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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/28508087/the-vitamin-d-for-enhancing-the-immune-system-in-cystic-fibrosis-disc-trial-rationale-and-design-of-a-multi-center-double-blind-placebo-controlled-trial-of-high-dose-bolus-administration-of-vitamin-d3-during-acute-pulmonary-exacerbation-of-cystic-fibrosis
#1
Vin Tangpricha, Ellen M Smith, Jose Binongo, Suzanne E Judd, Thomas R Ziegler, Seth Walker, Rabindra Tirouvanziam, Susu M Zughaier, Moon Jeong Lee, Supavit Chesdachai, Wendy A Hermes, James F Chmiel, Amit Gaggar, Ruth E Grossmann, Patricia M Joseph, Jessica A Alvarez
Vitamin D deficiency is highly prevalent in children and adults with cystic fibrosis (CF). Recent studies have found an association between vitamin D status and risk of pulmonary exacerbations in children and adults with CF. The ongoing Vitamin D for enhancing the Immune System in Cystic fibrosis (DISC) study is a multi-center, double-blind, randomized, placebo-controlled trial that will test the hypothesis of whether high dose vitamin D given as a single oral bolus of 250,000 IU to adults with CF during a pulmonary exacerbation followed by a maintenance dose of vitamin D will improve time to next pulmonary exacerbation and re-hospitalization, improve survival and lung function compared to placebo and reduce the rates of pulmonary exacerbation,...
June 2017: Contemporary Clinical Trials Communications
https://www.readbyqxmd.com/read/28502446/pancreatic-disorders
#2
REVIEW
Aliye Uc, Douglas S Fishman
Once considered uncommon, pancreatic diseases are increasingly recognized in the pediatric age group. Acute pancreatitis, acute recurrent pancreatitis, and chronic pancreatitis occur in children with an incidence approaching that of adults. Risk factors are broad, prompting the need for a completely different diagnostic and therapeutic approach in children. Although cystic fibrosis remains the most common cause of exocrine pancreatic insufficiency, other causes such as chronic pancreatitis may be as common as Shwachman Diamond syndrome...
June 2017: Pediatric Clinics of North America
https://www.readbyqxmd.com/read/28497760/the-intestinal-microbiome-and-paediatric-liver-disease
#3
REVIEW
Daniel H Leung, Dean Yimlamai
The intestinal microbiome has been the intense focus of recent study, but how the microbiota affects connected organs, such as the liver, has not been fully elucidated. The microbiome regulates intestinal permeability and helps to metabolise the human diet into small molecules, thus directly affecting liver health. Several studies have linked intestinal dysbiosis to the severity and progression of liver diseases, such as non-alcoholic fatty liver disease, non-alcoholic steatohepatitis, primary sclerosing cholangitis, total parenteral nutrition-associated liver disease, and cystic fibrosis-associated liver disease...
June 2017: Lancet. Gastroenterology & Hepatology
https://www.readbyqxmd.com/read/28482390/-clinical-analysis-of-11-children-with-pancreatic-cystic-fibrosis
#4
G L Wang, C N Zhao, J Zhou, F H Yu, H Q Shen, J Zhang, S Y Zhao, X W Xu
Objective: To increase the recognition of pancreatic cystic fibrosis (PCF) in children and facilitate diagnosing and treatment of this rare entity. Method: This is a retrospective analysis of children who presented to Beijing Children's Hospital affiliated to Capital Medical University from January 2010 to December 2015. We describe their clinical features, laboratory testing and management. Result: Eleven children were diagnosed with PCF by genetic testing or sweat chloride test during these 5 years, including 4 boys and 7 girls...
May 4, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28472055/abdominal-symptoms-in-cystic-fibrosis-and-their-relation-to-genotype-history-clinical-and-laboratory-findings
#5
Harold Tabori, Christin Arnold, Anke Jaudszus, Hans-Joachim Mentzel, Diane M Renz, Steffen Reinsch, Michael Lorenz, Ruth Michl, Andrea Gerber, Thomas Lehmann, Jochen G Mainz
BACKGROUND & AIMS: Abdominal symptoms (AS) are a hallmark of the multiorgan-disease cystic fibrosis (CF). However, the abdominal involvement in CF is insufficiently understood and, compared to the pulmonary manifestation, still receives little scientific attention. Aims were to assess and quantify AS and to relate them to laboratory parameters, clinical findings, and medical history. METHODS: A total of 131 patients with CF of all ages were assessed with a new CF-specific questionnaire (JenAbdomen-CF score 1...
2017: PloS One
https://www.readbyqxmd.com/read/28471913/increased-fat-absorption-from-enteral-formula-through-an-in-line-digestive-cartridge-in-patients-with-cystic-fibrosis
#6
Steven Freedman, David Orenstein, Phillip Black, Perry Brown, Karen McCoy, John Stevens, Danica Grujic, Russell Clayton
OBJECTIVES: Supplemental enteral nutrition (EN) is used by approximately 12% of people with cystic fibrosis (CF). The objective of this study was to evaluate the safety, tolerability, and fat absorption of a new in-line digestive cartridge (Relizorb) that hydrolyzes fat in enteral formula provided to patients with CF. METHODS: Patients with CF receiving EN participated in a multicenter, randomized, double-blind, crossover trial with an open-label safety evaluation period...
May 3, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28470972/oral-calorie-supplements-for-cystic-fibrosis
#7
REVIEW
Rosalind L Smyth, Oli Rayner
BACKGROUND: Poor nutrition occurs frequently in people with cystic fibrosis and is associated with other adverse outcomes. Oral calorie supplements are used to increase total daily calorie intake and improve weight gain. However, they are expensive and there are concerns they may reduce the amount of food eaten and not improve overall energy intake. This is an update of a previously published review. OBJECTIVES: To establish whether in people with cystic fibrosis, oral calorie supplements: increase daily calorie intake; and improve overall nutritional intake, nutritional indices, lung function, survival and quality of life...
May 4, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28449677/cystic-fibrosis-current-therapeutic-targets-and-future-approaches
#8
REVIEW
Misbahuddin M Rafeeq, Hussam Aly Sayed Murad
OBJECTIVES: Study of currently approved drugs and exploration of future clinical development pipeline therapeutics for cystic fibrosis, and possible limitations in their use. METHODS: Extensive literature search using individual and a combination of key words related to cystic fibrosis therapeutics. KEY FINDINGS: Cystic fibrosis is an autosomal recessive disorder due to mutations in CFTR gene leading to abnormality of chloride channels in mucus and sweat producing cells...
April 27, 2017: Journal of Translational Medicine
https://www.readbyqxmd.com/read/28437538/effects-of-diagnosis-by-newborn-screening-for-cystic-fibrosis-on-weight-and-length-in-the-first-year-of-life
#9
Daniel H Leung, Sonya L Heltshe, Drucy Borowitz, Daniel Gelfond, Margaret Kloster, James E Heubi, Michael Stalvey, Bonnie W Ramsey
Importance: Since the implementation of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or its association with correlates of disease among infants with CF who underwent NBS has not been well described. Objective: To examine incremental weight gain, linear growth, and clinical features in the first year of life among infants with CF who underwent NBS. Design, Setting, and Participants: The Baby Observational and Nutrition Study (BONUS), a multicenter, longitudinal, observational cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from January 7, 2012, through May 31, 2015...
April 24, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28436621/risk-factors-for-mortality-before-age-18-years-in-cystic-fibrosis
#10
Susanna A McColley, Michael S Schechter, Wayne J Morgan, David J Pasta, Marcia L Craib, Michael W Konstan
BACKGROUND: Understanding early-life risk factors for childhood death in cystic fibrosis (CF) is important for clinical care, including the identification of effective interventions. METHODS: Data from the Epidemiologic Study of Cystic Fibrosis (ESCF) collected 1994-2005 were linked with the Cystic Fibrosis Foundation Patient Registry (CFFPR) demographic and mortality data from 2013. Inclusion criteria were ≥1 visit annually at age 3-5 years and ≥1 FEV1 measurement at age 6-8 years...
April 24, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/28420572/nutritional-management-of-cystic-fibrosis-an-update-for-the-21st-century
#11
REVIEW
Sarah Collins
Nutritional management is an essential part of multidisciplinary care for infants, children and adults with cystic fibrosis (CF). In 2016 two updated nutritional consensus guidelines were published [1,2]. This review will explore some of the key points in the nutritional management of people with CF in the 21st Century.
March 14, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28417451/prophylactic-anti-staphylococcal-antibiotics-for-cystic-fibrosis
#12
REVIEW
Alan R Smyth, Margaret Rosenfeld
BACKGROUND: Staphylococcus aureus causes pulmonary infection in young children with cystic fibrosis. Prophylactic antibiotics are prescribed hoping to prevent such infection and lung damage. Antibiotics have adverse effects and long-term use might lead to infection with Pseudomonas aeruginosa. This is an update of a previously published review. OBJECTIVES: To assess continuous oral antibiotic prophylaxis to prevent the acquisition of Staphylococcus aureus versus no prophylaxis in people with cystic fibrosis, we tested these hypotheses...
April 18, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28392015/modeling-cystic-fibrosis-disease-progression-in-patients-with-the-rare-cftr-mutation-p67l
#13
Isobel E R MacKenzie, Valerie Paquette, Frances Gosse, Sheenagh George, Frederic Chappe, Valerie Chappe
BACKGROUND: The progression of cystic fibrosis (CF) in patients with the rare mutation P67L was examined to determine if it induced a milder form of CF compared to the common severe ΔF508 mutation. METHODS: Parameters of lung function, level of bacterial infection, nutritional status and hospitalization were used to represent CF progression. Age at diagnosis and pancreatic status were used to assess CF presentation. Analysis of data from the CF Canada Registry collected over a 15-year period included 266 ΔF508/ΔF508 homozygote patients from CF clinics in Atlantic Canada and 26 compound heterozygote patients with the rare P67L mutation from clinics across Canada...
May 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28388380/acquired-amino-acid-deficiencies-a-focus-on-arginine-and-glutamine
#14
Claudia R Morris, Jill Hamilton-Reeves, Robert G Martindale, Menaka Sarav, Juan B Ochoa Gautier
Nonessential amino acids are synthesized de novo and therefore not diet dependent. In contrast, essential amino acids must be obtained through nutrition since they cannot be synthesized internally. Several nonessential amino acids may become essential under conditions of stress and catabolic states when the capacity of endogenous amino acid synthesis is exceeded. Arginine and glutamine are 2 such conditionally essential amino acids and are the focus of this review. Low arginine bioavailability plays a pivotal role in the pathogenesis of a growing number of varied diseases, including sickle cell disease, thalassemia, malaria, acute asthma, cystic fibrosis, pulmonary hypertension, cardiovascular disease, certain cancers, and trauma, among others...
April 2017: Nutrition in Clinical Practice
https://www.readbyqxmd.com/read/28387447/withdrawn-interventions-for-fatigue-and-weight-loss-in-adults-with-advanced-progressive-illness
#15
REVIEW
Cathy Payne, Philip J Wiffen, Suzanne Martin
BACKGROUND: Fatigue and unintentional weight loss are two of the commonest symptoms experienced by people with advanced progressive illness. Appropriate interventions may bring considerable improvements in function and quality of life to seriously ill people and their families, reducing physical, psychological and spiritual distress. OBJECTIVES: To conduct an overview of the evidence available on the efficacy of interventions used in the management of fatigue and/or unintentional weight loss in adults with advanced progressive illness by reviewing the evidence contained within Cochrane reviews...
April 7, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28365620/current-characteristics-challenges-and-coping-strategies-of-young-people-with-cystic-fibrosis-as-they-transition-to-adulthood
#16
Kristina Askew, Jade Bamford, Nicholas Hudson, Juliana Moratelli, Rachel Miller, Alan Anderson, Simon Doe, Stephen J Bourke
This study provides detailed data on the current characteristics, perceptions and outcomes of 45 young people with cystic fibrosis (CF) as they transition into adulthood. Although many had severe disease, they generally coped well, found attendance at a transition clinic helpful and welcomed the increased independence of an adult healthcare environment. Levels of psychological distress were low with only 15.6% having anxiety and 6.7% depression. The main psychological coping strategy used was optimistic acceptance...
April 2017: Clinical Medicine: Journal of the Royal College of Physicians of London
https://www.readbyqxmd.com/read/28323913/accrual-of-bone-mass-in-children-and-adolescents-with-cystic-fibrosis
#17
Sonakshi Sharma, Mirjana Jaksic, Sheryl Fenwick, Catherine Byrnes, Tim Cundy
Context: Low bone density is a 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 bone mineral density (BMD), body mass index (BMI), and forced expiratory volume in 1 second (FEV1) z scores in children and adolescents with CF. Setting: Pediatric hospital specialist CF service...
May 1, 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
#18
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
#19
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
#20
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
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