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The heart in cystic fibrosis

Raquel Pinto Carbonera, Fernanda Maria Vendrusculo, Márcio Vinícius Fagundes Donadio
BACKGROUND: Interactive video games are recently being used as an exercise tool in cystic fibrosis (CF). This study aimed to assess the literature describing whether video games generate a physiological response similar to the exercise intensity needed for training in CF. METHODS: An online search in PubMed, Embase, Cochrane, SciELO, LILACS and PEDro databases was conducted and original studies describing physiological responses of the use of video games as exercise in CF were included...
October 2016: Respiratory Medicine
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
Sasiharan Sithamparanathan, Logan Thirugnanasothy, Stephen Clark, John H Dark, Andrew J Fisher, Kate F Gould, Asif Hasan, James L Lordan, Gerard Meachery, Gareth Parry, Paul A Corris
BACKGROUND: Lung transplant recipients have reduced long-term survival compared with other solid organ recipients. There is a lack of published data on the characteristics of very long term survivors. METHODS: We describe the demographics, clinical history and post-procedure function of all lung transplant recipients who have survived greater than 20 years at our centre. RESULTS: At the time of analysis there were 21 (16.4%) of 128 patients who survived over 20 years...
August 2016: Respiratory Medicine
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
Bruno E Balbo, Andressa G Amaral, Jonathan M Fonseca, Isac de Castro, Vera M Salemi, Leandro E Souza, Fernando Dos Santos, Maria C Irigoyen, Feng Qian, Roger Chammas, Luiz F Onuchic
Alterations in myocardial wall texture stand out among ADPKD cardiovascular manifestations in hypertensive and normotensive patients. To elucidate their pathogenesis, we analyzed the cardiac phenotype in Pkd1(cond/cond)Nestin(cre) (CYG+) cystic mice exposed to increased blood pressure, at 5 to 6 and 20 to 24 weeks of age, and Pkd1(+/-) (HTG+) noncystic mice at 5-6 and 10-13 weeks. Echocardiographic analyses revealed decreased myocardial deformation and systolic function in CYG+ and HTG+ mice, as well as diastolic dysfunction in older CYG+ mice, compared to their Pkd1(cond/cond) and Pkd1(+/+) controls...
September 2016: Kidney International
Deepa Raghavan, Ang Gao, Chul Ahn, Vaidehi Kaza, James Finklea, Fernando Torres, Raksha Jain
BACKGROUND: The overall life expectancy of women with cystic fibrosis (CF) is shorter compared with men with CF without accounting for lung transplant recipients. However, it is unclear how donor and recipient gender impact long-term outcomes in patients with CF who undergo lung transplantation. The purpose of this study was to determine if the gender disadvantage seen in women with CF before transplant continues to exist after lung transplant and if this is impacted by donor gender. METHODS: Patients with CF entered in the International Society for Heart and Lung Transplantation registry who were at least 18 years old and received a lung transplant between January 2000 and December 2012 were included and divided into groups based on donor-recipient gender combinations...
June 24, 2016: Journal of Heart and Lung Transplantation
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
Don Hayes, Allan R Glanville, David McGiffin, Joseph D Tobias, Dmitry Tumin
BACKGROUND: A survival advantage has been demonstrated for lung transplantation (LTx) in the cystic fibrosis (CF) population, but children may be at higher risk of post-transplant mortality than adults. METHODS: The registry of the International Society for Heart and Lung Transplantation (ISHLT) was queried for lung transplants performed during the period 1998 to 2014 in patients with CF. Period differences were assessed by dividing the sample into LTxs performed between 2006 and 2014 and those performed between 1998 and 2005...
September 2016: Journal of Heart and Lung Transplantation
Zoe Louise Saynor, Alan Robert Barker, Patrick John Oades, Craig Anthony Williams
PURPOSE: To investigate the effects of mild-to-moderate cystic fibrosis (CF) on the pulmonary oxygen uptake (V˙O2) kinetics of 7 pediatric patients (13.5 ± 2.8 y) versus 7 healthy matched controls (CON; 13.6 ± 2.4 y). We hypothesized that CF would slow the V˙O2 kinetic response at the onset of moderate (MOD) and very heavy (VH) intensity cycling. METHODS: Changes in breath-by-breath V˙O2, near-infrared spectroscopy-derived muscle deoxygenation ([HHb]) at the m...
June 9, 2016: Medicine and Science in Sports and Exercise
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
Jean-Marc Vallier, Mehdi Rouissi, Laurent Mely, Mathieu Gruet
PURPOSE: The modified shuttle test (MST) is increasingly used in clinical practice to assess functional capacity in patients with cystic fibrosis (CF). The purpose of this study was to evaluate the physiological responses of the MST in adults with CF as compared with the gold standard cardiopulmonary exercise test (CPET). METHODS: Participants performed an MST and a CPET on a cycle ergometer in random order. Oxygen (O2) uptake ((Equation is included in full-text article...
July 2016: Journal of Cardiopulmonary Rehabilitation and Prevention
D C D Hope, J M Palan
A 44-year-old woman, with a background of heart, lung and renal transplantation secondary to cystic fibrosis and type 1 diabetes, presented with tachycardia, hyperglycaemia, nausea and vomiting. She was initially managed for diabetic ketoacidosis with severe dehydration. However, persistent episodic hypertension and tachycardia led the investigating team to identify significantly raised urinary metanephrines and a left-sided adrenal mass; Iodine-123-meta-iodobenzylguanidine single photon emission computer tomography scan (MIBG SPECT/CT) showed avid uptake of tracer, confirming a left-sided phaeochromocytoma...
2016: BMJ Case Reports
Nanako Ushio, James K Chambers, Kennichi Watanabe, Takuya E Kishimoto, Jun-You Li, Hiroyuki Nakayama, Kazuyuki Uchida
A 7-year-old Duroc sow exhibited emaciation, loss of appetite and rapid breathing, and was euthanized. Histopathological examination revealed mild to moderate fibrosis of the heart, cystic kidneys and ulcerative enteritis associated with Balantidium infection. Additionally, a small nodule was incidentally found in the peripancreatic fat tissue. The nodule consisted of disarranged cellular components: pancreatic islet cells (either insulin-, glucagon- or somatostatin-positive), pancreatic acinar cells, hepatocytes (human hepatocyte-positive) and ductal cells (cytokeratin 19-positive)...
September 1, 2016: Journal of Veterinary Medical Science
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
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