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cystic fibrosis nutrition

Nyanza K L M Timmers, Rebecca K Stellato, Cornelis K van der Ent, Roderick H J Houwen, Janna W Woestenenk
Pancreatic-insufficient children with cystic fibrosis (CF) receive age-group-specific vitamin D supplementation according to international CF nutritional guidelines. The potential advantageous immunomodulatory effect of serum 25-hydroxy vitamin D (25(OH)D) on pulmonary function (PF) is yet to be established and is complicated by CF-related vitamin D malabsorption. We aimed to assess whether current recommendations are optimal for preventing deficiencies and whether higher serum 25(OH)D levels have long-term beneficial effects on PF...
November 16, 2018: British Journal of Nutrition
Ana Luiza Melo Dos Santos, Helen de Melo Santos, Marina Bettiol Nogueira, Hugo Tadashi Oshiro Távora, Maria de Lourdes Jaborandy Paim da Cunha, Renata Belém Pessoa de Melo Seixas, Luciana de Freitas Velloso Monte, Elisa de Carvalho
Purpose: The objective of this study was to describe the clinical phenotypes of children and adolescents with cystic fibrosis (CF); and to assess the role of pancreatic insufficiency and neonatal screening in diagnosis. Methods: A cross-sectional study was conducted, which included 77 patients attending a reference center of CF between 2014 and 2016. Epidemiological data, anthropometric measurements, and the presence of pulmonary, pancreatic, gastrointestinal and hepatobiliary manifestations were evaluated based on clinical data and complementary examinations...
October 2018: Pediatric Gastroenterology, Hepatology & Nutrition
Aliye Uc, Dana K Andersen, Drucy Borowitz, Marshall J Glesby, Julia Mayerle, Robert Sutton, Stephen J Pandol
A workshop was sponsored by the Division of Digestive Diseases and Nutrition, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, on July 25, 2018, in Pittsburgh, Penn. The workshop was designed to bring together a multidisciplinary group of experts to accelerate the development of therapeutics for clinical application in inflammatory diseases of the exocrine pancreas. Three separate working groups (acute pancreatitis, recurrent acute pancreatitis, and chronic pancreatitis) were formed to address the needs, gaps, and opportunities...
November 2018: Pancreas
Helge Hebestreit, Erik Hj Hulzebos, Jane E Schneiderman, Chantal Karila, Steven R Boas, Susi Kriemler, Tiffany Dwyer, Margareta Sahlberg, Don S Urquhart, Larry C Lands, Felix Ratjen, Tim Takken, Liobou Varanistkaya, Viktoria Rücker, Alexandra Hebestreit, Jakob Usemann, Thomas Radtke
RATIONALE: The prognostic value of cardiopulmonary exercise testing (CPET) for survival in cystic fibrosis (CF) in the context of current clinical management, when controlling for other known prognostic factors is unclear. OBJECTIVES: To determine the prognostic value of CPET-derived measures beyond peak oxygen uptake (VO2peak) following rigorous adjustment for other predictors. MEASUREMENTS AND MAIN RESULTS: Data from 10 CF-centers in Australia, Europe and North America were collected retrospectively...
October 15, 2018: American Journal of Respiratory and Critical Care Medicine
Thomas J C Ruffles, Ryan Black, Wayne Nicholls, Barbara Laing, Alan Isles
Introduction: We describe the case of a 16-year old male with cystic fiborosis (CF) who presented with an osteosarcoma of his right distal tibia. Case Report: Treatment consisted of neoadjuvant chemotherapy of cisplatin, doxorubicin and high dose methotrexate followed by distal tibial resection and free fibula flap reconstruction and consolidation chemotherapy. Treatment was complicated by a pulmonary exacerbation, where Pseudomonas aeruginosa (PsA) and Staphylococcus aureus were grown on sputum culture which was treated with a 2-week course of intravenous piptazobactam and tobramycin...
2018: Frontiers in Pediatrics
Sophie Corriveau, Jenna Sykes, Anne L Stephenson
PURPOSE OF REVIEW: Tracking patient outcomes using cystic fibrosis (CF) national data registries, we have seen a dramatic improvement in patient survival. As there are multiple ways to measure survival, it is important for readers to understand these different metrics in order to clearly translate this information to patients and their families. The aims of this review were to describe measures of survival and to review the recent literature pertaining to survival in CF to capture the changing epidemiology...
November 2018: Current Opinion in Pulmonary Medicine
Alana Schick, Rees Kassen
Chronic infection of the cystic fibrosis (CF) airway by the opportunistic pathogen Pseudomonas aeruginosa is the leading cause of morbidity and mortality for adult CF patients. Prolonged infections are accompanied by adaptation of P. aeruginosa to the unique conditions of the CF lung environment, as well as marked diversification of the pathogen into phenotypically and genetically distinct strains that can coexist for years within a patient. Little is known, however, about the causes of this diversification and its impact on patient health...
October 16, 2018: Proceedings of the National Academy of Sciences of the United States of America
Christopher H Goss, Yukihiro Kaneko, Lisa Khuu, Gail D Anderson, Sumedha Ravishankar, Moira L Aitken, Noah Lechtzin, Guolin Zhou, Daniel M Czyz, Kathryn McLean, Oyebode Olakanmi, Howard A Shuman, Mary Teresi, Ellen Wilhelm, Ellen Caldwell, Stephen J Salipante, Douglas B Hornick, Richard J Siehnel, Lev Becker, Bradley E Britigan, Pradeep K Singh
The lack of new antibiotics is among the most critical challenges facing medicine. The problem is particularly acute for Gram-negative bacteria. An unconventional antibiotic strategy is to target bacterial nutrition and metabolism. The metal gallium can disrupt bacterial iron metabolism because it substitutes for iron when taken up by bacteria. We investigated the antibiotic activity of gallium ex vivo, in a mouse model of airway infection, and in a phase 1 clinical trial in individuals with cystic fibrosis (CF) and chronic Pseudomonas aeruginosa airway infections...
September 26, 2018: Science Translational Medicine
Manuele Furnari, Alessandra De Alessandri, Federico Cresta, Maria Haupt, Marta Bassi, Angela Calvi, Riccardo Haupt, Giorgia Bodini, Iftikhar Ahmed, Francesca Bagnasco, Edoardo Giovanni Giannini, Rosaria Casciaro
BACKGROUND: Scientific literature shows a high prevalence of Small Intestinal Bacterial Overgrowth (SIBO) in patients with Cystic Fibrosis (CF). The role of SIBO in nutritional status and gastrointestinal symptoms in CF is not known. Our aim was to study epidemiology and clinical impact of SIBO while assessing the efficacy of rifaximin in eradicating SIBO in CF patients. METHODS: Symptoms questionnaire and Glucose Breath Test (GBT) were given to 79 CF patients (median age 19...
September 19, 2018: Journal of Gastroenterology
Katharina Staufer, Emina Halilbasic, Peter Hillebrand, Solveig Harm, Stefan Schwarz, Peter Jaksch, Danijel Kivaranovic, Walter Klepetko, Michael Trauner, Lili Kazemi-Shirazi
Background: Nutritional status is an important prognostic factor in patients with cystic fibrosis (CF) prior to lung transplantation. Objective: To investigate the impact of nutritional status on pulmonary function in CF transplant recipients. Methods: Adult double lung transplanted CF patients were consecutively included. The predictive value of nutritional status on lung function - measured by spirometry - was longitudinally assessed by body composition serially evaluated by a three-compartment model bioelectrical impedance analysis (BIA) in comparison to body mass index (BMI)...
August 2018: United European Gastroenterology Journal
Chinyere Onyearugbulem, Lauren Williams, Huirong Zhu, Maria C Gazzaneo, Ernestina Melicoff, Shailendra Das, Jorge Coss-Bu, Fong Lam, George Mallory, Flor M Munoz
Although infection is the leading cause of death in the first year following pediatric lung transplantation, there are limited data on risk factors for early infection. Sepsis remains under-recognized and under-reported in the early post-operative period for lung transplant recipients (LTR). We evaluated the incidence of infection and sepsis, and identified risk factors for infection in the early post-operative period in pediatric LTRs. A retrospective review of medical records of LTRs at a large quaternary-care hospital from January 2009 to March 2016 was conducted...
September 17, 2018: Transplant Infectious Disease: An Official Journal of the Transplantation Society
Kamini Jain, Claire Wainwright, Alan R Smyth
BACKGROUND: Early diagnosis and treatment of lower respiratory tract infections are the mainstay of management of lung disease in cystic fibrosis. When sputum samples are unavailable, treatment relies mainly on cultures from oropharyngeal specimens; however, there are concerns regarding the sensitivity of these to identify lower respiratory organisms.Bronchoscopy and related procedures (including bronchoalveolar lavage) though invasive, allow the collection of lower respiratory specimens from non-sputum producers...
September 17, 2018: Cochrane Database of Systematic Reviews
Joaquim Calvo-Lerma, Victoria Fornés-Ferrer, Ana Heredia, Ana Andrés
In vitro digestion research has scarcely addressed the assessment of the complexity of digestion in real food. The aim of the present study was to evaluate the influence of intestinal conditions, nonlipid components, and lipid organization within the food matrix on lipolysis extent. A selection of 52 foods was studied under different simulated intestinal conditions, including those related to patients with cystic fibrosis (pH6, bile salts 1 mM due to decreased pancreatic and biliary secretions) and to healthy subjects (pH7, bile salts 10 mM)...
October 2018: Journal of Food Science
Ala Shaikhkhalil, Candi Jump, Praveen S Goday
Structured nutrition rotations are rarely offered in pediatric gastroenterology fellowships. The NASPGHAN Nutrition Committee developed a curriculum to serve as the basis for a rotation in clinical nutrition. We worked directly with five fellowship programs to tailor the experience to individual institutions. As part of our pilot study, fellows completed knowledge assessments and self-assessment of comfort level at the start and end of the experience. We saw a trend in improvement of comfort level and increase in mean score on knowledge assessments, but the differences did not meet statistical significance...
September 11, 2018: Journal of Pediatric Gastroenterology and Nutrition
C Lehoux Dubois, E Labrèche, V Boudreau, J Colomba, M Mailhot, A Lavoie, R Rabasa-Lhoret, A Coriati
BACKGROUND: Due to lack of vitamin D absorption in patients with cystic fibrosis (CF), vitamin D supplementation becomes necessary. Our aim was to study the association between serum vitamin D levels and key clinical factors, such as nutritional status, pulmonary function and pulmonary exacerbations (PEx) frequency, in an adult CF population. METHODS: Prospective analysis of a published vitamin D (VitD3 ) supplementation protocol (N = 200 adult patients) over a follow-up period of 5 years...
August 25, 2018: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
Rasa Ruseckaite, Natalie Pekin, Susannah King, Erin Carr, Susannah Ahern, John Oldroyd, Arul Earnest, Claire Wainwright, David Armstrong
OBJECTIVES: To determine the association between the implementation of the 2006 Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis (CF) and the nutritional status of children participating in the Australian Cystic Fibrosis Data Registry (ACFDR). METHODS: This research consisted of a quantitative study using ACFDR data and a survey of clinicians and dietitians treating children with CF. Two independent cohorts of children (2-5 years and 6-11 years) were selected from ACFDR between 1998 and 2014 (N = 2304)...
September 2018: Respiratory Medicine
Niovi Papalexopoulou, Theodore G Dassios, Alan Lunt, Fiona Bartlett, Felicity Perrin, Cara J Bossley, Hilary A Wyatt, Anne Greenough
BACKGROUND: Nutrition is closely related to mortality and pulmonary and respiratory muscle function in cystic fibrosis (CF) patients. We initially validated results from a bioelectrical impedance device against dual energy x-ray absorptiometry (DEXA). We then determined whether fat free mass assessed by a portable impedance device rather than body mass index (BMI) better correlated with pulmonary function, respiratory muscle strength and exercise capacity in CF patients. METHODS: Eighteen young people and adults (median age 19, range 12-39 years) with CF had dual energy X-ray absorptiometry and direct segmental multi-frequency impedance analysis...
September 2018: Respiratory Medicine
Stephanie Van Biervliet, Bruno Hauser, Stijn Verhulst, Hedwig Stepman, Joris Delanghe, Jean-Pol Warzee, Bruno Pot, Tom Vandewiele, Michael Wilschanski
BACKGROUND: A potential positive effect of probiotics in cystic fibrosis (CF) on fecal calprotectin (FCP), pulmonary exacerbations and weight has been described in small controlled trials. METHODS: A double-blind multicenter cross-over study (2 × 4 m) was performed looking at abdominal pain, nutritional status, pulmonary function, pulmonary exacerbation, FCP and lactulose/mannitol gut permeability test. Patients kept a diary with daily scoring of abdominal pain, stool frequency and consistency as well as treatment changes...
October 2018: Clinical Nutrition ESPEN
Lenycia de Cassya Lopes Neri, Denise Pimentel Bergamaschi, Luiz Vicente Ribeiro Ferreira da Silva Filho
OBJECTIVE: To evaluate the nutritional profile of the population assisted at a reference center for cystic fibrosis treatment. METHODS: Cross-sectional study including patients with cystic fibrosis assisted at a pediatric reference center in São Paulo, Brazil, in 2014. All patients attending regular visits who agreed to participate in the study were included. A questionnaire on dietary habits (24-hour diet recall) and socioeconomic characteristics was applied. Anthropometric data (compared with the reference from the World Health Organization, 2006 and 2007) and pulmonary function data were collected from medical records...
August 9, 2018: Revista Paulista de Pediatria: Orgão Oficial da Sociedade de Pediatria de São Paulo
David Gomez-Pastrana, Chinedu Nwokoro, Mike McLean, Sarah Brown, Nanna Christiansen, Caroline S Pao
INTRODUCTION: Ivacaftor is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator that has been shown to improve the nutritional status and lung function of cystic fibrosis patients with the G551D mutation in clinical trials. The objective of this study was to describe the real-world progress of children receiving ivacaftor. METHODS: We describe the real-world progress of four children with cystic fibrosis and the F508del/G551D genotype comparing data during ivacaftor treatment with baseline and with the year before commencing treatment...
August 6, 2018: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
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