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cystic fibrosis nutrition

Gregory C Jones, Christopher A R Sainsbury
Cystic fibrosis is a common genetic condition and abnormal glucose handling leading to cystic fibrosis-related diabetes (CFRD) is a frequent comorbidity. CFRD is mainly thought to be the result of progressive pancreatic damage resulting in beta cell dysfunction and loss of insulin secretion. Whilst Oral Glucose Tolerance Testing is still recommended for diagnosing CFRD, the relationship between glucose abnormalities and adverse outcomes in CF is complex and occurs at stages of dysglycaemia occurring prior to diagnosis of diabetes by World Health Organisation criteria...
October 17, 2016: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
Joseph F Levy, Marjorie A Rosenberg, Philip M Farrell
BACKGROUND: Previous estimates of the cost of care for pediatric Cystic fibrosis (CF) showed wide variation, without specific summary of pulmonary drug costs. METHODS: Enrolled CF children from the Wisconsin newborn screening trial were evaluated quarterly per protocol. Assessments systematically included all treatments, hospitalizations, and nutritional and pulmonary outcomes. Direct medical costs from hospital billing and medical records from 1989 to 2010 were used to describe costs by age-ranges and subgroups throughout follow-up...
October 14, 2016: Pediatric Pulmonology
Valérie Boudreau, Quitterie Reynaud, Catherine Lehoux Dubois, Adèle Coriati, Katherine Desjardins, Isabelle Durieu, Rémi Rabasa-Lhoret
Nearly 50% of adult patients with cystic fibrosis (CF) have diabetes. The occurrence of CF-related diabetes (CFRD) is preceded and is associated with deterioration of lung function and nutritional status. Microvascular complications can occur, but the main cause of death is respiratory failure rather than cardiovascular causes as in type 1 or type 2 diabetes. Because other methods such as glycated hemoglobin (A1C) levels are less sensitive in patients with CF, the recommended screening test is the oral glucose tolerance test (OGTT) with a 75 g glucose dose...
October 2016: Canadian Journal of Diabetes
Michail Mantzios, Helen Egan, Carolyn Patchell
No abstract text is available yet for this article.
2016: Frontiers in Psychology
Jacqueline L Anderson, Caitlin Miles, Audrey C Tierney
BACKGROUND: An increasing body of research investigating the use of probiotics to improve health outcomes in patients with cystic fibrosis (CF) prompted the need to systematically assess and summarise the relevant literature. METHODS: An electronic search of five databases and three trial databases was conducted. Studies describing the administration of probiotics to patients with CF older than 2years, with a comparator group on respiratory, gastrointestinal and nutritional outcomes were included...
September 29, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Anne Mößeler, Marion Schmicke, Martin Höltershinken, Martin Beyerbach, Josef Kamphues
Pancreatic exocrine insufficiency (PEI) is a disease of diverse aetiology-e.g., majority of patients suffering from cystic fibrosis (CF) show PEI congenitally. Malnutrition and malabsorption of nutrients impair growth and nutritional status. As reduced fat digestion leads to a deficiency of fat-soluble vitamins the supplementation is standard, but absorption is a critical point in PEI-patients. The pancreatic duct ligated (PL) pig is an established model for PEI in humans and has been proven to be a suitable model to compare different vitamin additives for supplementation...
2016: International Journal of Molecular Sciences
Donatello Salvatore, Roberto Buzzetti, Gianni Mastella
Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries...
September 29, 2016: Pediatric Pulmonology
Karolina Wejnarska, Elwira Kolodziejczyk, Katarzyna Wertheim-Tysarowska, Maciej Dadalski, Agnieszka Sobczynska-Tomaszewska, Jarosław Kierkus, Jerzy Bal, Agnieszka Magdalena Rygiel, Grzegorz Oracz
OBJECTIVES: The etiological factors of chronic pancreatitis (CP) in children differ from those in adults. To date, no study has assessed the clinical course of CP in young children. The aim of our study was to evaluate the etiology and the clinical presentation of the disease in children with disease onset before 5 years of age in comparison to later-onset of CP. METHODS: A total of 276 children with CP, hospitalised from 1988 to 2015, were enrolled in the study...
September 24, 2016: Journal of Pediatric Gastroenterology and Nutrition
Christine Loong, Jessica Breton, Connie L Yang
OBJECTIVES: The present study sought to determine the long-term growth consequences after a pulmonary exacerbation in children with cystic fibrosis (CF). METHODS: Retrospective cohort study of pediatric patients with CF with a hospital admission for a pulmonary exacerbation. Logistic regression used to determine risk factors for failure to recover baseline body mass index (BMI) percentile. RESULTS: Of 72 patients, 43% failed to recover their baseline BMI percentile 12 months after discharge and these patients also had a lower forced expiratory volume in 1 second at follow-up...
October 2016: Journal of Pediatric Gastroenterology and Nutrition
Wenjie Wang, Ming-Hui Chen, Sy Han Chiou, Hui-Chuan Lai, Xiaojing Wang, Jun Yan, Zhumin Zhang
BACKGROUND: Persistent Pseudomonas aeruginosa (PPA) infection promotes lung function deterioration in children with cystic fibrosis (CF). Although early CF diagnosis through newborn screening (NBS) has been shown to provide nutritional/growth benefit, it is unclear whether NBS lowers the risk of PPA infection and how the effect of NBS vary with age. Modeling the onset age of PPA infection is challenging because 1) the onset age of PPA infection is interval censored in patient registry data; and 2) some risk factors such as NBS may have time-varying effects...
2016: BMC Medical Research Methodology
Isabelle J Schalk, Olivier Cunrath
Biological metal ions, including Co, Cu, Fe, Mg, Mn, Mo, Ni and Zn ions, are necessary for the survival and the growth of all microorganisms. Their biological functions are linked to their particular chemical properties: they play a role in structuring macromolecules and/or act as co-factors catalyzing diverse biochemical reactions. These metal ions are also essential for microbial pathogens during infection: they are involved in bacterial metabolism and various virulence factor functions. Therefore, during infection, bacteria need to acquire biological metal ions from the host such that there is competition for these ions between the bacterium and the host...
October 2016: Environmental Microbiology
Jacek Hochwälder, Agneta Bergsten Brucefors, Lena Hjelte
AIM: The CFQ-R is one of the most established disease-specific, health-related quality of life (HRQOL) measurements for patients with cystic fibrosis (CF). The aim was to evaluate the psychometric properties of the Swedish translation of CFQ-R in adults. METHOD: A total of 173 CF patients answered the CFQ-R. The CFQ-R was evaluated with regard to: (1) distributional properties; (2) reliability; and (3) construct validity. RESULTS: The majority of scales were negatively skewed with ceiling effects...
September 15, 2016: Upsala Journal of Medical Sciences
Peter Colin Gregory, Katrin Hoffmann, Josef Kamphues, Anne Möeler
Modern therapy of pancreatic exocrine insufficiency (PEI) using pancreatic enzyme replacement therapy (PERT) has largely been very effective and has greatly helped in improving the nutritional status of patients with PEI and in increasing the life expectancy in cystic fibrosis. It is believed that the use of predictable large animal models could play an important role in assessing and developing new therapies. This article reviews the pancreatic duct ligated (adult) minipig as a chronic model of total PEI, with a detailed look at the influence of PEI and response to PERT on prececal compared to fecal digestibility, to directly investigate effects on protein and starch digestion and absorption...
October 2016: Pancreas
Roy J Kim
Cystic fibrosis-related diabetes mellitus (CFRD) is the most common endocrine complication of cystic fibrosis (CF), affecting more than 50% of patients by the 4th decade of life. CFRD is often preceded by worsening pulmonary status and nutritional decline. Treatment of CFRD is associated with improvements in body weight and pulmonary function and a reduction in pulmonary exacerbations. Because of the clinical significance of CFRD, diabetes screening with an oral glucose tolerance test (OGTT) is recommended annually for all patients with CF starting at age 10 years...
September 1, 2016: Pediatric Annals
Sarah Jane Schwarzenberg, Sarah E Hempstead, Catherine M McDonald, Scott W Powers, Jamie Wooldridge, Shaina Blair, Steven Freedman, Elaine Harrington, Peter J Murphy, Lena Palmer, Amy E Schrader, Kyle Shiel, Jillian Sullivan, Melissa Wallentine, Bruce C Marshall, Amanda Radmer Leonard
Nutrition is integral to the care of individuals with cystic fibrosis (CF). Better nutritional status is associated with improved pulmonary function. In some individuals with CF, enteral tube feeding can be useful in achieving optimal nutritional status. Current nutrition guidelines do not include detailed recommendations for enteral tube feeding. The Cystic Fibrosis Foundation convened an expert panel to develop enteral tube feeding recommendations based on a systematic review of the evidence and expert opinion...
September 3, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Li Li, Lutz Krause, Shawn Somerset
BACKGROUND: Cystic fibrosis (CF) involves chronic inflammation and oxidative stress affecting mainly the respiratory and digestive systems. Survival rates for CF have improved with advances in treatment including nutritional interventions such as micronutrient supplementation. Diet can modulate gut microbiota in the general population with consequences on local and systemic immunity, and inflammation. The gut microbiota appears disrupted and may associate with pulmonary status in CF. This study investigated associations between micronutrient intakes and gut microbiota variations in a group of adults with CF...
August 27, 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
Lyle L Pritchard
Cystic fibrosis (CF) is an autosomal recessive genetic disease that occurs in approximately 1 in 2,500 white live births. It is less common in nonwhite individuals. A dysfunctional epithelial chloride channel leads to excessively thick mucus affecting multiple organ systems. Common issues include mucous plugging of the airway, lung inflammation, chronic pulmonary infections, intestinal malabsorption, and malnutrition. Universal screening of newborns for CF is recommended in many countries. CF can be diagnosed based on clinical evidence of disease along with genetic testing or other laboratory evidence of chloride channel dysfunction...
September 2016: FP Essentials
Lacrecia J Britton, Gabriela R Oates, Robert A Oster, Staci T Self, Robert B Troxler, Wynton C Hoover, Hector H Gutierrez, William T Harris
OBJECTIVE: The clinical benefit of newborn screening (NBS) for cystic fibrosis (CF) has been primarily nutritional, with less overt respiratory impact. Identification of risk factors for infant CF lung disease could facilitate targeted interventions to improve pulmonary outcomes. METHODS: This retrospective study evaluated socioeconomic information, clinical data, and results from routine infant pulmonary function testing (iPFT) of infants diagnosed with CF through NBS (N = 43) at a single CF center over a 4-year period (2008-2012)...
November 2016: Pediatric Pulmonology
Sze May Ng, Helen S Moore
BACKGROUND: Malabsorption of fat and protein contributes to poor nutritional status in people with cystic fibrosis. Impaired pancreatic function may also result in increased gastric acidity, leading in turn to heartburn, peptic ulcers and the impairment of oral pancreatic enzyme replacement therapy. The administration of gastric acid-reducing agents has been used as an adjunct to pancreatic enzyme therapy to improve absorption of fat and gastro-intestinal symptoms in people with cystic fibrosis...
2016: Cochrane Database of Systematic Reviews
Sanja Stanojevic, Alexandra McDonald, Valerie Waters, Sarah MacDonald, Eric Horton, Elizabeth Tullis, Felix Ratjen
BACKGROUND: Despite extensive knowledge regarding the effect of pulmonary exacerbations treated with intravenous antibiotics on clinical outcomes in cystic fibrosis (CF), there is little known about the role of milder pulmonary exacerbations treated with oral antibiotics (oPEx). METHODS: This was a retrospective cohort study of patients with CF followed at the Hospital for Sick Children and St. Michael's Hospital from 2009 to 2014. We evaluated the effect of oPEx on short-term clinical outcomes as the proportion of oPEx events in which 100% or 90% of baseline FEV1% predicted was recovered at the end of treatment...
August 18, 2016: Thorax
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