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Heart disease in cystic fibrosis

A E While, E Heery, A M Sheehan, I Coyne
BACKGROUND: The numbers of children with long-term illnesses surviving into adulthood and transferring from child to adult services has increased dramatically in the last 30 years. This study aimed to examine health-related quality of life pre- and post-transfer from child to adult healthcare for young people with three long-term illnesses. METHODS: A total of 217 young people with cystic fibrosis, congenital heart defects or diabetes attending child and adult hospital services in Dublin, Ireland completed a questionnaire survey...
September 27, 2016: Child: Care, Health and Development
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
David J Roach, Yannick Crémillieux, Robert J Fleck, Alan S Brody, Suraj D Serai, Rhonda D Szczesniak, Stephanie Kerlakian, John P Clancy, Jason C Woods
RATIONALE: Recent advancements in magnetic resonance imaging (MRI) have been made that improve our ability to assess pulmonary structure and function in cystic fibrosis (CF) patients. A non-ionizing imaging modality that can be used as a serial monitoring tool throughout life can positively impact patient care and outcomes. OBJECTIVES: The purpose of this study was to compare a new ultrashort echo-time (UTE) MRI method with computed tomography (CT) as a biomarker of lung structure abnormalities in young children with early CF lung disease...
August 23, 2016: Annals of the American Thoracic Society
Laura L Walkup, Robert P Thomen, Teckla G Akinyi, Erin Watters, Kai Ruppert, John P Clancy, Jason C Woods, Zackary I Cleveland
BACKGROUND: Hyperpolarized (129)Xe is a promising contrast agent for MRI of pediatric lung function, but its safety and tolerability in children have not been rigorously assessed. OBJECTIVE: To assess the feasibility, safety and tolerability of hyperpolarized (129)Xe gas as an inhaled contrast agent for pediatric pulmonary MRI in healthy control subjects and in children with cystic fibrosis. MATERIALS AND METHODS: Seventeen healthy control subjects (ages 6-15 years, 11 boys) and 11 children with cystic fibrosis (ages 8-16 years, 4 boys) underwent (129)Xe MRI, receiving up to three doses of (129)Xe gas prepared by either a commercially available or a homebuilt (129)Xe polarizer...
August 5, 2016: Pediatric Radiology
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...
2016: Cochrane Database of Systematic Reviews
Gregory Reychler, Margaux Debatisse, Patrick Lebecque, Thierry Pieters, Giuseppe Liistro, Sophie Gohy
BACKGROUND: Recently, gait speed reached an increasing importance in the management of respiratory patients. The aim of this retrospective study was to compare walking speed and physiological adaptations during the 6MWT in COPD and CF patients. METHODS: 6MWT performed by COPD and CF patients were retrospectively reviewed. Global and sequential walking speeds were measured on six minutes and every sequence of two minutes respectively. Heart rate, oxygen saturation and dyspnea were analyzed...
September 2016: Gait & Posture
J Michael Wells, Roopan F Farris, Taylor A Gosdin, Mark T Dransfield, Michelle E Wood, Scott C Bell, Steven M Rowe
BACKGROUND: Acute pulmonary exacerbations are associated with progressive lung function decline and increased mortality in cystic fibrosis. The role of pulmonary vascular disease in pulmonary exacerbations is unknown. We aimed to assess the association between pulmonary artery enlargement (defined as pulmonary artery diameter to ascending aorta diameter [PA:A] ratio >1), a marker of pulmonary vascular disease, and exacerbations. METHODS: In this cohort study, we used clinical, CT imaging, and prospective exacerbation data from a previous prospective clinical trial (derivation cohort) and from The Prince Charles Hospital (TPCH; Brisbane, QLD, Australia) cystic fibrosis registry (validation cohort)...
August 2016: Lancet Respiratory Medicine
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
Le Thi Thanh Thuy, Tuong Thi Van Thuy, Yoshinari Matsumoto, Hoang Hai, Yoshihiro Ikura, Katsutoshi Yoshizato, Norifumi Kawada
Cytoglobin (Cygb) was identified in hepatic stellate cells (HSCs) and pericytes of all organs; however, the effects of Cygb on cellular functions remain unclear. Here, we report spontaneous and age-dependent malformations in multiple organs of Cygb(-/-) mice. Twenty-six percent of young Cygb(-/-) mice (<1 year old) showed heart hypertrophy, cystic disease in the kidney or ovary, loss of balance, liver fibrosis and lymphoma. Furthermore, 71.3% (82/115) of aged Cygb(-/-) mice (1-2 years old) exhibited abnormalities, such as heart hypertrophy and cancer development in multiple organs; by contrast, 5...
2016: Scientific Reports
Giovanni Ribaudo, Mario Angelo Pagano, Sergio Bova, Giuseppe Zagotto
BACKGROUND: Phosphodiesterase 5 inhibitors (PDE5-Is) sildenafil, vardenafil, tadalafil and the recently approved avanafil represent the first-line choice for both on-demand and chronic treatment of erectile dysfunction (ED). In addition to this, sildenafil and tadalafil, have also been approved for the treatment of pulmonary arterial hypertension. Due to its expression and localization in many tissues, PDE5 and its regulation has been reported to be involved in several other diseases...
2016: Current Medicinal Chemistry
Alun C Jackson, Rachel P-T Liang, Erica Frydenberg, Rosemary O Higgins, Barbara M Murphy
AIMS AND OBJECTIVES: The aim of this review was to examine parent education programmes for families with children with special health care needs, to better design interventions focusing on the psychosocial aspects of living with a child's chronic condition. BACKGROUND: Studies of familial coping with children with special health care needs indicate high levels of parenting stress, with families with children with special health care needs at risk of major psychological and social disturbances and financial strain...
June 2016: Journal of Clinical Nursing
Laurens J Ceulemans, Sébastien Strypstein, Arne Neyrinck, Stijn Verleden, David Ruttens, Diethard Monbaliu, Paul De Leyn, Johan Vanhaecke, Bart Meyns, Frederik Nevens, Geert Verleden, Dirk Van Raemdonck, Jacques Pirenne
Combined liver/thoracic transplantation (cLiThTx) is a complex procedure for end-stage/advanced liver and heart(H)/lung(Lu) disease. To avoid futile use of multiple organs in single recipients, results should be scrutinously analyzed. Single-center cLiThTx (04/2000-12/2015) were reviewed for the following: demographics, indications, surgical technique, complications, rejection, and five-year patient survival. Results are reported as median (range). Fourteen consecutive patients underwent cLiThTx: 3 cLiHTx, 10 cLiLuTx, and 1 cLiHLuTx...
June 2016: Transplant International: Official Journal of the European Society for Organ Transplantation
Amparo Solé, Inés Pérez, Isabel Vázquez, Amparo Pastor, Juan Escrivá, Gabriel Sales, David Hervás, Allan R Glanville, Alexandra L Quittner
BACKGROUND: Despite well-known risk factors and predictive survival models, many patients with cystic fibrosis (CF) die while on the waiting list for lung transplant. We evaluated whether specific Cystic Fibrosis Questionnaire (CFQ-R) scales provide additional benefit to conventional tools in identifying referral timing and waitlist mortality. METHODS: From January 2010 to January 2015, 152 patients (34% on the waitlist) were evaluated with the CFQ-R and standard protocol quarterly...
June 2016: Journal of Heart and Lung Transplantation
O Göhl, D J Walker, S Walterspacher, D Langer, C M Spengler, T Wanke, M Petrovic, R-H Zwick, S Stieglitz, R Glöckl, D Dellweg, H-J Kabitz
Specific respiratory muscle training (IMT) improves the function of the inspiratory muscles. According to literature and clinical experience, there are 3 established methods: 1.) resistive load 2.) threshold load and 3.) normocapnic hyperpnea. Each training method and the associated devices have specific characteristics. Setting up an IMT should start with specific diagnostics of respiratory muscle function and be followed by detailed individual introduction to training. The aim of this review is to take a closer look at the different training methods for the most relevant indications and to discuss these results in the context of current literature...
January 2016: Pneumologie
Priscila Cilene León Bueno de Camargo, Ricardo Henrique de Oliveira Braga Teixeira, Rafael Medeiros Carraro, Silvia Vidal Campos, José Eduardo Afonso Junior, André Nathan Costa, Lucas Matos Fernandes, Luis Gustavo Abdalla, Marcos Naoyuki Samano, Paulo Manuel Pêgo-Fernandes
Lung transplantation is a well-established treatment for patients with advanced lung disease. The evaluation of a candidate for transplantation is a complex task and involves a multidisciplinary team that follows the patient beyond the postoperative period. Currently, the mean time on the waiting list for lung transplantation in the state of São Paulo, Brazil, is approximately 18 months. For Brazil as a whole, data from the Brazilian Organ Transplant Association show that, in 2014, there were 67 lung transplants and 204 patients on the waiting list for lung transplantation...
November 2015: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
Mandy E Spoorenberg, Marieke H A H van den Oord, Ted Meeuwsen, Tim Takken
BACKGROUND: During commercial air travel passengers are exposed to a low ambient cabin pressure, comparable to altitudes of 5000 to 8000 ft (1524 to 2438 m). In healthy passengers this causes a fall in partial pressure of oxygen, which results in relative hypoxemia, usually without symptoms. Patients with congenital heart or lung disease may experience more severe hypoxemia during air travel. This systematic review provides an overview of the current literature focusing on whether it is safe for patients with congenital heart or lung disease to fly...
January 2016: Aerospace Medicine and Human Performance
B K Pedersen, B Saltin
This review provides the reader with the up-to-date evidence-based basis for prescribing exercise as medicine in the treatment of 26 different diseases: psychiatric diseases (depression, anxiety, stress, schizophrenia); neurological diseases (dementia, Parkinson's disease, multiple sclerosis); metabolic diseases (obesity, hyperlipidemia, metabolic syndrome, polycystic ovarian syndrome, type 2 diabetes, type 1 diabetes); cardiovascular diseases (hypertension, coronary heart disease, heart failure, cerebral apoplexy, and claudication intermittent); pulmonary diseases (chronic obstructive pulmonary disease, asthma, cystic fibrosis); musculo-skeletal disorders (osteoarthritis, osteoporosis, back pain, rheumatoid arthritis); and cancer...
December 2015: Scandinavian Journal of Medicine & Science in Sports
Kirk Kee, Christopher Stuart-Andrews, Kris Nilsen, Jeremy P Wrobel, Bruce R Thompson, Matthew T Naughton
In the healthy lung, ventilation is distributed heterogeneously due to factors such as anatomical asymmetry and gravity. This ventilation heterogeneity increases pathologically in conditions such as asthma, chronic obstructive lung disease, and cystic fibrosis. In chronic heart failure, lung biopsy demonstrates evidence of peripheral lung fibrosis and small airways narrowing and distortion. We hypothesized that this would lead to increased ventilation heterogeneity. Furthermore, we proposed that rostral fluid shifts when seated patients lie supine would further increase ventilation heterogeneity...
October 2015: Physiological Reports
Igho J Onakpoya, Gail Hayward, Carl J Heneghan
BACKGROUND: Lower respiratory tract infections (LRTIs) in young children account for 1.4 million deaths annually worldwide. Antibiotics could be beneficial in preventing LRTIs in high-risk children, and may also help prevent school absenteeism and work days missed by children and/or carers. While it is well documented that the efficacy of antibiotic prophylaxis for RTIs decreases over time, there are no reviews that describe the use of antibiotic prophylaxis to prevent LRTIs in high-risk children aged 12 years and under...
2015: Cochrane Database of Systematic Reviews
H Morisse-Pradier, R Nove-Josserand, F Philit, A Senechal, F Berger, E Callet-Bauchu, A Traverse-Glehen, J-M Maury, R Grima, F Tronc, J-F Mornex
Graft-versus-host disease (GVHD) is a classic and frequent multisystemic complication of bone marrow allografts. It has also been reported after the transplantation of solid organs such as the liver or gut. Recent cases of GVHD have been reported after lung and heart-lung transplant. Skin, liver, gastrointestinal tract and bone marrow are the organ preferentially affected by GVHD. Corticosteroid is the first line treatment of GVHD. The prognosis reported in solid organ transplants is poor with infectious complications favoured by immunosuppressive therapy...
February 2016: Revue de Pneumologie Clinique
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