group comparison...
March 1, 2017: European Journal of Cardio-thoracic Surgery
https://www.readbyqxmd.com/read/28337699/lung-ultrasound-is-comparable-with-chest-roentgenogram-for-diagnosis-of-community-acquired-pneumonia-in-hospitalised-children
#15
Krishna Kumar Yadav, Shally Awasthi, Anit Parihar
OBJECTIVE: To evaluate the accuracy of lung ultrasound (LUS) in comparison to chest roentgenogram (CXR) in hospitalised children with community-acquired pneumonia (CAP). METHODS: This study was a hospital based prospective observational study, conducted between January 2014 and December 2014. Hospitalised children aged 2 to 59 mo with community-acquired pneumonia were included in the study. The informed written consent was taken from parents (or legal guardian) before recruitment...
July 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28314540/technological-advances-shed-light-on-left-ventricular-cardiac-disturbances-in-cystic-fibrosis
#16
REVIEW
Zahra N Sayyid, Zachary M Sellers
Cystic fibrosis (CF), the most common autosomal recessive lethal disease in Caucasians, causes chronic pulmonary disease and can lead to cor pulmonale with right ventricular dysfunction. The presence of the cystic fibrosis transmembrane conductance regulator (CFTR) in cardiac myocardia has prompted debate regarding possible defective ion channel-induced cardiomyopathy. Clinical heart disease in CF is considered rare and is restricted to case reports. It has been unclear if this is due to the lack of physiological importance of CFTR in the heart, the relatively short lifespan of those with CF, or a technical inability to detect subclinical disease...
July 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28247443/acute-kidney-injury-in-patients-treated-with-iv-beta-lactam-beta-lactamase-inhibitor-combinations
#17
W Cliff Rutter, David S Burgess
STUDY OBJECTIVE: Increased acute kidney injury (AKI) incidence has been reported in patients receiving piperacillin-tazobactam (PTZ) therapy compared with other β-lactams. The authors sought to determine if the addition of β-lactamase inhibitors impacts AKI incidence by comparing patients treated with PTZ or ampicillin-sulbactam (SAM). DESIGN: Retrospective cohort study. SETTING: Large academic tertiary care hospital. PATIENTS: Overall, 2448 patients received PTZ (n=1836) or SAM (n=612) for at least 48 hours between September 1, 2007, and September 30, 2015...
May 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28188010/the-1-min-sit-to-stand-test-in-cystic-fibrosis-insights-into-cardiorespiratory-responses
#18
Thomas Radtke, Helge Hebestreit, Milo A Puhan, Susi Kriemler
BACKGROUND: We aimed to characterize the cardiopulmonary response during a 1-min sit-to-stand (STS) test and compare peak exercise cardiorespiratory variables to a maximal cardiopulmonary exercise test (CPET) in cystic fibrosis (CF). We further aimed to assess the validity of the STS power index (PowerSTS) as a measure of exercise capacity. METHODS: Fifteen adult CF patients performed spirometry, CPET and the 1-min STS test with respiratory gas analysis. RESULTS: Peak-exercise cardiorespiratory variables during the 1-min STS test correlated strongly (r=0...
February 7, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28131631/ryanodine-receptor-modification-and-regulation-by-intracellular-ca-2-and-mg-2-in-healthy-and-failing-human-hearts
#19
K Walweel, P Molenaar, M S Imtiaz, A Denniss, C Dos Remedios, D F van Helden, A F Dulhunty, D R Laver, N A Beard
RATIONALE: Heart failure is a multimodal disorder, of which disrupted Ca(2+) homeostasis is a hallmark. Central to Ca(2+) homeostasis is the major cardiac Ca(2+) release channel - the ryanodine receptor (RyR2) - whose activity is influenced by associated proteins, covalent modification and by Ca(2+) and Mg(2+). That RyR2 is remodelled and its function disturbed in heart failure is well recognized, but poorly understood. OBJECTIVE: To assess Ca(2+) and Mg(2+) regulation of RyR2 from left ventricles of healthy, cystic fibrosis and failing hearts, and to correlate these functional changes with RyR2 modifications and remodelling...
March 2017: Journal of Molecular and Cellular Cardiology
https://www.readbyqxmd.com/read/28125022/pediatric-exercise-testing-value-and-implications-of-peak-oxygen-uptake
#20
REVIEW
Paolo T Pianosi, Robert I Liem, Robert G McMurray, Frank J Cerny, Bareket Falk, Han C G Kemper
Peak oxygen uptake (peak V ˙ O 2 ) measured by clinical exercise testing is the benchmark for aerobic fitness. Aerobic fitness, estimated from maximal treadmill exercise, is a predictor of mortality in adults. Peak V ˙ O 2 was shown to predict longevity in patients aged 7-35 years with cystic fibrosis over 25 years ago. A surge of exercise studies in young adults with congenital heart disease over the past decade has revealed significant prognostic information. Three years ago, the first clinical trial in children with pulmonary arterial hypertension used peak V ˙ O 2 as an endpoint that likewise delivered clinically relevant data...
January 24, 2017: Children
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