keyword
MENU ▼
Read by QxMD icon Read
search

cystic fibrosis nutrition

keyword
https://www.readbyqxmd.com/read/29140739/cftr-genotype-and-maximal-exercise-capacity-in-cystic-fibrosis-a-cross-sectional-study
#1
Thomas Radtke, Helge Hebestreit, Sabina Gallati, Jane E Schneiderman, Julia Braun, Daniel Stevens, Erik Hj Hulzebos, Tim Takken, Steven R Boas, Don S Urquhart, Larry C Lands, Sergio Tejero, Aleksandar Sovtic, Tiffany Dwyer, Milos Petrovic, Ryan A Harris, Chantal Karila, Daniela Savi, Jakob Usemann, Meir Mei-Zahav, Elpis Hatziagorou, Felix Ratjen, Susi Kriemler
RATIONALE: Cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in human skeletal muscle cells. Variations of CFTR dysfunction among patients with CF may present an important determinant of aerobic exercise capacity in CF. Previous studies on the relationship between CFTR genotype and aerobic exercise capacity are scarce and contradictory. OBJECTIVES: This study was designed to explore factors influencing aerobic exercise capacity, expressed as peak oxygen consumption (VO2peak) with a specific focus on CFTR genotype in children and adults with CF...
November 15, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29128317/lack-of-efficacy-of-lactobacillus-gg-in-reducing-pulmonary-exacerbations-and-hospital-admissions-in-children-with-cystic-fibrosis-a-randomised-placebo-controlled-trial
#2
Eugenia Bruzzese, Valeria Raia, Eliana Ruberto, Riccardo Scotto, Antonietta Giannattasio, Dario Bruzzese, Maria Cristina Cavicchi, Michela Francalanci, Carla Colombo, Nadia Faelli, Valeria Daccò, Giuseppe Magazzù, Stefano Costa, Vincenzina Lucidi, Fabio Majo, Alfredo Guarino
BACKGROUND: Intestinal dysbiosis has been described in Cystic Fibrosis (CF) and probiotics have been proposed to restore microbial composition. Aim of the study was to investigate the effects of Lactobacillus rhamnosus GG (LGG) on clinical outcomes in children with cystic fibrosis (CF). METHODS: A multicentre, randomised double-blind, clinical trial was conducted in children with CF. After 6months of baseline assessment, enrolled children (2 to 16years of age) received Lactobacillus GG (6×10(9)CFU/day) or placebo for 12months...
November 8, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/29119815/improving-transition-to-adult-healthcare-for-young-people-with-cystic-fibrosis-a-systematic-review
#3
Imelda Coyne, Aisling M Sheehan, Emily Heery, Alison E While
As survival increases worldwide, large numbers of young people will need to transition from child to adult cystic fibrosis (CF) services. Little is known about the best method for transitioning patients with CF and which transition programmes yield better outcomes. This paper provides a systematic review of the empirical literature on the outcomes and experiences of transition for young people with CF. Outcomes data were subject to a narrative synthesis and a thematic synthesis of experiences data. Structured transition programmes were associated with increased satisfaction, discussions about transition, self-care and self-advocacy skills, more independence, lower anxiety, and increased self-management and parent management of physiotherapy and nutritional supplementation...
September 2017: Journal of Child Health Care: for Professionals Working with Children in the Hospital and Community
https://www.readbyqxmd.com/read/29099606/comparison-of-nutrition-and-lung-function-outcomes-in-patients-with-cystic-fibrosis-living-in-canada-and-the-united-states
#4
Christopher H Goss, Jenna Sykes, Sanja Stanojevic, Bruce Marshall, Kristofer Petren, Josh Ostrenga, Aliza Fink, Alexander Elbert, Bradley S Quon, Anne L Stephenson
RATIONALE: A 10-year gap in the median age of survival for patients with cystic fibrosis (CF) was reported between patients living in Canada compared with the US. OBJECTIVES: Since both malnutrition and poor lung function are associated with an increased risk of mortality in CF, we investigated the temporal and longitudinal trends in lung function and nutrition between Canada and the US. METHODS: This cohort study used Canadian CF Registry and US CF Foundation Patient Registry data from 1990 to 2013...
November 3, 2017: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/29082636/bone-mineral-density-is-related-to-lung-function-outcomes-in-young-people-with-cystic-fibrosis-a-retrospective-study
#5
Nathan Smith, Angelina Lim, Matthew Yap, Louise King, Simon James, Alicia Jones, Sarath Ranganathan, Peter Simm
INTRODUCTION: Improvements in the medical management of cystic fibrosis (CF) in recent years have resulted in increased prevalence of long-term sequelae of the condition, such as low bone mineral density (BMD) and hence an increased risk of fractures in later life. Aim To explore the interaction between BMD and lung function, nutrition, and genotype. METHODS: This study was a retrospective audit of 202 children with CF from August 2000 to January 2016 to investigate associations between BMD Z-scores with clinical status, nutrition, and genetics using dual-energy absorptiometry X-ray data from the Royal Children's Hospital Melbourne, Australia...
October 30, 2017: Pediatric Pulmonology
https://www.readbyqxmd.com/read/29055982/vitamin-a-status-and-its-determinants-in-patients-with-cystic-fibrosis
#6
Ewa Sapiejka, Patrycja Krzyżanowska, Dariusz Walkowiak, Ewa Wenska-Chyży, Mariusz Szczepanik, Szczepan Cofta, Andrzej Pogorzelski, Wojciech Skorupa, Jarosław Walkowiak
BACKGROUND: Routine administration of vitamin A, recommended in CF patients, can help to prevent its deficiency. However, high vitamin A supplementation may lead to its excessive level and possible toxicity. Therefore, the aim of the present study was to assess the status of vitamin A and the determinants of its body resources in CF patients. METHODS: In 196 CF patients aged from 4 months to 47 years, the following parameters  were analysed: nutritional status (standardized body weight and height, serum albumin concentration) and clinical expression of disease (lung function - spirometry; biochemical markers of liver function - ALT, AST, GGT; respiratory tract colonization by Pseudomonas aeruginosa; diabetes; cirrhosis, non-cirrhotic liver disease; exocrine pancreatic function - fecal elastase-1 concentration; blood clotting -  INR  and  vitamin  A supplementation)...
July 2017: Acta Scientiarum Polonorum. Technologia Alimentaria
https://www.readbyqxmd.com/read/28991007/the-optimal-approach-to-nutrition-and-cystic-fibrosis-latest-evidence-and-recommendations
#7
Francis M Hollander, Nicole M de Roos, Harry G M Heijerman
PURPOSE OF REVIEW: Cystic fibrosis (CF) is a progressive genetic disease that affects multiple organ systems. Therapy is directed to maintain and optimize nutritional status and pulmonary function, as these are key factors in survival. In this review, the most recent findings regarding nutritional management associated with pulmonary function and outcome will be explored. RECENT FINDINGS: Evidence-based and expert-based guidelines emphasize the need for adequate nutritional intake to improve nutritional status...
November 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28986026/nutrition-prevention-and-management-of-nutritional-failure-in-cystic-fibrosis
#8
Jillian S Sullivan, Maria R Mascarenhas
Close monitoring of nutritional status is critical to the overall health of a patient with CF. As part of routine CF care, measurement of weight and height (and calculation of weight/length or BMI as appropriate) should be performed and analyzed at each visit. Early recognition of nutritional risk is imperative and evaluation with a multidisciplinary team should be performed to assess for caloric intake, caloric malabsorption, and other causes of poor weight gain and growth. Many tools are available to use for intervention, including oral supplementation, behavioral interventions, medications, nutritional therapies, and enteral tube feeding...
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986020/meconium-ileus-in-cystic-fibrosis
#9
Meghana Sathe, Roderick Houwen
Meconium ileus (MI) is often the first manifestation of cystic fibrosis (CF) and occurs in approximately 20% of patients diagnosed with CF. This article reviews the pathophysiology of MI and its clinical presentation. It focuses on the medical and surgical management emphasizing the importance of nutrition and a multidisciplinary approach to improve both short-term and long-term outcomes for CF patients with MI.
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28986018/cystic-fibrosis-disorders-of-the-large-intestine-dios-constipation-and-colorectal-cancer
#10
James M Abraham, Christopher J Taylor
Since 1966 when the Cystic Fibrosis Foundation Patient Registry (CFFPR) was founded, clinicians have witnessed significant advances in both the quality and quantity of life for patients living with Cystic Fibrosis (CF). As patients with CF live longer and fuller lives, increasing encumbrances from gastrointestinal manifestations of CF will be observed. This article serves to discuss "below the diaphragm" concerns involving the large intestine (Distal Intestinal Obstruction Syndrome, Constipation, and Colorectal Cancer)...
November 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28968008/-profile-of-gastrointestinal-diseases-in-a-pediatric-gastroenterology-center-in-colombia-15-years-of-follow-up
#11
Wilson Daza, Silvana Dadan, Michelle Higuera
INTRODUCTION: The profile of gastrointestinal diseases is constantly changing globally and locally affecting suspected diagnosis and medical methods. OBJECTIVE: To establish the main diagnoses in a gastroenterology, hepatology, and pediatric nutrition unit in Bogota between 2009 and 2013, and to compare with findings from the same unit during the two prior five-year periods (1997 to 2006). MATERIALS AND METHODS: We conducted a retrospective descriptive study...
September 1, 2017: Biomédica: Revista del Instituto Nacional de Salud
https://www.readbyqxmd.com/read/28965239/burkholderia-cepacia-complex-in-cystic-fibrosis-in-a-brazilian-reference-center
#12
Priscila Dentini, Fernando Augusto Lima Marson, Luciana Cardoso Bonadia, Carmen Sílvia Bertuzzo, Antônio Fernando Ribeiro, Carlos Emílio Levy, José Dirceu Ribeiro
The Burkholderia cepacia complex (BCC) can cause a severe decline in lung function in cystic fibrosis (CF). Our objective was to determine the BCC prevalence and to evaluate its clinical impact on CF. Clinical and laboratory variables were determined for CF patients with BCC (Group-A = 50 patients) and without BCC (Group-B = 134 patients). The microorganisms were identified by biochemical tests, the Vitek2(®)Compact test, recA-PCR and recA-nested-PCR with species-specific primers and DNA sequencing. The patients were evaluated by the Shwachman-Kulczycki score (SKCS), Bhalla score (BS), spirometry and body mass index (BMI)...
December 2017: Medical Microbiology and Immunology
https://www.readbyqxmd.com/read/28910260/shining-new-light-on-newborn-screening-of-cystic-fibrosis-in-the-province-of-quebec
#13
Léticia Khendek
Newborn screening of cystic fibrosis, a severe genetic disease with high treatment burden, is offered in all of North America with the exception of the province of Quebec. This condition, when diagnosed on symptomatic presentation, is marked by chronic infections and progressive lung function decline leading to eventual respiratory failure. Patients continue to have a median age of survival notably below the Canadian average. Despite prevalence rates of cystic fibrosis almost three times the national average in certain regions of Quebec, the province still does not offer screening to its newborns...
September 14, 2017: Canadian Journal of Public Health. Revue Canadienne de Santé Publique
https://www.readbyqxmd.com/read/28886227/lung-transplantation-in-cystic-fibrosis-patients-in-israel-the-importance-of-ethnicity-and-nutritional-status
#14
Hagit Levine, Dario Prais, Yael Raviv, Victorya Rusanov, Dror Rosengarten, Milton Saute, Moshe Hoshen, Huda Mussaffi, Hannah Blau, Mordechai R Kramer
OBJECTIVES: To assess the characteristics that correlate with better outcomes after lung transplantation for patients with cystic fibrosis (CF). METHODS: We retrospectively reviewed the charts of all patients with CF who underwent lung transplantation between 1996-2014 at Rabin Medical Center, Israel. RESULTS: Fifty patients with CF underwent 55 lung transplantations. Eighteen patients (36%) died during the study period. Actuarial survival was 83%, 68%, 62% and 39% at 1, 3, 5 and 10 years, respectively...
September 8, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28845426/lung-function-and-disease-severity-in-cystic-fibrosis-patients-heterozygous-for-p-arg117his
#15
Michal Shteinberg, Damian G Downey, Diane Beattie, John McCaughan, Alastair Reid, Nili Stein, J Stuart Elborn
Expression of p.Arg117His cystic fibrosis (CF) transmembrane conductance regulator is influenced by a polythymidine (poly-T) tract and a thymidine-guanine (TG) repeat on intron 9, which vary in length and affect exon 10 skipping. We compared clinical characteristics and the rate of progression of lung disease of CF patients carrying the p.Arg117His mutation with different intron 9 varying sequences (poly-T) and mutation classes in trans. Data were collected from patients in Northern Ireland, UK, including diagnostic features, sweat chloride, nutritional status, sputum microbiology, CF-related complications and lung function...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28837442/diagnosis-follow-up-and-treatment-of-cystic-fibrosis-related-liver-disease
#16
Ivo P van de Peppel, Anna Bertolini, Johan W Jonker, Frank A J A Bodewes, Henkjan J Verkade
PURPOSE OF REVIEW: To provide an insight and overview of the challenges in the diagnosis, follow-up and treatment of cystic fibrosis-related liver disease (CFLD). RECENT FINDINGS: The variable pathophysiology of CFLD complicates its diagnosis and treatment. A 'gold standard' for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension...
August 23, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28832807/methicillin-resistant-staphylococcus-aureus-in-cystic-fibrosis-patients-do-we-need-to-care-a-cohort-study
#17
Renata Wrobel Folescu Cohen, Tânia Wrobel Folescu, Pedro Daltro, Marcia Cristina Bastos Boechat, Danielle Ferreira Lima, Elizabeth Andrade Marques, Robson Souza Leão
CONTEXT AND OBJECTIVE: The prevalence of a variety of potentially pathogenic microorganisms in cystic fibrosis patients, such as methicillin-resistant Staphylococcus aureus (MRSA), has increased over the past decade. Given the increasing prevalence of MRSA and the few data available in the literature, better understanding of the clinical repercussions of colonization by this bacterium in cystic fibrosis patients becomes essential. This study aimed to evaluate the repercussions of chronic colonization by MRSA in cystic fibrosis patients...
August 21, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28814218/diagnosis-and-management-of-pancreatic-exocrine-insufficiency
#18
Mehrdad Nikfarjam, Jeremy S Wilson, Ross C Smith
In 2015, the Australasian Pancreatic Club (APC) published the Australasian guidelines for the management of pancreatic exocrine insufficiency (http://pancreas.org.au/2016/01/pancreatic-exocrine-insufficiency-guidelines). Pancreatic exocrine insufficiency (PEI) occurs when normal digestion cannot be sustained due to insufficient pancreatic digestive enzyme activity. This may be related to a breakdown, at any point, in the pancreatic digestive chain: pancreatic stimulation; synthesis, release or transportation of pancreatic enzymes; or synchronisation of secretions to mix with ingested food...
August 21, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28795206/children-and-adolescents-with-cystic-fibrosis-display-moderate-bone-microarchitecture-abnormalities-data-from-high-resolution-peripheral-quantitative-computed-tomography
#19
C Braun, J Bacchetta, P Braillon, R Chapurlat, J Drai, P Reix
We investigated whether bone microstructure assessed by high-resolution peripheral quantitative tomography (HR-pQCT) could be altered in children and teenagers with cystic fibrosis (CF). In comparison to their healthy counterparts, bone microstructure was mildly affected at the tibial level only. INTRODUCTION: Cystic fibrosis-related bone disease (CFBD) may alter bone health, ultimately predisposing patients to bone fractures. Our aim was to assess bone microstructure using high-resolution peripheral quantitative tomography (HR-pQCT) in a cohort of children and teenagers with CF in comparison to age-, puberty-, and gender-matched healthy volunteers (HVs)...
November 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28770464/long-term-follow-up-in-a-girl-with-cystic-fibrosis-and-diabetes-since-the-first-year-of-life
#20
Valentina Fattorusso, Alida Casale, Valeria Raia, Enza Mozzillo, Adriana Franzese
Diabetes mellitus is the most common comorbidity in cystic fibrosis (CF). Recently, more attention has been paid to early glucose metabolism derangements (GMDs). The subject of this report is a female patient, affected by CF since 3 months of age. She presented with intermittent diabetes during early childhood. At the age of 10 years, oral glucose tolerance test (OGTT) was performed and showed glucose intolerance (IGT) status; glargine insulin therapy was started. At the age of 13 years, CF-related diabetes with fasting hyperglycemia occurred, so rapid insulin at meals was added...
October 2017: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
keyword
keyword
108809
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"