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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/29768684/practical-approach-to-the-gastrointestinal-manifestations-of-cystic-fibrosis
#1
REVIEW
Rishi Bolia, Chee Y Ooi, Peter Lewindon, Jonathan Bishop, Sarath Ranganathan, Jo Harrison, Kristyn Ford, Natalie van der Haak, Mark R Oliver
Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner...
May 16, 2018: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/29739742/user-needs-in-the-development-of-a-health-app-ecosystem-for-self-management-of-cystic-fibrosis-user-centered-development-approach
#2
Jacqueline Floch, Annabel Zettl, Lena Fricke, Tina Weisser, Lisbet Grut, Thomas Vilarinho, Erlend Stav, Antonio Ascolese, Cornelia Schauber
BACKGROUND: Digital self-management in cystic fibrosis (CF) is foreseen as a means toward better understanding of the disease and its treatment and better adherence to the treatment. Mobile apps hold the potential to provide access to information, motivate, and strengthen compliance. However, to deliver high-quality apps, the development should be based on thorough knowledge about user needs. Empirical research on the user-centered development of mobile apps for health care is, however, still limited...
May 8, 2018: JMIR MHealth and UHealth
https://www.readbyqxmd.com/read/29724576/dietary-intake-of-energy-dense-nutrient-poor-and-nutrient-dense-food-sources-in-children-with-cystic-fibrosis
#3
Rosie Sutherland, Tamarah Katz, Victoria Liu, Justine Quintano, Rebecca Brunner, Chai Wei Tong, Clare E Collins, Chee Y Ooi
BACKGROUND: Prescription of a high-energy, high-fat diet is a mainstay of nutrition management in cystic fibrosis (CF). However, families may be relying on energy-dense, nutrient-poor (EDNP) foods rather than nutrient-dense (ND) foods to meet dietary targets. We aimed to evaluate the relative contribution of EDNP and ND foods to the usual diets of children with CF and identify sociodemographic factors associated with higher EDNP intakes. METHODS: This is a cross-sectional comparison of children with CF aged 2-18 years and age- and gender-matched controls...
April 30, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29694616/association-between-hypovitaminosis-d-and-frequency-of-pulmonary-exacerbations-in-children-and-adolescents-with-cystic-fibrosis
#4
Renata Ongaratto, Katiana Murieli da Rosa, Juliana Cristina Eloi, Matias Epifanio, Paulo Marostica, Leonardo Araújo Pinto
Objective We evaluated the association between vitamin D levels and nutritional status, pulmonary function and pulmonary exacerbations in children and adolescents with cystic fibrosis. Methods 25-hydroxyvitamin D (25(OH)D) levels of 37 children and adolescents were retrospectively evaluated. Pulmonary function, body mass index, height for age, and pulmonary exacerbations episodes were associated with vitamin D levels divided into two groups: sufficient (≥30ng/mL) and hypovitaminosis (<30ng/mL). Results Hypovitaminosis D (25(OH)D <30ng/mL) was observed in 54% of subjects...
2018: Einstein
https://www.readbyqxmd.com/read/29692998/early-diagnosis-from-newborn-screening-maximises-survival-in-severe-cystic-fibrosis
#5
Gloria Tridello, Carlo Castellani, Ilaria Meneghelli, Anna Tamanini, Baroukh M Assael
Newborn screening (NBS) for cystic fibrosis (CF) has been gradually established in several countries, but scant data are available on its long-term effects on survival. Our objective was to evaluate the long-term effects of CF NBS on survival. 586 patients, diagnosed and followed between 1971 and 2014 at the Verona CF Centre were analysed. Eligibility was confirmed in 342 cases diagnosed by NBS, 101 with meconium ileus and 143 through symptoms (44 out of 143 were NBS false negatives). The primary end-point was the 30-year overall survival in patients diagnosed by NBS...
April 2018: ERJ Open Research
https://www.readbyqxmd.com/read/29685811/effectiveness-of-ivacaftor-in-cystic-fibrosis-patients-with-non-g551d-gating-mutations
#6
Jennifer Guimbellot, George M Solomon, Arthur Baines, Sonya L Heltshe, Jill VanDalfsen, Elizabeth Joseloff, Scott D Sagel, Steven M Rowe
BACKGROUND: The cystic fibrosis transmembrane conductance regulator (CFTR) potentiator ivacaftor is approved for patients with CF with gating and residual function CFTR mutations. We report the results of an observational study investigating its effects in CF patients with non-G551D gating mutations. METHODS: Patients with non-G551D gating mutations were recruited to an open-label study evaluating ivacaftor. Primary outcomes included: lung function, sweat chloride, weight gain, and quality of life scores...
April 20, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29658186/use-of-an-in-line-digestive-cartridge-with-enteral-nutrition-improves-the-weight-trajectory-of-2-children-with-cystic-fibrosis-complicated-by-another-medical-diagnosis
#7
Catherine Giguere-Rich, Amy Mathew, Elizabeth Reid, Kimberly Autore, Margaret F Guill
This clinical observation describes the enteral nutrition (EN) management of 2 toddlers at high nutrition risk due to cystic fibrosis (CF), exocrine pancreatic insufficiency, and comorbid medical conditions. The first case report describes a boy with severe malabsorption after intestinal resection. The second case report reviews a boy with CF and neuroblastoma. When pancreatic enzyme replacement therapy with EN was not effective or appropriate, use of an in-line digestive cartridge was initiated. While using the digestive cartridge, both children showed improvements in their anthropometric measures...
April 2018: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/29658185/evaluating-changes-in-handgrip-strength-in-children-with-cystic-fibrosis-a-pilot-study
#8
Hannah T Gibson, Catherine M McDonald, Jennifer Willahan Derrick, Dennis L Eggett, Sarah Gunnell Bellini
BACKGROUND: Body mass index (BMI) is used to determine nutrition status in children with cystic fibrosis (CF); however, lean body mass (LBM) is more strongly associated with pulmonary function. Handgrip strength (HGS) measures muscle function and is reflective of LBM. The aims of this study were to assess relationships among HGS, nutrition status, and pulmonary function; changes in HGS posthospitalization; and any relationship between HGS and nutrient intake. METHODS: Twenty-three children with CF aged 6-18 years participated...
April 2018: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/29607494/inhaled-anti-pseudomonal-antibiotics-for-long-term-therapy-in-cystic-fibrosis
#9
REVIEW
Sherie Smith, Nicola J Rowbotham, Kate H Regan
BACKGROUND: Inhaled antibiotics are commonly used to treat persistent airway infection with Pseudomonas aeruginosa that contributes to lung damage in people with cystic fibrosis. Current guidelines recommend inhaled tobramycin for individuals with cystic fibrosis and persistent Pseudomonas aeruginosa infection who are aged six years or older. The aim is to reduce bacterial load in the lungs so as to reduce inflammation and deterioration of lung function. This is an update of a previously published review...
March 30, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/29557624/massive-hemorrhage-a-late-complication-of-replacement-percutaneous-endoscopic-gastrostomy-case-report
#10
Tamer Sekmenli, Metin Gündüz, Hikmet Akbulut, H Haldun Emiroglu, Mustafa Koplay, Ilhan Ciftci
Percutaneous endoscopic gastrostomy (PEG) is used as an alternative to enteral/nasoenteral feeding in situations where long-term oral feeding is ineffective or not tolerated. It is mostly preferred in patients with neurological conditions and also to support nutrition in patients with congenital heart diseases, cystic fibrosis, inflammatory bowel disease, and various oropharyngeal diseases. Although it is easily applicable compared to many invasive procedures, it has complications ranging from wound infection to death...
April 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29543697/nutritional-status-the-first-two-years-of-life-in-cystic-fibrosis-diagnosed-by-newborn-screening
#11
Anne Munck, Rym Boulkedid, Laurence Weiss, Pierre Foucaud, Nathalie Wizla-Derambure, Philippe Reix, François Bremont, Jocelyne Derelle, Julien Schroedt, Corinne Alberti
OBJECTIVE: To evaluate nutritional status and associated factors in a cystic fibrosis (CF) cohort diagnosed by newborn screening and followed up to month 24. METHODS: A prospective longitudinal multicenter study assessing nutritional status according to pancreatic status, feeding modalities, prescriptions, pulmonary outcome and biological nutritional parameters. RESULTS: One-hundred-and-five infants were recruited and 99 completed the study...
March 14, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/29541628/a-c-di-gmp-modulating-protein-regulates-swimming-motility-of-burkholderia-cenocepacia-in-response-to-arginine-and-glutamate
#12
Brijesh Kumar, John L Sorensen, Silvia T Cardona
Burkholderia cenocepacia is an opportunistic bacterium that can thrive in different environments, including the amino acid-rich mucus of the cystic fibrosis (CF) lung. B. cenocepacia responds to the nutritional conditions that mimic the CF sputum by increasing flagellin expression and swimming motility. Individual amino acids also induce swimming but not flagellin expression. Here, we show that modulation of the second messenger cyclic dimeric guanosine monophosphate (c-di-GMP) levels by the PAS-containing c-di-GMP phosphodiesterase, BCAL1069 (CdpA), regulates the swimming motility of B...
2018: Frontiers in Cellular and Infection Microbiology
https://www.readbyqxmd.com/read/29506377/-italian-cystic-fibrosis-registry-report-2011-2014
#13
Barbara Giordani, Annalisa Amato, Fabio Majo, Gianluca Ferrari, Serena Quattrucci, Laura Minicucci, Rita Padoan, Giovanna Floridia, Gianna Puppo Fornaro, Domenica Taruscio, Marco Salvatore
INTRODUCTION: The Italian Cystic Fibrosis Registry (ICFR) is based on a new agreement about the data flow towards the Registry signed on October, 4th 2016 by the Centre for Rare Diseases of the Italian National Institute of Health (NIH), the clinicians of the Italian National Referral and Support Centres for Cystic Fibrosis, the Paediatric Hospital "Bambino Gesù" (Rome), the Italian Cystic Fibrosis Society, and the Italian League for Cystic Fibrosis. OBJECTIVES: The aim of the present Report is to improve the knowledge on cystic fibrosis (CF) through the epidemiological description of Italian patients...
January 2018: Epidemiologia e Prevenzione
https://www.readbyqxmd.com/read/29487998/hyperthyrotropinemia-in-newly-diagnosed-cystic-fibrosis-patients-with-pancreatic-insufficiency-reversed-by-enzyme-therapy
#14
Aris Giannakopoulos, Anni Katelaris, Maria Noni, Theodore Karakonstantakis, Christina Kanaka-Gantenbein, Stavros Doudounakis
Patients with cystic fibrosis (CF) commonly present with an elevated TSH concentration, suggesting subclinical hypothyroidism. Its relation to concomitant pancreatic insufficiency and its natural course upon initiation of enzyme replacement have not been adequately studied. Herein, we investigated the thyroid function in newly diagnosed infants with CF and monitored the course of thyroid function response to pancreatic enzyme substitution treatment. Fourteen, newly diagnosed infants with CF and pancreatic insufficiency, were followed every 6-8 weeks for 6 months ensuing onset of pancreatic enzyme substitution therapy...
May 2018: European Journal of Pediatrics
https://www.readbyqxmd.com/read/29469024/association-between-body-composition-and-pulmonary-function-in-children-and-young-people-with-cystic-fibrosis
#15
Patrizia Calella, Giuliana Valerio, Matt Thomas, Helen McCabe, Jake Taylor, Malcolm Brodlie, Mario Siervo
OBJECTIVES: Body mass index (BMI) has significant limitations when assessing nutritional status in pediatric patients with cystic fibrosis (CF). We evaluated whether measurements of lean body mass (LBM) and fat mass (FM) are more sensitive nutritional parameters by testing their association with pulmonary function in adolescent patients with CF. METHODS: Sixty-nine male and female adolescents with CF were studied (age: 14.5 ± 2.3, BMI: 19.5 ± 2.3 kg/m2 )...
April 2018: Nutrition
https://www.readbyqxmd.com/read/29459095/the-pharmacist-s-role-in-supporting-people-living-with-cystic-fibrosis
#16
Olufunmilola Abraham, Jenny S Li, Kathy E Monangai, Alison M Feathers, Daniel Weiner
OBJECTIVES: To describe the critical need for pharmacists' involvement in outpatient care for people living with cystic fibrosis (CF). DATA SOURCES: Not applicable. SUMMARY: CF is a pulmonary condition that affects more than 30,000 children and adults in the United States and 70,000 people worldwide. Various complex medication regimens are given to patients with CF, some depending on the type of mutation they have in their CF transmembrane conductance regulator protein...
May 2018: Journal of the American Pharmacists Association: JAPhA
https://www.readbyqxmd.com/read/29457700/impact-of-home-spirometry-on-medication-adherence-among-adolescents-with-cystic-fibrosis
#17
Aarti Shakkottai, Niko Kaciroti, Lauren Kasmikha, Samya Z Nasr
OBJECTIVE: Medication adherence among adolescents with cystic fibrosis (CF) is often suboptimal and this has significant impact on their health and quality of life. The purpose of the study was to evaluate the impact of frequent home pulmonary function (PFT) monitoring on medication adherence among adolescents with CF. HYPOTHESIS: We hypothesized that weekly home PFT monitoring will improve adherence while not significantly adding to the treatment burden. METHODS: Individuals aged 12-21 years with CF were provided a spirometer to measure PFTs weekly for 1 year...
April 2018: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29444656/implementation-of-a-successful-eradication-protocol-for-burkholderia-cepacia-complex-in-cystic-fibrosis-patients
#18
Bryan A Garcia, Jacque L Carden, Dana L Goodwin, Tim A Smith, Amit Gaggar, Kevin Leon, Veena B Antony, Steven M Rowe, George M Solomon
BACKGROUND: Infection with Burkholderia cepacia complex (Bcc) results in a heterogeneous clinical course ranging from asymptomatic colonization of the airways to fulminant respiratory failure in patients with cystic fibrosis (CF). Early eradication of Pseudomonas aeruginosa improves clinical outcomes. The efficacy and clinical outcomes following implementation of an eradication protocol for Bcc are less well understood. METHODS: We developed and implemented a single center Bcc eradication protocol that included an intensive combination of intravenous, inhaled, and oral antibiotic therapies based on in vitro sensitivities...
February 14, 2018: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/29412427/association-of-nutritional-status-with-lung-function-and-morbidity-in-children-and-adolescents-with-cystic-fibrosis-a-36-month-cohort-study
#19
Daniela Barbieri Hauschild, Anauã Franco Rosa, Julia Carvalho Ventura, Eliana Barbosa, Emília Addison Machado Moreira, Norberto Ludwig Neto, Yara Maria Franco Moreno
OBJECTIVE: To evaluate the association between nutritional status, lung function and morbidity in a 36-month cohort in children and adolescents with cystic fibrosis. METHODS: Prospective cohort of children and adolescents with cystic fibrosis aged 1-15 years. At the baseline, the nutritional status was determined by weight-for-height and body mass index-for-age for children <2 years and ≥2 years, respectively, and classified as: nutritional failure, nutritional risk and acceptable; and by the 50th percentile, according to the World Health Organization (WHO) growth charts...
January 2018: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
https://www.readbyqxmd.com/read/29396026/the-effect-of-enteral-tube-feeding-in-cystic-fibrosis-a-registry-based-study
#20
Denis Libeert, Dimitri Declercq, Simeon Wanyama, Muriel Thomas, Sabine Van Daele, Frans De Baets, Stephanie Van Biervliet
BACKGROUND: Long-term effect of enteral tube feeding (ETF) in cystic fibrosis (CF) remains equivocal. METHODS: A Belgian CF registry based, retrospective, longitudinal study, evaluated the pre- and post- ETF (n = 113) clinical evolution and compared each patient with 2 age, gender, pancreatic status and genotype class-matched controls. RESULTS: At baseline ETF had a worse BMI z-score (p < 0.0001) and FEV1% (p < 0.0001) compared to controls...
March 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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