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cystic fibrosis nutrition

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https://www.readbyqxmd.com/read/28093464/socioeconomic-status-smoke-exposure-and-health-outcomes-in-young-children-with-cystic-fibrosis
#1
Thida Ong, Michael Schechter, Jing Yang, Limin Peng, Julia Emerson, Ronald L Gibson, Wayne Morgan, Margaret Rosenfeld
BACKGROUND: Lower socioeconomic status (SES) and environmental tobacco smoke (ETS) exposure are both associated with poorer disease outcomes in cystic fibrosis (CF), and children with low SES are disproportionately exposed to ETS. We analyzed a large cohort of young children with CF to distinguish the impact of SES and ETS on clinical outcomes. METHODS: The Early Pseudomonas Infection Control Observational study enrolled Pseudomonas-negative young children with CF <13 years of age...
January 16, 2017: Pediatrics
https://www.readbyqxmd.com/read/28086849/successful-outcome-following-pneumonectomy-in-a-teenage-boy-with-cystic-fibrosis-a-case-report
#2
Zheyi Liew, Santosh Mallikarjuna, Asif Hasan, F Kate Gould, Su Bunn, Matthew F Thomas, Jim L Lordan, Christopher O'Brien, Malcolm Brodlie
BACKGROUND: Cystic fibrosis lung disease is generally a diffuse process however rarely one lung may become particularly damaged through chronic collapse and consolidation resulting in end-stage bronchiectasis with relative sparing of the contralateral lung. This clinical situation is sometimes referred to as "destroyed lung". Lung resection surgery is seldom indicated in cystic fibrosis and the associated medical literature is relatively sparse. CASE PRESENTATION: A 14 year old boy was referred to our centre for lung transplantation assessment...
January 13, 2017: BMC Pulmonary Medicine
https://www.readbyqxmd.com/read/28078087/clostridium-difficile-infection-in-cystic-fibrosis-an-uncommon-but-life-threatening-complication
#3
Francesco Piccolo, Anna Sze Tai, Hooi Ee, Siobhain Mulrennan, Scott Bell, Gerard Ryan
Adults with cystic fibrosis (CF) have significant rates of asymptomatic Clostridium difficile carriage and are frequently exposed to risk factors for C. difficile infection (CDI). Despite this, the rate of reported CDI in CF is low. We describe three cases of near fatal CDI in adults with CF and review the literature regarding presentation, management, and recurrence prevention. Early recognition is important as the clinical presentation may be atypical and the illness can be severe and even life-threatening...
January 2017: Respirology Case Reports
https://www.readbyqxmd.com/read/28052955/hypoxia-and-sterile-inflammation-in-cystic-fibrosis-airways-mechanisms-and-potential-therapies
#4
REVIEW
Samuel T Montgomery, Marcus A Mall, Anthony Kicic, Stephen M Stick
Cystic fibrosis is one of the most common autosomal recessive genetic diseases in Caucasian populations. Diagnosis via newborn screening and targeted nutritional and antibiotic therapy have improved outcomes, however respiratory failure remains the key cause of morbidity and mortality. Progressive respiratory disease in cystic fibrosis is characterised by chronic neutrophilic airway inflammation associated with structural airway damage leading to bronchiectasis and decreased lung function. Mucus obstruction is a characteristic early abnormality in the cystic fibrosis airway, associated with neutrophilic inflammation often in the absence of detectable infection...
January 2017: European Respiratory Journal: Official Journal of the European Society for Clinical Respiratory Physiology
https://www.readbyqxmd.com/read/28049743/alternative-indexes-to-estimate-the-functional-capacity-from-the-6-minute-walk-test-in-children-and-adolescents-with-cystic-fibrosis
#5
Renata Tiemi Okuro, Maria Angela Gonçalves de Oliveira Ribeiro, José Dirceu Ribeiro, Rafaela Coelho Minsky, Camila Isabel Santos Schivinski
BACKGROUND: Cystic fibrosis is a multi-systemic disease related to reduced functional capacity. The distance covered in the 6-min walk test (6MWT) has been known to assess functional capacity, but little is known about other indexes that can be derived. We sought to compare the performance during the 6MWT and the estimated indexes of functional capacity from the 6MWT between subjects with cystic fibrosis (CF) and healthy individuals as well as to assess the relationship among these indexes and disease severity, pulmonary function, and nutritional status in CF...
January 3, 2017: Respiratory Care
https://www.readbyqxmd.com/read/28000919/antibiotic-treatment-for-nontuberculous-mycobacteria-lung-infection-in-people-with-cystic-fibrosis
#6
REVIEW
Valerie Waters, Felix Ratjen
BACKGROUND: Nontuberculous mycobacteria are mycobacteria, other than those in the Mycobacterium tuberculosis complex, and are commonly found in the environment. Nontuberculous mycobacteria species (most commonly Mycobacterium avium complex and Mycobacterium abscessus) are isolated from the respiratory tract of approximately 5% to 40% of individuals with cystic fibrosis; they can cause lung disease in people with cystic fibrosis leading to more a rapid decline in lung function and even death in certain circumstances...
December 19, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27966575/clinical-impact-of-vitamin-d-treatment-in-cystic-fibrosis-a-pilot-randomized-controlled-trial
#7
T Pincikova, D Paquin-Proulx, J K Sandberg, M Flodström-Tullberg, L Hjelte
BACKGROUND/OBJECTIVES: Vitamin D insufficiency in cystic fibrosis is common. Vitamin D3 is currently preferred over D2. We aimed to study the efficacy of vitamin D2 and D3 at increasing serum 25-hydroxyvitamin D (s25OHD) concentrations and their effect on respiratory health in cystic fibrosis. SUBJECTS/METHODS: Sixteen CF patients were randomized to receive vitamin D2 or D3 or to serve as controls. The starting dose of 5000 IU (<16 years old) or 7143 IU/day (⩾16 years old) was further individually adjusted...
December 14, 2016: European Journal of Clinical Nutrition
https://www.readbyqxmd.com/read/27922234/long-term-azithromycin-therapy-in-patients-with-cystic-fibrosis
#8
Nagehan Emiralioğlu, Zeynelabidin Öztürk, Ebru Yalçın, Deniz Doğru, Uğur Özçelik, Nural Kiper
Inflammation is a central contributor to the pathogenesis of cystic fibrosis (CF) pulmonary disease; so limiting the excessive production of inflammatory mediators represents a major therapeutic strategy for slowing the decline in lung function and improving survival. The macrolide antibiotic azithromycin (AZM) has anti-inflammatory properties and immunomodulatory effects that may be beneficial in CF. The aim of this study was to document the long term use of AZM effect on pulmonary function, nutritional status and number of pulmonary exacerbations in patients with CF...
2016: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/27916550/bmi-fails-to-identify-poor-nutritional-status-in-stunted-children-with-cf
#9
Michael W Konstan, David J Pasta, Jeffrey S Wagener, Donald R VanDevanter, Wayne J Morgan
BACKGROUND: Body mass index (BMI) is currently emphasized for evaluating nutritional status in children with cystic fibrosis (CF). Weight for age (WFA) and height for age (HFA) may get less attention. METHODS: Data from the Epidemiologic Study of Cystic Fibrosis were used to compare patient WFA, HFA, and BMI percentiles for children age 2 to 18years. RESULTS: For children with BMI between the 25th and 50th percentiles, 16.8% had WFA <10th percentile and 26...
December 1, 2016: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/27878805/pancreatic-enzyme-replacement-therapy-for-people-with-cystic-fibrosis
#10
REVIEW
Usha Rani Somaraju, Arturo Solis-Moya
BACKGROUND: Most people with cystic fibrosis (80% to 90%) need pancreatic enzyme replacement therapy to prevent malnutrition. Enzyme preparations need to be taken whenever food is taken, and the dose needs to be adjusted according to the food consumed. A systematic review on the efficacy and safety of pancreatic enzyme replacement therapy is needed to guide clinical practice, as there is variability between centres with respect to assessment of pancreatic function, time of commencing treatment, dose and choice of supplements...
23, 2016: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/27873431/hypoglycemia-is-common-in-children-with-cystic-fibrosis-and-seen-predominantly-in-females
#11
Belma Haliloglu, Yasemin Gokdemir, Zeynep Atay, Saygin Abali, Tulay Guran, Fazilet Karakoc, Refika Ersu, Bulent Karadag, Serap Turan, Abdullah Bereket
OBJECTIVE: To determine the prevalence of hypoglycemia in children and adolescents with cystic fibrosis (CF) in 2-hour oral glucose tolerance test (OGTT) and continuous glucose monitoring (CGM) under free-living conditions. RESEARCH DESIGN AND METHODS: Height, weight, body mass index (BMI), hemoglobin A1c (HbA1c), and Forced expiratory volume (FEV1%) were measured in children with CF (aged 5-18 years). Following OGTT, CGM was installed for 3 days. The total hypoglycemic and hyperglycemic time (%) during 3 days was measured...
November 22, 2016: Pediatric Diabetes
https://www.readbyqxmd.com/read/27859825/do-children-with-cystic-fibrosis-receiving-outreach-care-have-poorer-clinical-outcomes-than-those-treated-at-a-specialist-cystic-fibrosis-centre
#12
Heinrich C Weber, Philip F Robinson, Nicole Saxby, Sean A Beggs, Ingrid Els, Rodney I Ehrlich
INTRODUCTION: Although cystic fibrosis (CF) centre care is generally considered ideal, children living in regional Australia receive outreach care supported by the academic CF centres. METHODS: This is a retrospective database review of children with CF treated at the Royal Children's Hospital in Melbourne and its outreach clinics in Albury (Victoria), and Tasmania. The aim was to compare the outcomes of children with CF managed at an academic centre with that of outreach care, using lung function, nutritional status and Pseudomonas aeruginosa colonisation...
November 17, 2016: Australian Journal of Rural Health
https://www.readbyqxmd.com/read/27834866/staphylococcus-aureus-infection-reduces-nutrition-uptake-and-nucleotide-biosynthesis-in-a-human-airway-epithelial-cell-line
#13
Philipp Gierok, Manuela Harms, Karen Methling, Falko Hochgräfe, Michael Lalk
The Gram positive opportunistic human pathogen Staphylococcus aureus induces a variety of diseases including pneumonia. S. aureus is the second most isolated pathogen in cystic fibrosis patients and accounts for a large proportion of nosocomial pneumonia. Inside the lung, the human airway epithelium is the first line in defence with regard to microbial recognition and clearance as well as regulation of the immune response. The metabolic host response is, however, yet unknown. To address the question of whether the infection alters the metabolome and metabolic activity of airway epithelial cells, we used a metabolomics approach...
November 9, 2016: Metabolites
https://www.readbyqxmd.com/read/27805836/lumacaftor-ivacaftor-in-patients-aged-6-11-years-with-cystic-fibrosis-homozygous-for-f508del-cftr
#14
Carlos E Milla, Felix Ratjen, Gautham Marigowda, Fang Liu, David Waltz, Margaret Rosenfeld
RATIONALE: Combination lumacaftor/ivacaftor has been shown to improve lung function and other endpoints in patients aged ≥12 years with cystic fibrosis homozygous for F508del-CFTR but has not been assessed in younger patients. OBJECTIVES: This open-label phase 3 trial evaluated the safety, tolerability, pharmacodynamics, and efficacy of lumacaftor/ivacaftor combination therapy in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR. METHODS: Patients (N = 58) received 200 mg lumacaftor/250 mg ivacaftor orally every 12 hours for 24 weeks in addition to their existing cystic fibrosis medications...
November 2, 2016: American Journal of Respiratory and Critical Care Medicine
https://www.readbyqxmd.com/read/27797455/implementation-of-cystic-fibrosis-clinical-pathways-improved-physician-adherence-to-care-guidelines
#15
Sachinkumar B Singh, Annie U Shelton, Barbara Greenberg, Timothy D Starner
INTRODUCTION: There is significant variability in clinical outcomes, including growth and lung function, between the various cystic fibrosis (CF) centers. No specific or unique therapeutic practices have been identified to account for these differences. However, more uniform care within centers was associated with better outcomes. The objective of this study was to implement clinical pathways for diagnosis and treatment of nutritional failure and lung inflammation in order to achieve better health care provider adherence to center-specific, agreed-on practices...
October 31, 2016: Pediatric Pulmonology
https://www.readbyqxmd.com/read/27784314/quality-of-sweat-test-st-based-on-the-proportion-of-sweat-sodium-na-and-sweat-chloride-cl-as-diagnostic-parameter-of-cystic-fibrosis-are-we-on-the-right-way
#16
Alethéa Guimarães Faria, Fernando Augusto Lima Marson, Carla Cristina de Souza Gomez, Maria Ângela Gonçalves de Oliveira Ribeiro, Lucas Brioschi Morais, Maria de Fátima Servidoni, Carmen Sílvia Bertuzzo, Eulália Sakano, Maura Goto, Ilma Aparecida Paschoal, Mônica Corso Pereira, Gabriel Hessel, Carlos Emílio Levy, Adyléia Aparecida Dalbo Contrera Toro, Andressa Oliveira Peixoto, Maria Cristina Ribeiro Simões, Elizete Aparecida Lomazi, Roberto José Negrão Nogueira, Antônio Fernando Ribeiro, José Dirceu Ribeiro
BACKGROUND: To assess the quality of sweat test (ST) based on the proportion of sweat sodium and sweat chloride as diagnostic parameter of cystic fibrosis (CF). METHODS: A retrospective study of 5,721 sweat samples and subsequent descriptive analysis were carried out. The test was considered "of good quality" (correct) when: (i) sweat chloride was lower than 60 mEq/L, and sweat sodium was higher than sweat chloride; (ii) sweat chloride was higher than 60 mEq/L, and sweat sodium was lower than sweat chloride...
October 26, 2016: Diagnostic Pathology
https://www.readbyqxmd.com/read/27782961/using-the-objective-structured-clinical-exam-osce-to-assess-acgme-competencies-in-pediatric-gastroenterology-fellows
#17
Aliza B Solomon, Rachel Reed, Keith Benkov, Joseph Kingsbery, Sarah S Lusman, Lisa B Malter, Jeremiah Levine, Simon Rabinowitz, Martin Wolff, Sondra Zabar, Elizabeth Weinshel
BACKGROUND: The Accreditation Council for Graduate Medical Education (ACGME) has described six core competencies with which trainees should demonstrate proficiency. Using the Objective Structured Clinical Exam (OSCE), we aimed to assess four of these competencies among Pediatric GI fellows (PG). METHODS: Eight first-year PG's from six medical centers in the New York area participated in a four-station OSCE with trained standardized patient (SP) actors. The cases included an "ED Consult" for lower GI bleeding; "Breaking Bad News" focusing on CF nutritional complications; "Second Opinion" for abdominal pain; "Transition of Care" for inflammatory bowel disease...
October 25, 2016: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/27754353/the-impact-of-secondhand-smoke-exposure-on-children-with-cystic-fibrosis-a-review
#18
Benjamin T Kopp, Juan Antonio Ortega-García, S Christy Sadreameli, Jack Wellmerling, Estelle Cormet-Boyaka, Rohan Thompson, Sharon McGrath-Morrow, Judith A Groner
Secondhand smoke exposure (SHSe) has multiple adverse effects on lung function and growth, nutrition, and immune function in children; it is increasingly being recognized as an important modifier of disease severity for children with chronic diseases such as cystic fibrosis (CF). This review examines what is known regarding the prevalence of SHSe in CF, with the majority of reviewed studies utilizing parental-reporting of SHSe without an objective biomarker of exposure. A wide range of SHSe is reported in children with CF, but under-reporting is common in studies involving both reported and measured SHSe...
October 12, 2016: International Journal of Environmental Research and Public Health
https://www.readbyqxmd.com/read/27752941/a-practical-approach-to-glucose-abnormalities-in-cystic-fibrosis
#19
REVIEW
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...
December 2016: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
https://www.readbyqxmd.com/read/27740724/innovative-assessment-of-inpatient-and-pulmonary-drug-costs-for-children-with-cystic-fibrosis
#20
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...
December 2016: Pediatric Pulmonology
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