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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/30232597/the-role-of-small-intestinal-bacterial-overgrowth-in-cystic-fibrosis-a-randomized-case-controlled-clinical-trial-with-rifaximin
#1
Manuele Furnari, Alessandra De Alessandri, Federico Cresta, Maria Haupt, Marta Bassi, Angela Calvi, Riccardo Haupt, Giorgia Bodini, Iftikhar Ahmed, Francesca Bagnasco, Edoardo Giovanni Giannini, Rosaria Casciaro
BACKGROUND: Scientific literature shows a high prevalence of Small Intestinal Bacterial Overgrowth (SIBO) in patients with Cystic Fibrosis (CF). The role of SIBO in nutritional status and gastrointestinal symptoms in CF is not known. Our aim was to study epidemiology and clinical impact of SIBO while assessing the efficacy of rifaximin in eradicating SIBO in CF patients. METHODS: Symptoms questionnaire and Glucose Breath Test (GBT) were given to 79 CF patients (median age 19...
September 19, 2018: Journal of Gastroenterology
https://www.readbyqxmd.com/read/30228893/impact-of-nutritional-status-on-pulmonary-function-after-lung-transplantation-for-cystic-fibrosis
#2
Katharina Staufer, Emina Halilbasic, Peter Hillebrand, Solveig Harm, Stefan Schwarz, Peter Jaksch, Danijel Kivaranovic, Walter Klepetko, Michael Trauner, Lili Kazemi-Shirazi
Background: Nutritional status is an important prognostic factor in patients with cystic fibrosis (CF) prior to lung transplantation. Objective: To investigate the impact of nutritional status on pulmonary function in CF transplant recipients. Methods: Adult double lung transplanted CF patients were consecutively included. The predictive value of nutritional status on lung function - measured by spirometry - was longitudinally assessed by body composition serially evaluated by a three-compartment model bioelectrical impedance analysis (BIA) in comparison to body mass index (BMI)...
August 2018: United European Gastroenterology Journal
https://www.readbyqxmd.com/read/30221817/risk-factors-for-infection-after-pediatric-lung-transplantation
#3
Chinyere Onyearugbulem, Lauren Williams, Huirong Zhu, Maria C Gazzaneo, Ernestina Melicoff, Shailendra Das, Jorge Coss-Bu, Fong Lam, George Mallory, Flor M Munoz
Although infection is the leading cause of death in the first year following pediatric lung transplantation, there are limited data on risk factors for early infection. Sepsis remains under-recognized and under-reported in the early post-operative period for lung transplant recipients (LTR). We evaluated the incidence of infection and sepsis and identified risk factors for infection in the early post-operative period in pediatric LTRs. A retrospective review of medical records of LTRs at a large quaternary-care hospital from January 2009 to March 2016 was conducted...
September 17, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
https://www.readbyqxmd.com/read/30221745/bronchoscopy-guided-antimicrobial-therapy-for-cystic-fibrosis
#4
REVIEW
Kamini Jain, Claire Wainwright, Alan R Smyth
BACKGROUND: Early diagnosis and treatment of lower respiratory tract infections are the mainstay of management of lung disease in cystic fibrosis. When sputum samples are unavailable, treatment relies mainly on cultures from oropharyngeal specimens; however, there are concerns regarding the sensitivity of these to identify lower respiratory organisms.Bronchoscopy and related procedures (including bronchoalveolar lavage) though invasive, allow the collection of lower respiratory specimens from non-sputum producers...
September 17, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/30216443/in-vitro-digestion-of-lipids-in-real-foods-influence-of-lipid-organization-within-the-food-matrix-and-interactions-with-nonlipid-components
#5
Joaquim Calvo-Lerma, Victoria Fornés-Ferrer, Ana Heredia, Ana Andrés
In vitro digestion research has scarcely addressed the assessment of the complexity of digestion in real food. The aim of the present study was to evaluate the influence of intestinal conditions, nonlipid components, and lipid organization within the food matrix on lipolysis extent. A selection of 52 foods was studied under different simulated intestinal conditions, including those related to patients with cystic fibrosis (pH6, bile salts 1 mM due to decreased pancreatic and biliary secretions) and to healthy subjects (pH7, bile salts 10 mM)...
September 14, 2018: Journal of Food Science
https://www.readbyqxmd.com/read/30211846/development-and-pilot-implementation-of-a-nutrition-curriculum-and-rotation-in-pediatric-gastroenterology-fellowships
#6
Ala Shaikhkhalil, Candi Jump, Praveen S Goday
Structured nutrition rotations are rarely offered in pediatric gastroenterology fellowships. The NASPGHAN Nutrition Committee developed a curriculum to serve as the basis for a rotation in clinical nutrition. We worked directly with five fellowship programs to tailor the experience to individual institutions. As part of our pilot study, fellows completed knowledge assessments and self-assessment of comfort level at the start and end of the experience. We saw a trend in improvement of comfort level and increase in mean score on knowledge assessments, but the differences did not meet statistical significance...
September 11, 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/30190118/extra-skeletal-impact-of-vitamin-d-supplementation-protocol-in-an-adult-population-with-cystic-fibrosis
#7
C Lehoux Dubois, E Labrèche, V Boudreau, J Colomba, M Mailhot, A Lavoie, R Rabasa-Lhoret, A Coriati
BACKGROUND: Due to lack of vitamin D absorption in patients with cystic fibrosis (CF), vitamin D supplementation becomes necessary. Our aim was to study the association between serum vitamin D levels and key clinical factors, such as nutritional status, pulmonary function and pulmonary exacerbations (PEx) frequency, in an adult CF population. METHODS: Prospective analysis of a published vitamin D (VitD3 ) supplementation protocol (N = 200 adult patients) over a follow-up period of 5 years...
August 25, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
https://www.readbyqxmd.com/read/30170805/evaluating-the-impact-of-2006-australasian-clinical-practice-guidelines-for-nutrition-in-children-with-cystic-fibrosis-in-australia
#8
Rasa Ruseckaite, Natalie Pekin, Susannah King, Erin Carr, Susannah Ahern, John Oldroyd, Arul Earnest, Claire Wainwright, David Armstrong
OBJECTIVES: To determine the association between the implementation of the 2006 Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis (CF) and the nutritional status of children participating in the Australian Cystic Fibrosis Data Registry (ACFDR). METHODS: This research consisted of a quantitative study using ACFDR data and a survey of clinicians and dietitians treating children with CF. Two independent cohorts of children (2-5 years and 6-11 years) were selected from ACFDR between 1998 and 2014 (N = 2304)...
September 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/30170803/nutritional-status-and-pulmonary-outcome-in-children-and-young-people-with-cystic-fibrosis
#9
Niovi Papalexopoulou, Theodore G Dassios, Alan Lunt, Fiona Bartlett, Felicity Perrin, Cara J Bossley, Hilary A Wyatt, Anne Greenough
BACKGROUND: Nutrition is closely related to mortality and pulmonary and respiratory muscle function in cystic fibrosis (CF) patients. We initially validated results from a bioelectrical impedance device against dual energy x-ray absorptiometry (DEXA). We then determined whether fat free mass assessed by a portable impedance device rather than body mass index (BMI) better correlated with pulmonary function, respiratory muscle strength and exercise capacity in CF patients. METHODS: Eighteen young people and adults (median age 19, range 12-39 years) with CF had dual energy X-ray absorptiometry and direct segmental multi-frequency impedance analysis...
September 2018: Respiratory Medicine
https://www.readbyqxmd.com/read/30144894/probiotics-in-cystic-fibrosis-patients-a-double-blind-crossover-placebo-controlled-study-pilot-study-from-the-espghan-working-group-on-pancreas-cf
#10
Stephanie Van Biervliet, Bruno Hauser, Stijn Verhulst, Hedwig Stepman, Joris Delanghe, Jean-Pol Warzee, Bruno Pot, Tom Vandewiele, Michael Wilschanski
BACKGROUND: A potential positive effect of probiotics in cystic fibrosis (CF) on fecal calprotectin (FCP), pulmonary exacerbations and weight has been described in small controlled trials. METHODS: A double-blind multicenter cross-over study (2 × 4 m) was performed looking at abdominal pain, nutritional status, pulmonary function, pulmonary exacerbation, FCP and lactulose/mannitol gut permeability test. Patients kept a diary with daily scoring of abdominal pain, stool frequency and consistency as well as treatment changes...
October 2018: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/30110111/evaluation-of-nutritional-status-in-patients-with-cystic-fibrosis-according-to-age-group
#11
Lenycia de Cassya Lopes Neri, Denise Pimentel Bergamaschi, Luiz Vicente Ribeiro Ferreira da Silva Filho
OBJECTIVE: To evaluate the nutritional profile of the population assisted at a reference center for cystic fibrosis treatment. METHODS: Cross-sectional study including patients with cystic fibrosis assisted at a pediatric reference center in São Paulo, Brazil, in 2014. All patients attending regular visits who agreed to participate in the study were included. A questionnaire on dietary habits (24-hour diet recall) and socioeconomic characteristics was applied. Anthropometric data (compared with the reference from the World Health Organization, 2006 and 2007) and pulmonary function data were collected from medical records...
August 9, 2018: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
https://www.readbyqxmd.com/read/30093322/-real-world-effectiveness-of-ivacaftor-in-children-with-cystic-fibrosis-and-the-g551d-mutation
#12
David Gomez-Pastrana, Chinedu Nwokoro, Mike McLean, Sarah Brown, Nanna Christiansen, Caroline S Pao
INTRODUCTION: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that has been shown to improve the nutritional status and lung function of cystic fibrosis patients with the G551D mutation in clinical trials. The objective of this study was to describe the real-world progress of children receiving ivacaftor. METHODS: We describe the real-world progress of four children with cystic fibrosis and the F508del/G551D genotype comparing data during ivacaftor treatment with baseline and with the year before commencing treatment...
August 6, 2018: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/30091146/vitamin-a-and-beta-%C3%AE-carotene-supplementation-for-cystic-fibrosis
#13
REVIEW
Jorrit Jv de Vries, Anne B Chang, Catherine M Bonifant, Elizabeth Shevill, Julie M Marchant
BACKGROUND: People with cystic fibrosis (CF) and pancreatic insufficiency are at risk of a deficiency in fat-soluble vitamins, including vitamin A. Vitamin A deficiency predominantly causes eye and skin problems, while excessive levels of vitamin A can harm the respiratory and skeletal systems in children and interfere with the metabolism of other fat-soluble vitamins. Most CF centres administer vitamin A as supplements to reduce the frequency of vitamin A deficiency in people with CF and to improve clinical outcomes such as growth, although the recommended dose varies between different guidelines...
August 9, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/30077689/critical-care-of-the-adult-patient-with-cystic-fibrosis
#14
REVIEW
Christopher S King, A Whitney Brown, Shambhu Aryal, Kareem Ahmad, Scott Donaldson
Cystic fibrosis (CF) is a multiorgan, genetic disease resulting from dysfunction of the CF transmembrane conductance regulator. The primary clinical manifestations are bronchiectasis, chronic pulmonary infections with recurrent exacerbations, and pancreatic insufficiency. Advances in CF care have led to improved survival, with more than one-half of those affected being adults. As a result, adult pulmonary and critical care physicians increasingly will be involved in the care of these patients. Patients with CF are at risk for numerous conditions that require ICU admission, including respiratory failure, massive hemoptysis, pneumothorax, hepatic failure, and bowel obstruction...
August 2, 2018: Chest
https://www.readbyqxmd.com/read/30074573/absorption-and-safety-with-sustained-use-of-relizorb-evaluation-assure-study-in-patients-with-cystic-fibrosis-receiving-enteral-feeding
#15
John Stevens, Colby Wyatt, Perry Brown, Dhiren Patel, Danica Grujic, Steven D Freedman
OBJECTIVES: Pancreatic insufficiency (PI) and malabsorption of fats lead to reduced caloric intake, inability to maintain weight, and increased gastrointestinal symptoms. Thus, enteral nutrition (EN) is used in patients with cystic fibrosis (CF) and poor nutritional status. The current study evaluated safety, tolerability, and improvement of fatty acid (FA) status in red blood cell (RBC) membranes, a marker of long-term FA absorption, with an in-line digestive cartridge (RELiZORB) that hydrolyzes fat in enteral formula...
October 2018: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/30058245/cystic-fibrosis-liver-disease-outcomes-and-risk-factors-in-a-large-cohort-of-french-patients
#16
Pierre-Yves Boelle, Dominique Debray, Loic Guillot, Annick Clement, Harriet Corvol
Cystic fibrosis (CF)-related liver disease (CFLD) is a common symptom in patients with CF. However, its prevalence, risk factors, and evolution are unclear. We analyzed a large database of patients with CF to investigate the incidence of CFLD, its related risk factors, and the use and effect of ursodeoxycholic acid (UDCA) treatment. We retrospectively analyzed 3,328 CF patients with pancreatic insufficiency born after 1985 and recruited into the French CF Modifier Gene Study since 2004. We determined liver status, age at CFLD and severe CFLD onset, sex, CFTR genotype, history of meconium ileus, treatment with UDCA, and respiratory and nutritional status...
July 30, 2018: Hepatology: Official Journal of the American Association for the Study of Liver Diseases
https://www.readbyqxmd.com/read/30052271/treatment-for-chronic-methicillin-sensitive-staphylococcus-aureus-pulmonary-infection-in-people-with-cystic-fibrosis
#17
REVIEW
Molla Imaduddin Ahmed, Saptarshi Mukherjee
BACKGROUND: Cystic fibrosis is an inherited life-threatening multisystem disorder with lung disease characterized by abnormally thick airway secretions and persistent bacterial infection. Chronic, progressive lung disease is the most important cause of morbidity and mortality in the condition and is therefore the main focus of clinical care and research. Staphylococcus aureus is a major cause of chest infection in people with cystic fibrosis. Early onset, as well as chronic, lung infection with this organism in young children and adults results in worsening lung function, poorer nutrition and increases the airway inflammatory response, thus leading to a poor overall clinical outcome...
July 27, 2018: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/30019967/gastrointestinal-pathophysiology-and-nutrition-in-cystic-fibrosis
#18
Thomas L Ratchford, Jeffrey H Teckman, Dhiren R Patel
Cystic fibrosis (CF) is a severe, progressive, multisystemic disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. Optimizing nutrition is critical, as higher growth parameters are associated with better pulmonary function and outcomes, but unfortunately patients with this disease are prone to malnutrition, growth failure, and vitamin deficiencies. The purpose of this review is to provide a timely highlight of the physiologic processes and outcome data to support today's management strategies, as well as review these principles themselves...
September 2018: Expert Review of Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/29981489/cystic-fibrosis-body-composition-and-health-outcomes-a-systematic-review
#19
REVIEW
Patrizia Calella, Giuliana Valerio, Malcolm Brodlie, Lorenzo Maria Donini, Mario Siervo
OBJECTIVES: Patients with cystic fibrosis are characterized by an increased risk of nutrient malabsorption and inflammation, which may influence body composition. We examined the differences in body composition between patients with cystic fibrosis and healthy controls and how body composition differences may impact disease risk and mortality. METHODS: Three different electronic databases (PubMed, Web of Science, and Embase) were used to find articles from inception until March 2017...
April 6, 2018: Nutrition
https://www.readbyqxmd.com/read/29946004/anaerobic-bacteria-cultured-from-cystic-fibrosis-airways-correlate-to-milder-disease-a-multisite-study
#20
Marianne S Muhlebach, Joseph E Hatch, Gisli G Einarsson, Stef J McGrath, Deirdre F Gilipin, Gillian Lavelle, Bojana Mirkovic, Michelle A Murray, Paul McNally, Nathan Gotman, Sonia Davis Thomas, Matthew C Wolfgang, Peter H Gilligan, Noel G McElvaney, J Stuart Elborn, Richard C Boucher, Michael M Tunney
Anaerobic and aerobic bacteria were quantitated in respiratory samples across three cystic fibrosis (CF) centres using extended culture methods. Subjects aged 1-69 years who were clinically stable provided sputum (n=200) or bronchoalveolar lavage (n=55). 18 anaerobic and 39 aerobic genera were cultured from 59% and 95% of samples, respectively; 16 out of 57 genera had a ≥5% prevalence across centres.Analyses of microbial communities using co-occurrence networks in sputum samples showed groupings of oral, including anaerobic, bacteria, whereas typical CF pathogens formed distinct entities...
July 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
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