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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/29333815/prevalence-of-vitamin-k-deficiency-and-associated-factors-in-non-supplemented-cystic-fibrosis-patients
#1
Patrycja Krzyżanowska, Sławomira Drzymala-Czyż, Nataliya Rohovyk, Lyudmyla Bober, Jerzy Moczkco, Marta Rachel, Jarosław Walkowiak
INTRODUCTION: Vitamin K deficiency is highly prevalent in cystic fibrosis (CF) patients despite supplementation. Moreover, no reliable risk factors for its occurrence are known. The aim was to assess the prevalence of vitamin K deficiency and associated factors in non-supplemented CF patients. METHODS: Prothrombin concentration induced by vitamin K absence (PIVKA-II) and the undercarboxylated osteocalcin percentage (u-OC) were determined. In all patients clinical status was assessed and its relation to vitamin K deficiency determined...
February 1, 2018: Archivos Argentinos de Pediatría
https://www.readbyqxmd.com/read/29303286/-cystic-fibrosis-in-adults
#2
Libor Fila
Cystic fibrosis (CF) is an inherited disease caused by mutations in the transmembrane conductance regulator (CFTR) gene. The disease leads to dysfunction of the exocrine glands with high concentration of chloride in the sweat and formation of abnormally viscous mucus in the respiratory, digestive and reproductive tract. Chronic sinopulmonary disease, exocrine pancreatic insufficiency, liver disease, intestinal obstruction, impaired nutritional status, salt loss syndrome and male infertility dominates in the clinical presentation...
2018: Vnitr̆ní Lékar̆ství
https://www.readbyqxmd.com/read/29277839/effect-of-synbiotic-supplementation-in-children-and-adolescents-with-cystic-fibrosis-a-randomized-controlled-clinical-trial
#3
Maiara Brusco de Freitas, Emilia Addison Machado Moreira, Diane de Lima Oliveira, Camila Tomio, Julia Salvan da Rosa, Yara Maria Franco Moreno, Eliana Barbosa, Norberto Ludwig Neto, Vittoria Buccigrossi, Alfredo Guarino, Tânia Silvia Fröde
BACKGROUND/OBJECTIVES: Cystic fibrosis (CF) is characterized by excessive activation of immune processes. The aim of this study was to evaluate the effect of synbiotic supplementation on the inflammatory response in children/adolescents with CF. SUBJECTS/METHODS: A randomized, placebo-controlled, double-blind, clinical-trial was conducted with control group (CG, n = 17), placebo-CF-group (PCFG, n = 19), synbiotic CF-group (SCFG, n = 22), PCFG negative (n = 8) and positive (n = 11) bacteriology, and SCFG negative (n = 12) and positive (n = 10) bacteriology...
December 26, 2017: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/29232848/a-metagenomic-and-in-silico-functional-prediction-of-gut-microbiota-profiles-may-concur-in-discovering-new-cystic-fibrosis-patient-targeted-probiotics
#4
Pamela Vernocchi, Federica Del Chierico, Andrea Quagliariello, Danilo Ercolini, Vincenzina Lucidi, Lorenza Putignani
Cystic fibrosis (CF) is a life-limiting hereditary disorder that results in aberrant mucosa in the lungs and digestive tract, chronic respiratory infections, chronic inflammation, and the need for repeated antibiotic treatments. Probiotics have been demonstrated to improve the quality of life of CF patients. We investigated the distribution of gut microbiota (GM) bacteria to identify new potential probiotics for CF patients on the basis of GM patterns. Fecal samples of 28 CF patients and 31 healthy controls (HC) were collected and analyzed by 16S rRNA-based pyrosequencing analysis of GM, to produce CF-HC paired maps of the distribution of operational taxonomic units (OTUs), and by Phylogenetic Investigation of Communities by Reconstruction of Unobserved States (PICRUSt) for Kyoto Encyclopedia of Genes and Genomes (KEGG) biomarker prediction...
December 9, 2017: Nutrients
https://www.readbyqxmd.com/read/29220199/risk-factors-for-gaps-in-care-during-transfer-from-pediatric-to-adult-cystic-fibrosis-programs-in-the-united-states
#5
Gregory S Sawicki, Joshua Ostrenga, Kristofer Petren, Aliza K Fink, Emma D'Agostino, Camila Strassle, Michael S Schechter, Margaret Rosenfeld
RATIONALE: With improved survival into adulthood, the number of dedicated adult cystic fibrosis (CF) care programs has expanded in the US over the past decade. Transfer from pediatric to adult CF programs represents a potential time for lapses in recommended healthcare. OBJECTIVES: To describe variability in transfer between pediatric and adult CF care programs and to identify factors associated with prolonged gaps in care. METHODS: Using the US CF Foundation Patient Registry (CFFPR), we identified individuals with CF who transferred care from a pediatric to adult CF care program during 2007-2013...
December 8, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/29214071/is-bronchoscopy-an-obsolete-tool-in-cystic-fibrosis-the-role-of-bronchoscopy-in-cystic-fibrosis-and-its-clinical-use
#6
REVIEW
Lisa Paul
Cystic fibrosis (CF) is a progressive life threatening multisystem genetic disease which affects the CF transmembrane conductance regulator channel. Respiratory causes remain the most common mortality in CF. With the onset of newborn screening, initiating treatments both for prophylaxis and disease management, optimizing nutritional support, and developing therapies targeting CF transmembrane conductance regulator protein, this has significantly changed the face of managing this devastating disease. Bronchoscopy and related procedures such as bronchoalveolar lavage (BAL), transbronchial biopsies, and protected brush sampling have been looked at in the management of CF as patients with CF continue to live longer with the help of newer therapies, the microbiome in the lung becomes less diverse along with increased occurrences for noninfectious causes of airway diseases...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29173443/omalizumab-treatment-for-allergic-bronchopulmonary-aspergillosis-in-young-patients-with-cystic-fibrosis
#7
Caroline Perisson, Leila Destruys, Dominique Grenet, Laurence Bassinet, Jocelyne Derelle, Isabelle Sermet-Gaudelus, Caroline Thumerelle, Anne Prevotat, Vincent Rosner, Annick Clement, Harriet Corvol
BACKGROUND: Allergic bronchopulmonary aspergillosis (ABPA) is a severe lung disease complication caused by an Aspergillus fumigatus-induced hypersensitivity that affects 2-15% of patients with cystic fibrosis (CF). The mainstay treatment consists of a combination of corticosteroids and antifungals. However, repeated or long-term corticosteroid therapies can lead to serious side effects. The monoclonal anti-IgE antibody, omalizumab, has demonstrated its efficacy in allergic asthma. As ABPA results from a hypersensitivity to a specific allergen, omalizumab might benefit CF patients with ABPA...
December 2017: Respiratory Medicine
https://www.readbyqxmd.com/read/29140739/cftr-genotype-and-maximal-exercise-capacity-in-cystic-fibrosis-a-cross-sectional-study
#8
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
#9
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 (6×109CFU/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
#10
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
#11
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
#12
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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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