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Cystic Fibrosis Sleep

Fidelma Moran, Judy M Bradley, Amanda J Piper
BACKGROUND: Non-invasive ventilation may be a means to temporarily reverse or slow the progression of respiratory failure in cystic fibrosis by providing ventilatory support and avoiding tracheal intubation. Using non-invasive ventilation, in the appropriate situation or individuals, can improve lung mechanics through increasing airflow and gas exchange and decreasing the work of breathing. Non-invasive ventilation thus acts as an external respiratory muscle. This is an update of a previously published review...
February 20, 2017: Cochrane Database of Systematic Reviews
Moya Vandeleur, Lisa M Walter, David S Armstrong, Philip Robinson, Gillian M Nixon, Rosemary S C Horne
OBJECTIVE: To measure sleep patterns and quality, objectively and subjectively, in clinically stable children with cystic fibrosis (CF) and healthy control children, and to examine the relationship between sleep quality and disease severity. STUDY DESIGN: Clinically stable children with CF and healthy control children (7-18 years of age) were recruited. Sleep patterns and quality were measured at home with actigraphy (14 days). Overnight peripheral capillary oxygen saturation was measured via the use of pulse oximetry...
March 2017: Journal of Pediatrics
Bruna Ziegler, Christiano Perin, Fernanda Cano Casarotto, Simone Chaves Fagondes, Sérgio Saldanha Menna-Barreto, Paulo de Tarso Roth Dalcin
BACKGROUND: In cystic fibrosis (CF) patients, end stage of pulmonary disease is characterized by pulmonary hypertension (PH), hypoxemia, decrease in exercise tolerance, and sleep quality. OBJECTIVE: To evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with PH in CF patients aged 16 years or older. METHODS: In a cross-sectional study, 51 clinically stable CF patients underwent a clinical evaluation, an overnight polysomnography and answered sleep questionnaires (Pittsburgh Sleep Quality Index and Epworth sleepiness scale)...
December 7, 2016: Clinical Respiratory Journal
K A Waters, A Lowe, P Cooper, S Vella, Hiran Selvadurai
BACKGROUND: In Cystic Fibrosis (CF), early detection and treatment of respiratory disease is considered the standard for respiratory care. Overnight polysomnography (PSG) may help identify respiratory deterioration in young patients with CF. METHODS: A prospective cohort study of 46 patients with CF, aged 8-12years, from a specialist clinic in a tertiary paediatric hospital. Daytime pulmonary function, shuttle test exercise testing and overnight PSG were studied...
March 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Urte Sommerwerck, Bjoern E Kleibrink, Frederike Kruse, Manuela J Scherer, Yi Wang, Markus Kamler, Helmut Teschler, Gerhard Weinreich
BACKGROUND: The aim of our study was to determine the prevalence and clinical predictors of obstructive sleep apnea in lung transplantation recipients. METHODS: In a cross-sectional analysis we studied 77 clinically stable lung transplant recipients (45 men, time range after lung transplantation [LTX]: from one month to 15 years). Indications for LTX were 36 chronic obstructive lung disease (COPD), 27 idiopathic pulmonary fibrosis (IPF), eight cystic fibrosis, and six others...
May 2016: Sleep Medicine
Jennifer M Allen, Danielle M Graef, Jennifer H Ehrentraut, Brooklee L Tynes, Valerie M Crabtree
BACKGROUND: Sleep disruption is a common comorbidity of pediatric pain. Consequences of pain and disrupted sleep, evidence for the pain-sleep relation, and how aspects of illness, treatment, and pharmacological pain management may contribute to or exacerbate these issues are presented. AIMS: This conceptual review explored the relation between pain and sleep in children diagnosed with chronic medical or developmental conditions. The goal of this review is to expand upon the literature by examining common themes in sleep disturbances associated with painful conditions across multiple pediatric illnesses...
July 15, 2016: CNS Neuroscience & Therapeutics
Rodrigo Strehl Machado, Frederick W Woodley, Beth Skaggs, Carlo Di Lorenzo, Ihuoma Eneli, Mark Splaingard, Hayat Mousa
PURPOSE: This study was performed to evaluate the quality of sleep in snoring obese children without obstructive sleep apnea (OSA); and to study the possible relationship between sleep interruption and gastroesophageal reflux (GER) in snoring obese children. METHODS: Study subjects included 13 snoring obese children who were referred to our sleep lab for possible sleep-disordered breathing. Patients underwent multichannel intraluminal impedance and esophageal pH monitoring with simultaneous polysomnography...
March 2016: Pediatric Gastroenterology, Hepatology & Nutrition
A M Silva, A Descalço, M Salgueiro, L Pereira, C Barreto, T Bandeira, R Ferreira
UNLABELLED: Sleep disturbance has been described in cystic fibrosis (CF) patients as relevant to clinical and lung function predictive factors helping to improve the diagnosis and early intervention. Related paediatric studies are scarce. OBJECTIVE: To describe respiratory sleep disturbance (RSD) and its association with spirometric indices in a population of CF children. A second aim was to determine if spirometric indices and wake-time SpO2 are predictors of sleep disturbance...
July 2016: Revista Portuguesa de Pneumologia
Elisabeth Jameson, Simon Jones, Tracey Remmington
BACKGROUND: Mucopolysaccharidosis type I can be classified as three clinical sub-types; Hurler syndrome, Hurler-Scheie syndrome and Scheie syndrome, with the scale of severity being such that Hurler syndrome is the most severe and Scheie syndrome the least severe. It is a rare, autosomal recessive disorder caused by a deficiency of alpha-L-iduronidase. Deficiency of this enzyme results in the accumulation of glycosaminoglycans within the tissues. The clinical manifestations are facial dysmorphism, hepatosplenomegaly, upper airway obstruction, skeletal deformity and cardiomyopathy...
2016: Cochrane Database of Systematic Reviews
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
Helen E Jo, Tamera J Corte, Stephen J Wort, Neil D Eves, Amanda Piper, Claire Wainwright
No abstract text is available yet for this article.
April 2016: Respirology: Official Journal of the Asian Pacific Society of Respirology
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...
2016: Cochrane Database of Systematic Reviews
Ruth L Dentice, Mark R Elkins, Peter G Middleton, Jennifer R Bishop, Peter A B Wark, Douglas J Dorahy, Christopher J Harmer, Honghua Hu, Peter T P Bye
BACKGROUND: The mucoactive effects of hypertonic saline should promote exacerbation resolution in people with cystic fibrosis (CF). OBJECTIVES: To determine the effects of hypertonic saline inhalation during hospitalisation for exacerbation of CF on length of stay, lung function, symptoms, oxygenation, exercise tolerance, quality of life, bacterial load and time to next hospitalisation. METHODS: 132 adults with an exacerbation of CF were randomised to inhale three nebulised doses a day of either 4 mL 7% saline or a taste-masked control of 0...
February 2016: Thorax
Andrew Turnbull, Ian M Balfour-Lynn
This review highlights important advances in paediatric respiratory medicine since 2014, excluding cystic fibrosis. It focuses mainly on the more common conditions, bronchopulmonary dysplasia, bronchiolitis and preschool wheezing, asthma, pneumonia and sleep, and highlights some of the rarer conditions such as primary ciliary dyskinesia and interstitial lung disease (ILD).
February 2016: Archives of Disease in Childhood
Newton Santos Faria Júnior, Luis Vicente Franco Oliveira, Eduardo Araújo Perez, Ezequiel Fernandes de Oliveira, Nadua Apostolico, Nixon Alves Pereira, Israel dos Reis dos Santos, Jessica Julioti Urbano, Ismael Dias Souza, Igor Bastos Polonio, José Gustavo Barian Romaldini, Déborah Madeu Pereira, Vera Lúcia dos Santos Alves, Ângela Honda Souza, Oliver Augusto Nascimento, José Roberto Jardim, Roberto Stirbulov
INTRODUCTION: Bronchiectasis is a chronic disorder characterised by permanent and irreversible abnormal dilation of the bronchi and bronchioles, primarily caused by repeated cycles of pulmonary infections and inflammation, which lead to reduced mucociliary clearance and to the excessive production of sputum. Patients with non-cystic fibrosis bronchiectasis may be predisposed to hypoxemia during sleep, or to symptoms that may lead to arousals and thereby reduce the quality of life, because of the irreversible dilation of the bronchi and the presence of secretions and airflow obstruction...
2015: BMJ Open
Efstathia M Konstantinidi, Andreas S Lappas, Anna S Tzortzi, Panagiotis K Behrakis
PURPOSE: The aim of this study was to evaluate the 30-year progress of research on exhaled breath condensate in a disease-based approach. METHODS: We searched PubMed/Medline, ScienceDirect, and Google Scholar using the following keywords: exhaled breath condensate (EBC), biomarkers, pH, asthma, gastroesophageal reflux (GERD), smoking, COPD, lung cancer, NSCLC, mechanical ventilation, cystic fibrosis, pulmonary arterial hypertension (PAH), idiopathic pulmonary fibrosis, interstitial lung diseases, obstructive sleep apnea (OSA), and drugs...
2015: TheScientificWorldJournal
Ava Lorenc, Patricia Ronan, Awais Mian, Susan Madge, Siobhan B Carr, Penny Agent, Nicola Robinson
BACKGROUND: Cystic fibrosis (CF) is a genetic disorder affecting respiratory and digestive systems. People with CF experience physical symptoms; cough, poor lung ventilation, recurrent infections, poor weight gain, diarrhoea, and malnutrition, as well as lower quality of life. Tai Chi, a Chinese form of meditative movement, may help with the symptoms of CF and help people with CF to exercise. However, there is very little research in this area. OBJECTIVE: To evaluate the feasibility of studying Tai Chi for CF and to compare the effectiveness of Tai Chi to standard care and face-to-face Tai Chi with online Tai Chi for people with CF...
May 26, 2015: Chinese Journal of Integrative Medicine
Gabriele C Forte, Gabriela C Barni, Christiano Perin, Fernanda C Casarotto, Simone C Fagondes, Paulo de Tarso Roth Dalcin
BACKGROUND: Health-related quality of life (HRQOL) has received much attention in patients with cystic fibrosis (CF). The goal of this study was to evaluate the association between clinical, lung function, sleep quality, and polysomnographic variables with 2 HRQOL questionnaires, the shorter-version World Health Organization Quality of Life (WHOQOL-BREF) and Cystic Fibrosis Quality of Life (CFQOL) questionnaires, in adult subjects with CF. METHODS: In a cross-sectional study, 51 subjects underwent clinical evaluation and overnight polysomnography and answered WHOQOL-BREF, CFQOL, Pittsburgh Sleep Quality Index, and Epworth Sleepiness Scale questionnaires...
October 2015: Respiratory Care
Toby M Maher, Amanda Piper, Yuanlin Song, Marcos I Restrepo, Neil D Eves
No abstract text is available yet for this article.
July 2015: Respirology: Official Journal of the Asian Pacific Society of Respirology
Handan Inönü Köseoğlu, Asiye Kanbay, Oğuz Köktürk
Interstitial lung diseases (ILD), are a group of diseases which can involve pulmonary interstitium, small airways, and vessels, and diffusely affect pulmonary parenchyma as a consequence development of inflammation, and fibrosis leading to respiratory failure, and finally death. Obstructive sleep apnea syndrome (OSAS) is a disorder which courses with its systemic outcomes, and increasing morbidity, and mortality when accompanied with other respiratory system diseases. Concomitancy of OSAS with other lung diseases including chronic obstructive pulmonary disease, asthma, ILD, cystic fibrosis is termed as ''overlap syndrome''...
2014: Tüberküloz Ve Toraks
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