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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/28721579/survival-of-patients-with-cystic-fibrosis-depending-on-mutation-type-and-nutritional-status
#1
A Szwed, A John, J Goździk-Spychalska, W Czaiński, W Czerniak, J Ratajczak, H Batura-Gabryel
The purpose of the study was to evaluate the influence of nutrition and of the severity of mutation type on survival rate in cystic fibrosis (CF) patients. Data were longitudinally collected from 60 hospitalized adult CF patients, aged 18-50. The variables consisted of body mass index (BMI) ratio, Cole's BMI cut-off points, severity of mutation type, and survival rate of CF patients. We found that the mean BMI was strongly associated with the severity of mutation type and was significantly lower in patients with severe mutations of grade I and II...
July 19, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28711222/retrospective-observational-study-of-french-patients-with-cystic-fibrosis-and-a-gly551asp-cftr-mutation-after-1-and-2years-of-treatment-with-ivacaftor-in-a-real-world-setting
#2
Dominique Hubert, Clémence Dehillotte, Anne Munck, Valérie David, Jinmi Baek, Laurent Mely, Stéphane Dominique, Sophie Ramel, Isabelle Danner Boucher, Sylvaine Lefeuvre, Quitterie Reynaud, Virginie Colomb-Jung, Prissile Bakouboula, Lydie Lemonnier
BACKGROUND: Ivacaftor has been shown to improve lung function and body weight in patients with CF and a gating mutation. Real-world evaluation is warranted to examine its safety and effectiveness over the long term. METHODS: A retrospective observational multicentre study collected clinical data in the year before and the 2years after ivacaftor initiation in patients with CF and a Gly551Asp-CFTR mutation. RESULTS: Fifty-seven patients were included...
July 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28697970/cystic-fibrosis-priorities-and-progress-for-future-therapies
#3
REVIEW
Eitan Kerem
Significant improvement in the survival of patients with CF has been achieved in the last decades. The improved clinical status of the patients is mainly the result of a better understanding of the natural course of infection and inflammation in CF that has led to the implementation of strategies that increase the life expectancy and quality of life of the patients. These strategies include prompt diagnosis, timely and aggressive nutritional support, augmentation of MCC and improved mucous drainage, initiation of antimicrobial and anti-inflammatory therapy as soon as possible, early treatment of acute exacerbations, implementation of effective hygienic measures in and outside CF centers and prompt identification and treatment of CF-related complications...
June 12, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28646623/pregnancy-outcome-in-women-with-cystic-fibrosis-related-diabetes
#4
Quitterie Reynaud, Stéphanie Poupon-Bourdy, Muriel Rabilloud, Lina Al Mufti, Christine Rousset Jablonski, Lydie Lemonnier, Raphaële Nove-Josserand, Sandrine Touzet, Isabelle Durieu
With increasing life expectancy more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis related diabetes) influenced pregnancy outcome and the clinical status of these women MATERIAL AND METHODS: We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery RESULTS: A total 249 women delivered 314 infants...
June 24, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28627192/-excess-weight-in-patients-with-cystic-fibrosis-is-it-always-beneficial
#5
David González Jiménez, Rosana Muñoz-Codoceo, María Garriga-García, Manuel Molina-Arias, Marina Álvarez-Beltrán, Ruth García-Romero, Cecilia Martínez-Costa, Silvia María Meavilla-Olivas, Luis Rodrigo Peña-Quintana, Silvia Gallego Gutiérrez, José Manuel Marugán de Miguelsanz, Lucrecia Suárez Cortina, Esperanza Natividad Castejón Ponce, Rosaura Leis Trabazo, Fayna Martín Cruz, Juan José Díaz Martín, Carlos Bousoño García
INTRODUCTION: The primary objective of this study was to find out the prevalence of overweight and obese status, as well as their association to pulmonary function, total cholesterol and vitamin D in patients with cystic fibrosis (CF). MATERIALS AND METHODS: This is a multicenter descriptive and cross-sectional study. Twelve Spanish hospitals participated. 451 patients with CF were included. Adults were classified according to body mass index (BMI) and children were classified according to BMI percentiles (WHO tables)...
June 5, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28601425/the-changing-face-of-nutrition-in-cystic-fibrosis
#6
EDITORIAL
Susan P Wolfe, Clare Collins
No abstract text is available yet for this article.
July 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28584399/immunonutrition-in-patients-with-cyctic-fibrosis-leads-to-drop-of-serum-amyloid-a-and-increase-of-oxidative-stress
#7
Ondrej Hloch, Jiri Charvat, Libor Fila, Havlin Jan
The aim of the present study is to evaluate of the impact of immunonutrition on parameters of oxidative stress and inflammation in patients with cystic fibrosis and malnutrition. In the 30 patients with cystic fibrosis and long-term enteral nutrition support for malnutrition the effect of standard and immunonutrion sipping on oxidative stress and inflammatory activity parameters was compared. Malonyldialdehyde (MDA) as parameter of oxidative stress and serum amyloid A (SAA), interleukin 1 and 6, hsCRP, IgM, IgA, IgG as parameters of inflammatory activity were examined...
May 2017: Journal of Clinical Biochemistry and Nutrition
https://www.readbyqxmd.com/read/28579360/complications-of-long-and-intermediate-term-venous-catheters-in-cystic-fibrosis-patients-a-multicenter-study
#8
Teresa L May, Alex H Gifford, Thomas Lahiri, Adam Black, Janet Trang, Alexandra G Cornell, Karyll Gonzalez, Scott Morin, Mark Napier, Christine W Duarte, Jonathan B Zuckerman
BACKGROUND: Totally implantable venous access devices (TIVADs) or peripherally inserted central venous catheters (PICCs) are commonly used in the care of patients with cystic fibrosis (CF), but they are associated with various complications, including thrombosis, infection, and insertion site symptoms. METHODS: We conducted a retrospective review of PICC and TIVAD use in adults and children with CF over an 8-year period at 3 accredited care centers. Patient attributes included CFTR genotype, comorbidities, lung function, body mass index, use of anticoagulation, and respiratory tract microbiology...
June 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
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
#9
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
#10
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
#11
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
#12
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
#13
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
#14
Steven Freedman, David Orenstein, Philip 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...
July 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28470972/oral-calorie-supplements-for-cystic-fibrosis
#15
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
#16
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
#17
MULTICENTER STUDY
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...
June 1, 2017: JAMA Pediatrics
https://www.readbyqxmd.com/read/28436621/risk-factors-for-mortality-before-age-18-years-in-cystic-fibrosis
#18
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
#19
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
#20
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
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