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cystic fibrosis nutrition

Thomas L Ratchford, Jeffrey H Teckman, Dhiren R Patel
Cystic fibrosis (CF) is a severe, progressive, multi-systemic disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) 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...
July 18, 2018: Expert Review of Gastroenterology & Hepatology
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
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, ages 1-69 years, who were clinically stable provided sputum (n=200) or bronchoalveolar lavage (n=55). Eighteen anaerobic and 39 aerobic genera were cultured from 59% and 95% of samples, respectively; 16/57 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...
June 25, 2018: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
Simone Gambazza, Valentina Turrin, Chiara Speziali, Anna Brivio, Lauretta Valmarana, Federica Carta, Anna Marta Clotilde Bulfamante, Carla Colombo
OBJECTIVE: A 6-minute walk work (6MWW), that is the product of distance walked at the 6-minute walk test (6MWT) multiplied by body weight, has been suggested as an appropriate variable for estimating functional capacity. Under the hypothesis that 6MWW strongly correlates with expiratory muscle strength (PE max), as found in Chronic Obstructive Pulmonary Disease (COPD), the aim of the study was to determine the degree of this correlation in adults with cystic fibrosis. METHODS: A cross-sectional study was carried out at the Regional Referral Centre for Cystic Fibrosis, where patients with cystic fibrosis attending their regular follow-up, aged 18 years or older with mild-to-normal lung disease, were asked to participate...
June 26, 2018: Physiotherapy Research International: the Journal for Researchers and Clinicians in Physical Therapy
Joaquim Gea, Antoni Sancho-Muñoz, Roberto Chalela
Nutritional abnormalities are frequent in different chronic respiratory diseases such as chronic obstructive pulmonary disease (COPD), bronchiectasis, cystic fibrosis (CF), interstitial fibrosis and lung cancer, having important clinical consequences. However, nutritional abnormalities often remained underdiagnosed due to the relative lack of awareness of health professionals. Therefore, systematic anthropometry or even better, assessment of body composition, should be performed in all patients with chronic respiratory conditions, especially following exacerbation periods when malnutrition becomes more accentuated...
May 2018: Journal of Thoracic Disease
Maisam Abu-El-Haija, Aliye Uc, Steven L Werlin, Alvin Jay Freeman, Miglena Georgieva, Danijela Jojkić-Pavkov, Daina Kalnins, Brigitte Kochavi, Bart G P Koot, Stephanie Van Biervliet, Jaroslaw Walkowiak, Michael Wilschanski, Veronique D Morinville
OBJECTIVES: Wide variations exist in how physicians manage the nutritional aspects of children affected by acute pancreatitis (AP), acute recurrent pancreatitis (ARP), and chronic (CP) pancreatitis. Better consensus for optimal management is needed. METHODS: This consensus statement on nutrition in pediatric pancreatic diseases was developed through a joint ESPGHAN-NASPGHAN working group that performed an evidence-based search of the literature on nutrition in AP, ARP, and CP with a focus on pediatrics...
July 2018: Journal of Pediatric Gastroenterology and Nutrition
Jessica Green, Francis J Gilchrist, Will Carroll
BACKGROUND: Cystic fibrosis (CF) is the most common, life-limiting, genetically inherited disease. It affects multiple organs, particularly the respiratory system. However, gastrointestinal problems such as constipation and distal intestinal obstruction syndrome (DIOS) are also important and well-recognised complications in CF. They share similar symptoms e.g. bloating, abdominal pain, but are distinct conditions. Constipation occurs when there is gradual faecal impaction of the colon, but DIOS occurs when there is an accumulation of faeces and sticky mucus, forming a mass in the distal part of the small intestine...
June 12, 2018: Cochrane Database of Systematic Reviews
J Enrique Dominguez-Muñoz
PURPOSE OF REVIEW: Pancreatic exocrine insufficiency (PEI), defined as a secretion of pancreatic enzymes and bicarbonate insufficient to maintain a normal digestion, is a frequent but frequently underdiagnosed and undertreated condition. PEI may be secondary to different pancreatic diseases and extrapancreatic conditions. Recent data support the high clinical relevance of PEI and its treatment. RECENT FINDINGS: Together with symptoms of maldigestion, PEI is associated with nutritional deficiencies leading to osteoporosis, low-trauma fractures, sarcopenia and increased mortality...
June 7, 2018: Current Opinion in Gastroenterology
Mary Rozga, Deepa Handu
No abstract text is available yet for this article.
May 24, 2018: Journal of the Academy of Nutrition and Dietetics
Shaina M Willen, Michael R DeBaun
Although sickle cell disease and cystic fibrosis are two of the most common monogenic diseases presenting in childhood worldwide, cystic fibrosis and sickle cell disease enjoy vastly different funding and collaborative research efforts. Pulmonary complications in cystic fibrosis have well established guidelines and multidisciplinary involvement focusing on comorbidities, routine monitoring, infectious complications, nutrition, and treatment recommendations. These guidelines can provide a framework on which to build knowledge of lung disease in sickle cell disease...
June 2018: Pediatric Clinics of North America
Krista Revert, Laurence Audran, Jocelyne Pengam, Pascal Lesne, Dominique Pougheon Bertrand
BACKGROUND: The Cystic Fibrosis (CF) center in Roscoff (Brittany) has been involved in therapeutic education programs (TEP) since 2006 and took part in the pilot phase of the French quality improvement program (QIP) since 2011. The aim was to improve the nutritional status of children with cystic fibrosis aged 2-12 years old in order to optimize their health status as they enter adolescence. METHODS: A multidisciplinary quality team was created in order to select and address a specific health problem among our pediatric population...
February 8, 2018: Orphanet Journal of Rare Diseases
Rishi Bolia, Chee Y Ooi, Peter Lewindon, Jonathan Bishop, Sarath Ranganathan, Jo Harrison, Kristyn Ford, Natalie van der Haak, Mark R Oliver
Cystic fibrosis (CF) is the most common, life-shortening, genetic illness affecting children in Australia and New Zealand. The genetic abnormality results in abnormal anion transport across the apical membrane of epithelial cells in a number of organs, including the lungs, gastrointestinal tract, liver and genito-urinary tract. Thus, CF is a multi-system disorder that requires a multi-disciplinary approach. Respiratory disease is the predominant cause of both morbidity and mortality in patients with CF. However, there are significant and clinically relevant gastrointestinal, liver, pancreatic and nutritional manifestations that must be detected and managed in a timely and structured manner...
June 2018: Journal of Paediatrics and Child Health
Jacqueline Floch, Annabel Zettl, Lena Fricke, Tina Weisser, Lisbet Grut, Thomas Vilarinho, Erlend Stav, Antonio Ascolese, Cornelia Schauber
BACKGROUND: Digital self-management in cystic fibrosis (CF) is foreseen as a means toward better understanding of the disease and its treatment and better adherence to the treatment. Mobile apps hold the potential to provide access to information, motivate, and strengthen compliance. However, to deliver high-quality apps, the development should be based on thorough knowledge about user needs. Empirical research on the user-centered development of mobile apps for health care is, however, still limited...
May 8, 2018: JMIR MHealth and UHealth
Rosie Sutherland, Tamarah Katz, Victoria Liu, Justine Quintano, Rebecca Brunner, Chai Wei Tong, Clare E Collins, Chee Y Ooi
BACKGROUND: Prescription of a high-energy, high-fat diet is a mainstay of nutrition management in cystic fibrosis (CF). However, families may be relying on energy-dense, nutrient-poor (EDNP) foods rather than nutrient-dense (ND) foods to meet dietary targets. We aimed to evaluate the relative contribution of EDNP and ND foods to the usual diets of children with CF and identify sociodemographic factors associated with higher EDNP intakes. METHODS: This is a cross-sectional comparison of children with CF aged 2-18 years and age- and gender-matched controls...
April 30, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Renata Ongaratto, Katiana Murieli da Rosa, Juliana Cristina Eloi, Matias Epifanio, Paulo Marostica, Leonardo Araújo Pinto
Objective We evaluated the association between vitamin D levels and nutritional status, pulmonary function and pulmonary exacerbations in children and adolescents with cystic fibrosis. Methods 25-hydroxyvitamin D (25(OH)D) levels of 37 children and adolescents were retrospectively evaluated. Pulmonary function, body mass index, height for age, and pulmonary exacerbations episodes were associated with vitamin D levels divided into two groups: sufficient (≥30ng/mL) and hypovitaminosis (<30ng/mL). Results Hypovitaminosis D (25(OH)D <30ng/mL) was observed in 54% of subjects...
2018: Einstein
Gloria Tridello, Carlo Castellani, Ilaria Meneghelli, Anna Tamanini, Baroukh M Assael
Newborn screening (NBS) for cystic fibrosis (CF) has been gradually established in several countries, but scant data are available on its long-term effects on survival. Our objective was to evaluate the long-term effects of CF NBS on survival. 586 patients, diagnosed and followed between 1971 and 2014 at the Verona CF Centre were analysed. Eligibility was confirmed in 342 cases diagnosed by NBS, 101 with meconium ileus and 143 through symptoms (44 out of 143 were NBS false negatives). The primary end-point was the 30-year overall survival in patients diagnosed by NBS...
April 2018: ERJ Open Research
Jennifer Guimbellot, George M Solomon, Arthur Baines, Sonya L Heltshe, Jill VanDalfsen, Elizabeth Joseloff, Scott D Sagel, Steven M Rowe
BACKGROUND: The cystic fibrosis transmembrane conductance regulator (CFTR) potentiator ivacaftor is approved for patients with CF with gating and residual function CFTR mutations. We report the results of an observational study investigating its effects in CF patients with non-G551D gating mutations. METHODS: Patients with non-G551D gating mutations were recruited to an open-label study evaluating ivacaftor. Primary outcomes included: lung function, sweat chloride, weight gain, and quality of life scores...
April 21, 2018: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
Catherine Giguere-Rich, Amy Mathew, Elizabeth Reid, Kimberly Autore, Margaret F Guill
This clinical observation describes the enteral nutrition (EN) management of 2 toddlers at high nutrition risk due to cystic fibrosis (CF), exocrine pancreatic insufficiency, and comorbid medical conditions. The first case report describes a boy with severe malabsorption after intestinal resection. The second case report reviews a boy with CF and neuroblastoma. When pancreatic enzyme replacement therapy with EN was not effective or appropriate, use of an in-line digestive cartridge was initiated. While using the digestive cartridge, both children showed improvements in their anthropometric measures...
April 2018: Nutrition in Clinical Practice
Hannah T Gibson, Catherine M McDonald, Jennifer Willahan Derrick, Dennis L Eggett, Sarah Gunnell Bellini
BACKGROUND: Body mass index (BMI) is used to determine nutrition status in children with cystic fibrosis (CF); however, lean body mass (LBM) is more strongly associated with pulmonary function. Handgrip strength (HGS) measures muscle function and is reflective of LBM. The aims of this study were to assess relationships among HGS, nutrition status, and pulmonary function; changes in HGS posthospitalization; and any relationship between HGS and nutrient intake. METHODS: Twenty-three children with CF aged 6-18 years participated...
April 2018: Nutrition in Clinical Practice
Sherie Smith, Nicola J Rowbotham, Kate H Regan
BACKGROUND: Inhaled antibiotics are commonly used to treat persistent airway infection with Pseudomonas aeruginosa that contributes to lung damage in people with cystic fibrosis. Current guidelines recommend inhaled tobramycin for individuals with cystic fibrosis and persistent Pseudomonas aeruginosa infection who are aged six years or older. The aim is to reduce bacterial load in the lungs so as to reduce inflammation and deterioration of lung function. This is an update of a previously published review...
March 30, 2018: Cochrane Database of Systematic Reviews
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