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Cardiac disease in cystic fibrosis

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https://www.readbyqxmd.com/read/29155436/enzyme-replacement-therapy-for-infantile-onset-pompe-disease
#1
REVIEW
Min Chen, Lingli Zhang, Shuyan Quan
BACKGROUND: Infantile-onset Pompe disease is a rare and progressive autosomal-recessive disorder caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA). Current treatment involves enzyme replacement therapy (with recombinant human alglucosidase alfa) and symptomatic therapies (e.g. to control secretions). Children who are cross-reactive immunological material (CRIM)-negative require immunomodulation prior to commencing enzyme replacement therapy.Enzyme replacement therapy was developed as the most promising therapeutic approach for Pompe disease; however, the evidence is lacking, especially regarding the optimal dose and dose frequency...
November 20, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28856715/feasibility-of-quantitative-regional-ventilation-and-perfusion-mapping-with-phase-resolved-functional-lung-preful-mri-in-healthy-volunteers-and-copd-cteph-and-cf-patients
#2
Andreas Voskrebenzev, Marcel Gutberlet, Filip Klimeš, Till F Kaireit, Christian Schönfeld, Alexander Rotärmel, Frank Wacker, Jens Vogel-Claussen
PURPOSE: In this feasibility study, a phase-resolved functional lung imaging postprocessing method for extraction of dynamic perfusion and ventilation parameters using a conventional 1H lung MRI Fourier decomposition acquisition is introduced. METHODS: Time series of coronal gradient-echo MR images with a temporal resolution of 288 to 324 ms of two healthy volunteers, one patient with chronic thromboembolic hypertension, one patient with cystic fibrosis, and one patient with chronic obstructive pulmonary disease were acquired at 1...
August 30, 2017: Magnetic Resonance in Medicine: Official Journal of the Society of Magnetic Resonance in Medicine
https://www.readbyqxmd.com/read/28693067/-chinese-expert-consensus-for-non-antiinfective-effects-and-clinical-use-of-macrolides
#3
J T Lin, Y M Zhang, X Zhou, C Z Wang, M Huang, C T Liu, C G Wu, H Y Wan, W C Yu, Y R Dai
Important/potential value of macrolides has been proved in the management of chronic respiratory diseases by increasing basic and clinical trials.Through three face-to-face discussions, 10 experts examined important data and drafted this consensus related to macrolides: (1) mechanism of non-antiinfective effects; (2) clinical use in chronic respiratory diseases; (3) cautions of long-term use.The mechanism out of non-antiinfective effects includes anti-inflammatory effect, modifying airway secretion, immune-regulation related to antibacterial effect, corticoid saving effect and anti-viral effect...
July 1, 2017: Zhonghua Nei Ke za Zhi [Chinese Journal of Internal Medicine]
https://www.readbyqxmd.com/read/28548990/outbreaks-of-nontuberculous-mycobacteria
#4
Geeta Sood, Nikki Parrish
PURPOSE OF REVIEW: The purpose of this review is to summarize the emerging literature on nontuberculous mycobacteria outbreaks in healthcare settings. As our ability to identify mycobacterial species develops, we are better able to recognize epidemiologic connections and better understand the prevalence and importance of these outbreaks and pseudo-outbreaks in healthcare settings. RECENT FINDINGS: The number of outbreaks related to nontuberculous outbreaks is increasing because of heightened awareness and better diagnostic tests for species level identification of mycobacteria...
August 2017: Current Opinion in Infectious Diseases
https://www.readbyqxmd.com/read/28314540/technological-advances-shed-light-on-left-ventricular-cardiac-disturbances-in-cystic-fibrosis
#5
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/28006108/digitoxin-for-airway-inflammation-in-cystic-fibrosis-preliminary-assessment-of-safety-pharmacokinetics-and-dose-finding
#6
Pamela L Zeitlin, Marie Diener-West, Karen A Callahan, Seakwoo Lee, C Conover Talbot, Bette Pollard, Michael P Boyle, Noah Lechtzin
RATIONALE: Cystic fibrosis (CF) lung disease progresses by a combination of airway inflammation, bacterial colonization, and infection. Airway inflammation is predominantly neutrophilic and complicates airway clearance therapies through cellular debris; excessive DNA; excessive and viscous mucus; and high concentrations of neutrophils, IL-8, and related cytokines liberated along the nuclear factor-κB signaling pathway. OBJECTIVES: We conducted a preliminary, single-site, randomized, double-blind, placebo-controlled study to evaluate the effects over 28 days of two dose levels (0...
February 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/27966195/beyond-bronchitis-a-review-of-the-congenital-and-acquired-abnormalities-of-the-bronchus
#7
REVIEW
Thomas Marini, Susan K Hobbs, Abhishek Chaturvedi, Kathrine Kaproth-Joslin
Anomalies of the bronchus can be both congenital and acquired. Several different congenital aberrations of the bronchial anatomy are commonly encountered including tracheal bronchus, accessory cardiac bronchus, and bronchial agenesis/aplasia/hypoplasia. In addition, Williams-Campbell syndrome and cystic fibrosis are two other congenital conditions that result in bronchial pathology. Acquired pathology affecting the bronchi can typically be divided into three broad categories of bronchial disease: bronchial wall thickening, dilatation/bronchiectasis, and obstruction/stenosis...
February 2017: Insights Into Imaging
https://www.readbyqxmd.com/read/27780555/relation-of-left-atrial-size-to-atrial-fibrillation-in-patients-aged-%C3%A2-22%C3%A2-years
#8
Douglas Y Mah, Divya Shakti, Kimberlee Gauvreau, Steven D Colan, Mark E Alexander, Dominic J Abrams, David W Brown
Left atrial (LA) dilation has been shown to be associated with atrial fibrillation (AF) in the adult population, with some studies indicating that larger LAs are more prone to AF recurrence. The relation of LA size to AF in the pediatric and young adult population has not been investigated. In this study, all pediatric patients (aged ≤22 years) who presented to Boston Children's Hospital from January 2002 to December 2012 with AF were reviewed. Patients with significant congenital heart disease, cardiomyopathies, proven channelopathies, previous cardiac surgery, end-stage renal disease, or severe lung disease/cystic fibrosis were excluded...
January 1, 2017: American Journal of Cardiology
https://www.readbyqxmd.com/read/27578468/heart-involvement-in-cystic-fibrosis-a-specific-cystic-fibrosis-related-myocardial-changes
#9
REVIEW
Fabien Labombarda, Eric Saloux, Jacques Brouard, Emmanuel Bergot, Paul Milliez
Cystic fibrosis is a complex multi-systemic chronic disease characterized by progressive organ dysfunction with development of fibrosis, possibly affecting the heart. Over the last four decades pathological, experimental, and clinical evidence points towards the existence of a specific myocardial involvement in cystic fibrosis. Multi-modality cardiac imaging, especially recent echocardiographic techniques, evidenced diastolic and/or systolic ventricular dysfunction in cystic fibrosis leading to the concept of a cystic fibrosis-related cardiomyopathy...
September 2016: Respiratory Medicine
https://www.readbyqxmd.com/read/27454104/enzyme-replacement-therapy-for-anderson-fabry-disease
#10
REVIEW
Regina El Dib, Huda Gomaa, Raíssa Pierri Carvalho, Samira E Camargo, Rodrigo Bazan, Pasqual Barretti, Fellype C Barreto
BACKGROUND: Anderson-Fabry disease is an X-linked defect of glycosphingolipid metabolism. Progressive renal insufficiency is a major source of morbidity, additional complications result from cardio- and cerebro-vascular involvement. Survival is reduced among affected males and symptomatic female carriers.This is an update of a Cochrane review first published in 2010, and previously updated in 2013. OBJECTIVES: To evaluate the effectiveness and safety of enzyme replacement therapy compared to other interventions, placebo or no interventions, for treating Anderson-Fabry disease...
July 25, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27357469/incidence-risk-factors-and-prognosis-of-postoperative-atrial-arrhythmias-after-lung-transplantation-a-systematic-review-and-meta-analysis
#11
REVIEW
Jun Fan, Kun Zhou, Shuangjiang Li, Heng Du, Guowei Che
Postoperative atrial arrhythmia is the most common dysrhythmia seen after lung transplantation. However, risk factors for postoperative atrial arrhythmias and their impact on outcomes were inconsistent. The aim of our study was to conduct a meta-analysis to analyse risk factors of postoperative atrial arrhythmias and the impact of postoperative atrial arrhythmias on outcomes after lung transplantation. All eligible articles from MEDLINE, EMBASE, CENTRAL and the Chinese BioMedical Literature Database (until November 2015) were incorporated into this study...
November 2016: Interactive Cardiovascular and Thoracic Surgery
https://www.readbyqxmd.com/read/27238162/-health-status-of-populations-living-in-french-overseas-territories-in-2012-compared-with-metropolitan-france-an-analysis-of-the-national-health-insurance-database
#12
COMPARATIVE STUDY
A Filipovic-Pierucci, A Rigault, A Fagot-Campagna, P Tuppin
BACKGROUND: This study uses healthcare consumption to compare the health status of beneficiaries of the French national health insurance general scheme between individuals living in French overseas territories (FOT) and those living in metropolitan France. METHODS: Data were extracted from the French national health insurance database (Sniiram) for 2012, using algorithms, 56 groups of diseases and 27 groups of hospital activity were isolated. Standardized morbidity ratio for age and sex (SMR) were used to compare FOT to mainland France...
June 2016: Revue D'épidémiologie et de Santé Publique
https://www.readbyqxmd.com/read/27073834/renoprotective-effects-of-dnase-i-treatment-in-a-rat-model-of-ischemia-reperfusion-induced-acute-kidney-injury
#13
Victoria Peer, Ramzia Abu Hamad, Sylvia Berman, Shai Efrati
BACKGROUND: Massive DNA destruction/accumulation of cell-free DNA debris is a sensitive biomarker of progressive organ/tissue damage. Deleterious consequences of DNA debris accumulation are evident in cardiac ischemia, thrombosis, auto-inflammatory diseases, SLE-induced lupus nephritis and cystic fibrosis. In case of renal pathologies, degradation and elimination of DNA debris are suppressed, due to downregulated DNAse-I activity within the diseased kidneys. The aim of the current study was to evaluate whether exogenous DNAse-I administration might exert renoprotective effects in the setting of acute kidney injury (AKI or acute renal failure)...
2016: American Journal of Nephrology
https://www.readbyqxmd.com/read/26943923/enzyme-replacement-therapy-with-galsulfase-for-mucopolysaccharidosis-type-vi
#14
REVIEW
Marcela Junqueira Brunelli, Álvaro N Atallah, Edina M K da Silva
BACKGROUND: Mucopolysaccharidosis type VI or Maroteaux-Lamy syndrome is a rare genetic disorder caused by the deficiency of arylsulphatase B. The resultant accumulation of dermatan sulphate causes lysosomal damage.The clinical symptoms are related to skeletal dysplasia (i.e. short stature and degenerative joint disease). Other manifestations include cardiac disease, impaired pulmonary function, ophthalmological complications, hepatosplenomegaly, sinusitis, otitis, hearing loss and sleep apnea...
2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/26845288/enzyme-replacement-therapy-with-idursulfase-for-mucopolysaccharidosis-type-ii-hunter-syndrome
#15
REVIEW
Edina M K da Silva, Maria Wany Louzada Strufaldi, Regis B Andriolo, Laercio A Silva
BACKGROUND: Mucopolysaccharidosis II, also known as Hunter syndrome, is a rare, X-linked disease caused by a deficiency of the lysosomal enzyme iduronate-2-sulfatase, which catalyses a step in the catabolism of glycosaminoglycans. The glycosaminoglycans accumulate within tissues affecting multiple organs and physiologic systems. The clinical manifestations include neurologic involvement, severe airways obstruction, skeletal deformities and cardiomyopathy. The disease has a variable age of onset and variable rate of progression...
February 5, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/26693372/cystic-fibrosis-in-a-female-infant-with-cardiac-ocular-and-musculoskeletal-anomalies
#16
Azhar Farooqui, Susan Gamal Eldin, Muna Dawood Ali, Ali AlTalhi, Ahmad AlDigheari
Cystic fibrosis (CF) remains the most common hereditary disease in the western population. Its concomitant presence with other congenital abnormalities is a rare phenomenon with very little documentation. In this case report we describe a case of cystic fibrosis in a female infant with cardiac, ocular, and musculoskeletal abnormalities. A brief literature review is also provided.
2015: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/26366639/psychosocial-predictors-of-mortality-following-lung-transplantation
#17
RANDOMIZED CONTROLLED TRIAL
P J Smith, J A Blumenthal, E P Trulock, K E Freedland, R M Carney, R D Davis, B M Hoffman, S M Palmer
Lung transplantation has become an increasingly common treatment for patients with end-stage lung disease. Few studies have examined psychosocial risk factors for mortality in transplant recipients, despite evidence suggesting that elevated levels of negative affect are associated with greater mortality following major cardiac surgery. We therefore examined the relationship between negative affect early after lung transplantation and long-term survival in a sample of 132 lung transplant recipients (28 cystic fibrosis, 64 chronic obstructive pulmonary disease, 26 idiopathic pulmonary fibrosis, 14 other) followed for up to 13...
January 2016: American Journal of Transplantation
https://www.readbyqxmd.com/read/26341519/impaired-cardiac-and-peripheral-hemodynamic-responses-to-inhaled-%C3%AE-%C3%A2-agonist-in-cystic-fibrosis
#18
Erik H Van Iterson, Stephen R Karpen, Sarah E Baker, Courtney M Wheatley, Wayne J Morgan, Eric M Snyder
BACKGROUND: Pulmonary system dysfunction is a hallmark of cystic fibrosis (CF) disease. In addition to impaired cystic fibrosis transmembrane conductance regulator protein, dysfunctional β2-adrenergic receptors (β2AR) contribute to low airway function in CF. Recent observations suggest CF may also be associated with impaired cardiac function that is demonstrated by attenuated cardiac output (Q), stroke volume (SV), and cardiac power (CP) at both rest and during exercise. However, β2AR regulation of cardiac and peripheral vascular tissue, in-vivo, is unknown in CF...
2015: Respiratory Research
https://www.readbyqxmd.com/read/26119592/the-impact-of-cystic-fibrosis-transmembrane-regulator-disruption-on-cardiac-function-and-stress-response
#19
Kai Jiang, Sen Jiao, Megan Vitko, Rebecca Darrah, Chris A Flask, Craig A Hodges, Xin Yu
BACKGROUND: Altered cardiac function has been observed in cystic fibrosis transmembrane regulator (CFTR) knockout mice. However, whether this alteration is a direct effect of CFTR disruption in the heart, or is secondary due to systemic loss of CFTR, remains to be elucidated. METHODS: Cardiac function of mice with muscle-specific or global knockout of CFTR was evaluated at baseline and under β-stimulation by MRI in vivo. Myocyte contractility and Ca2+ transients were measured in vitro...
January 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/25598988/pediatric-myocarditis-a-sentinel-of-non-cardiac-chronic-diseases
#20
Gábor Mogyorósy, Enikő Felszeghy, Tamás Kovács, Andrea Berkes, László Tóth, György Balla, Ilma Korponay-Szabó
INTRODUCTION: Although long-term outcome studies in large pediatric myocarditis/cardiomyopathy populations have been reported in literature, none of them focused on comorbidities. METHODS: All children and adolescents (age <18 years) treated with myocarditis at the Department of Pediatrics, University of Debrecen, Hungary were followed. Patients suffering from myocarditis during the period 1996-2011 were enrolled. RESULTS: Over the 16-year period, a diagnosis of myocarditis was established in nine children...
December 2014: Interventional Medicine & Applied Science
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