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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/28323913/accrual-of-bone-mass-in-children-and-adolescents-with-cystic-fibrosis
#1
Sonakshi Sharma, Mirjana Jaksic, Sheryl Fenwick, Catherine Byrnes, Tim Cundy
Context: Low bone density is a recognised complication of cystic fibrosis (CF). Hypothesis: Accrual of bone mass is most impaired in the sickest children, as judged by nutritional status and pulmonary function. Design: Retrospective analysis of correlation between lumbar spine BMD, BMI and FEV1 z-score in children and adolescents with CF. Setting: Specialist CF service at a pediatric hospital. Patients: 60 participants aged 5...
February 22, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/28320633/nutritional-status-nutrient-intake-and-use-of-enzyme-supplements-in-paediatric-patients-with-cystic-fibrosis-a-european-multicentre-study-with-reference-to-current-guidelines
#2
Joaquim Calvo-Lerma, Jessie M Hulst, Inês Asseiceira, Ine Claes, Maria Garriga, Carla Colombo, Victoria Fornés, Sandra Woodcock, Tiago Martins, Mieke Boon, Mar Ruperto, Sylvia Walet, Chiara Speziali, Peter Witters, Etna Masip, Celeste Barreto, Kris de Boeck, Carmen Ribes-Koninckx
BACKGROUND: The New European guidelines have established the most updated recommendations on nutrition and pancreatic enzyme replacement therapy (PERT) in CF. In the context of MyCyFAPP project - a European study in children with CF aimed at developing specific tools for improvement of self-management - the objective of the current study was to assess nutritional status, daily energy and macronutrient intake, and PERT dosing with reference to these new guidelines. METHODS: Cross sectional study in paediatric patients with CF from 6 European centres...
March 17, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28302638/innovative-approach-for-self-management-and-social-welfare-of-children-with-cystic-fibrosis-in-europe-development-validation-and-implementation-of-an-mhealth-tool-mycyfapp
#3
Joaquim Calvo-Lerma, Celia P Martinez-Jimenez, Juan-Pablo Lázaro-Ramos, Ana Andrés, Paula Crespo-Escobar, Erlend Stav, Cornelia Schauber, Lucia Pannese, Jessie M Hulst, Lucrecia Suárez, Carla Colombo, Celeste Barreto, Kris de Boeck, Carmen Ribes-Koninckx
INTRODUCTION: For the optimal management of children with cystic fibrosis, there are currently no efficient tools for the precise adjustment of pancreatic enzyme replacement therapy, either for advice on appropriate dietary intake or for achieving an optimal nutrition status. Therefore, we aim to develop a mobile application that ensures a successful nutritional therapy in children with cystic fibrosis. METHODS AND ANALYSIS: A multidisciplinary team of 12 partners coordinate their efforts in 9 work packages that cover the entire so-called 'from laboratory to market' approach by means of an original and innovative co-design process...
March 16, 2017: BMJ Open
https://www.readbyqxmd.com/read/28262916/vitamin-e-supplementation-in-people-with-cystic-fibrosis
#4
REVIEW
Peter O Okebukola, Sonal Kansra, Joanne Barrett
BACKGROUND: People with cystic fibrosis are at an increased risk of fat-soluble vitamin deficiency including vitamin E. Vitamin E deficiency can cause a host of conditions such as haemolytic anaemia, cerebellar ataxia and cognitive difficulties. Vitamin E supplementation is widely recommended in cystic fibrosis and aims to ameliorate this deficiency. This is an updated version of the review. OBJECTIVES: To determine the effects of any level of vitamin E supplementation on the frequency of vitamin E deficiency disorders in people with cystic fibrosis...
March 6, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28262570/peripheral-muscle-abnormalities-in-cystic-fibrosis-etiology-clinical-implications-and-response-to-therapeutic-interventions
#5
Mathieu Gruet, Thierry Troosters, Samuel Verges
Peripheral muscle dysfunction is an important systemic consequence of cystic fibrosis (CF) with major clinical implications, such as exercise intolerance and reduced quality of life. Evidence is now accumulating that lack of physical activity is unlikely to be the sole explanation for peripheral muscle dysfunction of patients with CF. Particularly, the demonstration of CFTR expression in both murine and human skeletal muscle suggests the potential implication of intrinsic CF-related factors. By combining data from both human and animal models, this review describes CF peripheral muscle abnormalities and critically reviews the advances in understanding the impact of the underlying mechanisms...
March 2, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28230293/influence-of-complex-childhood-diseases-on-variation-in-growth-and-skeletal-development
#6
REVIEW
Babette S Zemel
The study of human growth and skeletal development by human biologists is framed by the larger theoretical concerns regarding the underpinnings of population variation and human evolution. This unique perspective is directly relevant to the assessment of child health and well-being at the individual and group level, as well as the construction of growth charts. Environmental, behavioral (nutrition and physical activity), and disease-related factors can prevent attainment of full genetic potential for growth...
February 23, 2017: American Journal of Human Biology: the Official Journal of the Human Biology Council
https://www.readbyqxmd.com/read/28185886/macronutrient-intake-in-preschoolers-with-cystic-fibrosis-and-the-relationship-between-macronutrients-and-growth
#7
Stephanie S Filigno, Shannon M Robson, Rhonda D Szczesniak, Leigh A Chamberlin, Meredith A Baker, Stephanie M Sullivan, John Kroner, Scott W Powers
BACKGROUND: Adequate nutrition is essential for growth in children with cystic fibrosis (CF). The new CF Foundation Clinical Practice Guidelines bring attention to monitoring macronutrient intake as well as total energy. METHODS: Dietary intake of 75 preschool children with CF and pancreatic insufficiency was examined and compared to the Clinical Practice Guidelines. Regression analyses examined relationships between macronutrient intake and growth. RESULTS: Approximately 45% of children met the 110% minimum recommended dietary allowance (RDA) recommendation...
February 6, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28184354/gallium-protoporphyrin-ix-inhibits-pseudomonas-aeruginosa-growth-by-targeting-cytochromes
#8
Sarah Hijazi, Paolo Visca, Emanuela Frangipani
Pseudomonas aeruginosa is a challenging pathogen due to both innate and acquired resistance to antibiotics. It is capable of causing a variety of infections, including chronic lung infection in cystic fibrosis (CF) patients. Given the importance of iron in bacterial physiology and pathogenicity, iron-uptake and metabolism have become attractive targets for the development of new antibacterial compounds. P. aeruginosa can acquire iron from a variety of sources to fulfill its nutritional requirements both in the environment and in the infected host...
2017: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/28178425/synthetic-cystic-fibrosis-sputum-medium-diminishes-i-burkholderia-cenocepacia-i-antifungal-activity-against-i-aspergillus-fumigatus-i-independently-of-phenylacetic-acid-production
#9
Tasia Joy Lightly, Ryan R Phung, John L Sorensen, Silvia T Cardona
Phenylacetic acid (PAA), an intermediate of phenylalanine degradation, is emerging as a signal molecule in microbial interactions with the host. In this work, we explore the presence of phenylalanine and PAA catabolism in three microbial pathogens of the cystic fibrosis (CF) lung microbiome: <i>Pseudomonas aeruginosa</i>, <i>Burkholderia cenocepacia</i> and <i>Aspergillus fumigatus</i>. While <i>in silico</i> analysis of <i>B. cenocepacia</i> J2315 and <i>A...
February 8, 2017: Canadian Journal of Microbiology
https://www.readbyqxmd.com/read/28155088/the-enigmatic-gut-in-cystic-fibrosis-linking-inflammation-dysbiosis-and-the-increased-risk-of-malignancy
#10
REVIEW
Millie Garg, Chee Y Ooi
PURPOSE OF REVIEW: Intestinal inflammation, dysbiosis, and increased gastrointestinal malignancy risks are well-described in patients with cystic fibrosis (CF). However, there is limited understanding of their pathophysiology. This review aims to discuss these issues and assess potential links between them. RECENT FINDINGS: Evidence of links between intestinal inflammation and dysbiosis (an imbalance in intestinal microbial populations) exist. Recent studies have demonstrated reduction in intestinal inflammation with probiotic administration...
February 2017: Current Gastroenterology Reports
https://www.readbyqxmd.com/read/28134234/-the-importance-of-a-well-balanced-diet-in-the-comprehensive-treatment-of-cystic-fobrosis
#11
REVIEW
Karolina Brzostek, Anna Ukleja
Cystic fibrosis (CF) is a genetic disease characterized by formation and accumulation of sticky, thick slime in human's organs. It runs to impairment of lungs functioning that results in development of higher risk of malnutrition and chronic food deficiencies. One of the elements of CF's treatment is nutrition therapy including properly composed diet considering accelerated demand for energy, proteins and lipids. The replenishment of nutrition treatment is pancreatic enzymes and fat-soluble vitamins supplementation...
January 23, 2017: Polski Merkuriusz Lekarski: Organ Polskiego Towarzystwa Lekarskiego
https://www.readbyqxmd.com/read/28132571/the-diagnosis-and-management-of-respiratory-viral-infections-in-cystic-fibrosis
#12
William Flight, Andrew Jones
Respiratory viruses, such as those that cause influenza and the common cold, are a regular feature of life for the entire human population. Among people with CF, these viruses are associated with prolonged respiratory illness and show a clear association with pulmonary exacerbations which in turn are associated with lung function decline and risk of death. Human rhinovirus is the most commonly encountered respiratory viral pathogen in CF although adenovirus, bocavirus, coronavirus, influenza, parainfluenza, metapneumovirus and respiratory syncytial virus are all also responsible for infections in this population...
February 3, 2017: Expert Review of Respiratory Medicine
https://www.readbyqxmd.com/read/28114922/a-prospective-pilot-study-of-home-monitoring-in-adults-with-cystic-fibrosis-home-cf-protocol-for-a-randomised-controlled-trial
#13
Jocelyn Choyce, Karen L Shaw, Alice J Sitch, Hema Mistry, Joanna L Whitehouse, Edward F Nash
BACKGROUND: Home monitoring has the potential to detect early pulmonary exacerbations in people with cystic fibrosis (CF), with consequent improvements in health outcomes and healthcare associated costs. This study aims to assess the effects of home monitoring on hospital admissions, quality of life, antibiotic requirements, exacerbation frequency, lung function, nutritional outcomes, anxiety, depression, costs and health outcomes, as well as the qualitative effects on the patient experience...
January 23, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28093464/socioeconomic-status-smoke-exposure-and-health-outcomes-in-young-children-with-cystic-fibrosis
#14
Thida Ong, Michael Schechter, Jing Yang, Limin Peng, Julia Emerson, Ronald L Gibson, Wayne Morgan, Margaret Rosenfeld
BACKGROUND: Lower socioeconomic status (SES) and environmental tobacco smoke (ETS) exposure are both associated with poorer disease outcomes in cystic fibrosis (CF), and children with low SES are disproportionately exposed to ETS. We analyzed a large cohort of young children with CF to distinguish the impact of SES and ETS on clinical outcomes. METHODS: The Early Pseudomonas Infection Control Observational study enrolled Pseudomonas-negative young children with CF <13 years of age...
February 2017: Pediatrics
https://www.readbyqxmd.com/read/28086849/successful-outcome-following-pneumonectomy-in-a-teenage-boy-with-cystic-fibrosis-a-case-report
#15
Zheyi Liew, Santosh Mallikarjuna, Asif Hasan, F Kate Gould, Su Bunn, Matthew F Thomas, Jim L Lordan, Christopher O'Brien, Malcolm Brodlie
BACKGROUND: Cystic fibrosis lung disease is generally a diffuse process however rarely one lung may become particularly damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relative sparing of the contralateral lung. This clinical situation is sometimes referred to as "destroyed lung". Lung resection surgery is seldom indicated in cystic fibrosis and the associated medical literature is relatively sparse. CASE PRESENTATION: A 14 year old boy was referred to our centre for lung transplantation assessment...
January 13, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28078087/clostridium-difficile-infection-in-cystic-fibrosis-an-uncommon-but-life-threatening-complication
#16
Francesco Piccolo, Anna Sze Tai, Hooi Ee, Siobhain Mulrennan, Scott Bell, Gerard Ryan
Adults with cystic fibrosis (CF) have significant rates of asymptomatic Clostridium difficile carriage and are frequently exposed to risk factors for C. difficile infection (CDI). Despite this, the rate of reported CDI in CF is low. We describe three cases of near fatal CDI in adults with CF and review the literature regarding presentation, management, and recurrence prevention. Early recognition is important as the clinical presentation may be atypical and the illness can be severe and even life-threatening...
January 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28052955/hypoxia-and-sterile-inflammation-in-cystic-fibrosis-airways-mechanisms-and-potential-therapies
#17
REVIEW
Samuel T Montgomery, Marcus A Mall, Anthony Kicic, Stephen M Stick
Cystic fibrosis is one of the most common autosomal recessive genetic diseases in Caucasian populations. Diagnosis via newborn screening and targeted nutritional and antibiotic therapy have improved outcomes, however respiratory failure remains the key cause of morbidity and mortality. Progressive respiratory disease in cystic fibrosis is characterised by chronic neutrophilic airway inflammation associated with structural airway damage leading to bronchiectasis and decreased lung function. Mucus obstruction is a characteristic early abnormality in the cystic fibrosis airway, associated with neutrophilic inflammation often in the absence of detectable infection...
January 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28049743/alternative-indexes-to-estimate-the-functional-capacity-from-the-6-minute-walk-test-in-children-and-adolescents-with-cystic-fibrosis
#18
Renata Tiemi Okuro, Maria Angela Gonçalves de Oliveira Ribeiro, José Dirceu Ribeiro, Rafaela Coelho Minsky, Camila Isabel Santos Schivinski
BACKGROUND: Cystic fibrosis is a multi-systemic disease related to reduced functional capacity. The distance covered in the 6-min walk test (6MWT) has been known to assess functional capacity, but little is known about other indexes that can be derived. We sought to compare the performance during the 6MWT and the estimated indexes of functional capacity from the 6MWT between subjects with cystic fibrosis (CF) and healthy individuals as well as to assess the relationship among these indexes and disease severity, pulmonary function, and nutritional status in CF...
January 3, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28000919/antibiotic-treatment-for-nontuberculous-mycobacteria-lung-infection-in-people-with-cystic-fibrosis
#19
REVIEW
Valerie Waters, Felix Ratjen
BACKGROUND: Nontuberculous mycobacteria are mycobacteria, other than those in the Mycobacterium tuberculosis complex, and are commonly found in the environment. Nontuberculous mycobacteria species (most commonly Mycobacterium avium complex and Mycobacterium abscessus) are isolated from the respiratory tract of approximately 5% to 40% of individuals with cystic fibrosis; they can cause lung disease in people with cystic fibrosis leading to more a rapid decline in lung function and even death in certain circumstances...
December 19, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27966575/clinical-impact-of-vitamin-d-treatment-in-cystic-fibrosis-a-pilot-randomized-controlled-trial
#20
T Pincikova, D Paquin-Proulx, J K Sandberg, M Flodström-Tullberg, L Hjelte
BACKGROUND/OBJECTIVES: Vitamin D insufficiency in cystic fibrosis is common. Vitamin D3 is currently preferred over D2. We aimed to study the efficacy of vitamin D2 and D3 at increasing serum 25-hydroxyvitamin D (s25OHD) concentrations and their effect on respiratory health in cystic fibrosis. SUBJECTS/METHODS: Sixteen CF patients were randomized to receive vitamin D2 or D3 or to serve as controls. The starting dose of 5000 IU (<16 years old) or 7143 IU/day (⩾16 years old) was further individually adjusted...
February 2017: European Journal of Clinical Nutrition
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