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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/28230293/influence-of-complex-childhood-diseases-on-variation-in-growth-and-skeletal-development
#1
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
#2
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
#3
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
#4
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
#5
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
#6
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
#7
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
#8
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
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...
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
#15
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
https://www.readbyqxmd.com/read/27922234/long-term-azithromycin-therapy-in-patients-with-cystic-fibrosis
#16
Nagehan Emiralioğlu, Zeynelabidin Öztürk, Ebru Yalçın, Deniz Doğru, Uğur Özçelik, Nural Kiper
Inflammation is a central contributor to the pathogenesis of cystic fibrosis (CF) pulmonary disease; so limiting the excessive production of inflammatory mediators represents a major therapeutic strategy for slowing the decline in lung function and improving survival. The macrolide antibiotic azithromycin (AZM) has anti-inflammatory properties and immunomodulatory effects that may be beneficial in CF. The aim of this study was to document the long term use of AZM effect on pulmonary function, nutritional status and number of pulmonary exacerbations in patients with CF...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27916550/bmi-fails-to-identify-poor-nutritional-status-in-stunted-children-with-cf
#17
Michael W Konstan, David J Pasta, Jeffrey S Wagener, Donald R VanDevanter, Wayne J Morgan
BACKGROUND: Body mass index (BMI) is currently emphasized for evaluating nutritional status in children with cystic fibrosis (CF). Weight for age (WFA) and height for age (HFA) may get less attention. METHODS: Data from the Epidemiologic Study of Cystic Fibrosis were used to compare patient WFA, HFA, and BMI percentiles for children age 2 to 18years. RESULTS: For children with BMI between the 25th and 50th percentiles, 16.8% had WFA <10th percentile and 26...
December 1, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27878805/pancreatic-enzyme-replacement-therapy-for-people-with-cystic-fibrosis
#18
REVIEW
Usha Rani Somaraju, Arturo Solis-Moya
BACKGROUND: Most people with cystic fibrosis (80% to 90%) need pancreatic enzyme replacement therapy to prevent malnutrition. Enzyme preparations need to be taken whenever food is taken, and the dose needs to be adjusted according to the food consumed. A systematic review on the efficacy and safety of pancreatic enzyme replacement therapy is needed to guide clinical practice, as there is variability between centres with respect to assessment of pancreatic function, time of commencing treatment, dose and choice of supplements...
23, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27873431/hypoglycemia-is-common-in-children-with-cystic-fibrosis-and-seen-predominantly-in-females
#19
Belma Haliloglu, Yasemin Gokdemir, Zeynep Atay, Saygin Abali, Tulay Guran, Fazilet Karakoc, Refika Ersu, Bulent Karadag, Serap Turan, Abdullah Bereket
OBJECTIVE: To determine the prevalence of hypoglycemia in children and adolescents with cystic fibrosis (CF) in 2-hour oral glucose tolerance test (OGTT) and continuous glucose monitoring (CGM) under free-living conditions. RESEARCH DESIGN AND METHODS: Height, weight, body mass index (BMI), hemoglobin A1c (HbA1c), and Forced expiratory volume (FEV1%) were measured in children with CF (aged 5-18 years). Following OGTT, CGM was installed for 3 days. The total hypoglycemic and hyperglycemic time (%) during 3 days was measured...
November 22, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27859825/do-children-with-cystic-fibrosis-receiving-outreach-care-have-poorer-clinical-outcomes-than-those-treated-at-a-specialist-cystic-fibrosis-centre
#20
Heinrich C Weber, Philip F Robinson, Nicole Saxby, Sean A Beggs, Ingrid Els, Rodney I Ehrlich
INTRODUCTION: Although cystic fibrosis (CF) centre care is generally considered ideal, children living in regional Australia receive outreach care supported by the academic CF centres. METHODS: This is a retrospective database review of children with CF treated at the Royal Children's Hospital in Melbourne and its outreach clinics in Albury (Victoria), and Tasmania. The aim was to compare the outcomes of children with CF managed at an academic centre with that of outreach care, using lung function, nutritional status and Pseudomonas aeruginosa colonisation...
November 17, 2016: Australian Journal of Rural Health
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