keyword
MENU ▼
Read by QxMD icon Read
search

cystic fibrosis nutrition

keyword
https://www.readbyqxmd.com/read/28910260/shining-new-light-on-newborn-screening-of-cystic-fibrosis-in-the-province-of-quebec
#1
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
https://www.readbyqxmd.com/read/28886227/lung-transplantation-in-cystic-fibrosis-patients-in-israel-the-importance-of-ethnicity-and-nutritional-status
#2
Hagit Levine, Dario Prais, Yael Raviv, Victorya Rusanov, Dror Rosengarten, Milton Saute, Moshe Hoshen, Huda Mussaffi, Hannah Blau, Mordechai R Kramer
OBJECTIVES: To assess the characteristics that correlate with better outcomes after lung transplantation for patients with cystic fibrosis (CF). METHODS: We retrospectively reviewed the charts of all patients with CF who underwent lung transplantation between 1996-2014 at Rabin Medical Center, Israel. RESULTS: Fifty patients with CF underwent 55 lung transplantations. Eighteen patients (36%) died during the study period. Actuarial survival was 83%, 68%, 62% and 39% at 1, 3, 5 and 10 years, respectively...
September 8, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/28845426/lung-function-and-disease-severity-in-cystic-fibrosis-patients-heterozygous-for-p-arg117his
#3
Michal Shteinberg, Damian G Downey, Diane Beattie, John McCaughan, Alastair Reid, Nili Stein, J Stuart Elborn
Expression of p.Arg117His cystic fibrosis (CF) transmembrane conductance regulator is influenced by a polythymidine (poly-T) tract and a thymidine-guanine (TG) repeat on intron 9, which vary in length and affect exon 10 skipping. We compared clinical characteristics and the rate of progression of lung disease of CF patients carrying the p.Arg117His mutation with different intron 9 varying sequences (poly-T) and mutation classes in trans. Data were collected from patients in Northern Ireland, UK, including diagnostic features, sweat chloride, nutritional status, sputum microbiology, CF-related complications and lung function...
January 2017: ERJ Open Research
https://www.readbyqxmd.com/read/28837442/diagnosis-follow-up-and-treatment-of-cystic-fibrosis-related-liver-disease
#4
Ivo P van de Peppel, Anna Bertolini, Johan W Jonker, Frank A J A Bodewes, Henkjan J Verkade
PURPOSE OF REVIEW: To provide an insight and overview of the challenges in the diagnosis, follow-up and treatment of cystic fibrosis-related liver disease (CFLD). RECENT FINDINGS: The variable pathophysiology of CFLD complicates its diagnosis and treatment. A 'gold standard' for CFLD diagnosis is lacking. Over the past years, new techniques to diagnose features of CFLD, such as transient elastography, have been investigated. Although most of these tests confirm cystic fibrosis-related liver involvement (CFLI), they are, however, not suitable to distinguish various phenotypical presentations or predict progression to clinically relevant cirrhosis or portal hypertension...
August 23, 2017: Current Opinion in Pulmonary Medicine
https://www.readbyqxmd.com/read/28832807/methicillin-resistant-staphylococcus-aureus-in-cystic-fibrosis-patients-do-we-need-to-care-a-cohort-study
#5
Renata Wrobel Folescu Cohen, Tânia Wrobel Folescu, Pedro Daltro, Marcia Cristina Bastos Boechat, Danielle Ferreira Lima, Elizabeth Andrade Marques, Robson Souza Leão
CONTEXT AND OBJECTIVE: The prevalence of a variety of potentially pathogenic microorganisms in cystic fibrosis patients, such as methicillin-resistant Staphylococcus aureus (MRSA), has increased over the past decade. Given the increasing prevalence of MRSA and the few data available in the literature, better understanding of the clinical repercussions of colonization by this bacterium in cystic fibrosis patients becomes essential. This study aimed to evaluate the repercussions of chronic colonization by MRSA in cystic fibrosis patients...
August 21, 2017: São Paulo Medical Journal, Revista Paulista de Medicina
https://www.readbyqxmd.com/read/28814218/diagnosis-and-management-of-pancreatic-exocrine-insufficiency
#6
Mehrdad Nikfarjam, Jeremy S Wilson, Ross C Smith
In 2015, the Australasian Pancreatic Club (APC) published the Australasian guidelines for the management of pancreatic exocrine insufficiency (http://pancreas.org.au/2016/01/pancreatic-exocrine-insufficiency-guidelines). Pancreatic exocrine insufficiency (PEI) occurs when normal digestion cannot be sustained due to insufficient pancreatic digestive enzyme activity. This may be related to a breakdown, at any point, in the pancreatic digestive chain: pancreatic stimulation; synthesis, release or transportation of pancreatic enzymes; or synchronisation of secretions to mix with ingested food...
August 21, 2017: Medical Journal of Australia
https://www.readbyqxmd.com/read/28795206/children-and-adolescents-with-cystic-fibrosis-display-moderate-bone-microarchitecture-abnormalities-data-from-high-resolution-peripheral-quantitative-computed-tomography
#7
C Braun, J Bacchetta, P Braillon, R Chapurlat, J Drai, P Reix
We investigated whether bone microstructure assessed by high-resolution peripheral quantitative tomography (HR-pQCT) could be altered in children and teenagers with cystic fibrosis (CF). In comparison to their healthy counterparts, bone microstructure was mildly affected at the tibial level only. INTRODUCTION: Cystic fibrosis-related bone disease (CFBD) may alter bone health, ultimately predisposing patients to bone fractures. Our aim was to assess bone microstructure using high-resolution peripheral quantitative tomography (HR-pQCT) in a cohort of children and teenagers with CF in comparison to age-, puberty-, and gender-matched healthy volunteers (HVs)...
August 9, 2017: Osteoporosis International
https://www.readbyqxmd.com/read/28770464/long-term-follow-up-in-a-girl-with-cystic-fibrosis-and-diabetes-since-the-first-year-of-life
#8
Valentina Fattorusso, Alida Casale, Valeria Raia, Enza Mozzillo, Adriana Franzese
Diabetes mellitus is the most common comorbidity in cystic fibrosis (CF). Recently, more attention has been paid to early glucose metabolism derangements (GMDs). The subject of this report is a female patient, affected by CF since 3 months of age. She presented with intermittent diabetes during early childhood. At the age of 10 years, oral glucose tolerance test (OGTT) was performed and showed glucose intolerance (IGT) status; glargine insulin therapy was started. At the age of 13 years, CF-related diabetes with fasting hyperglycemia occurred, so rapid insulin at meals was added...
August 2, 2017: Diabetes Therapy: Research, Treatment and Education of Diabetes and related Disorders
https://www.readbyqxmd.com/read/28767774/factors-associated-with-malnutrition-in-adolescent-and-adult-patients-with-cystic-fibrosis
#9
Gabriela Cristofoli Barni, Gabriele Carra Forte, Luis Felipe Forgiarini, Claudine Lacerda de Oliveira Abrahão, Paulo de Tarso Roth Dalcin
Objective: To determine the prevalence of malnutrition in patients attending an adult cystic fibrosis (CF) program and to investigate the associations of malnutrition with the clinical characteristics of those patients. Methods: This was a cross-sectional study involving patients with clinically stable CF patients (16 years of age or older). The patients underwent clinical assessment, nutritional assessments, pulmonary function tests, and pancreatic function assessment...
July 31, 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28747058/pancreatic-enzyme-replacement-therapy-in-cystic-fibrosis-dose-variability-and-coefficient-of-fat-absorption
#10
Joaquim Calvo-Lerma, Sandra Martínez-Barona, Etna Masip, Victoria Fornés, Carmen Ribes-Koninckx
OBJECTIVES: Pancreatic enzyme replacement therapy (PERT) remains a backbone in the nutritional treatment of cystic fibrosis. Currently, there is a lack of an evidence-based tool that allows dose adjustment. To date, no studies have found an association between PERT dose and fat absorption. Therefore, the aim of the study was to assess the influence of both the PERT dose and the variability in this dose on the coefficient of fat absorption (CFA). METHODS: This is a retrospective longitudinal study of 16 pediatric patients (192 food records) with three consecutive visits to the hospital over a twelve-month period...
July 27, 2017: Revista Española de Enfermedades Digestivas
https://www.readbyqxmd.com/read/28746534/brazilian-guidelines-for-the-diagnosis-and-treatment-of-cystic-fibrosis
#11
Rodrigo Abensur Athanazio, Luiz Vicente Ribeiro Ferreira da Silva Filho, Alberto Andrade Vergara, Antônio Fernando Ribeiro, Carlos Antônio Riedi, Elenara da Fonseca Andrade Procianoy, Fabíola Villac Adde, Francisco José Caldeira Reis, José Dirceu Ribeiro, Lídia Alice Torres, Marcelo Bicalho de Fuccio, Matias Epifanio, Mônica de Cássia Firmida, Neiva Damaceno, Norberto Ludwig-Neto, Paulo José Cauduro Maróstica, Samia Zahi Rached, Suzana Fonseca de Oliveira Melo
Cystic fibrosis (CF) is an autosomal recessive genetic disorder characterized by dysfunction of the CFTR gene. It is a multisystem disease that most often affects White individuals. In recent decades, various advances in the diagnosis and treatment of CF have drastically changed the scenario, resulting in a significant increase in survival and quality of life. In Brazil, the current neonatal screening program for CF has broad coverage, and most of the Brazilian states have referral centers for the follow-up of individuals with the disease...
May 2017: Jornal Brasileiro de Pneumologia: Publicaça̋o Oficial da Sociedade Brasileira de Pneumologia e Tisilogia
https://www.readbyqxmd.com/read/28721579/survival-of-patients-with-cystic-fibrosis-depending-on-mutation-type-and-nutritional-status
#12
A Szwed, A John, J Goździk-Spychalska, W Czaiński, W Czerniak, J Ratajczak, H Batura-Gabryel
The purpose of the study was to evaluate the influence of nutrition and of the severity of mutation type on survival rate in cystic fibrosis (CF) patients. Data were longitudinally collected from 60 hospitalized adult CF patients, aged 18-50. The variables consisted of body mass index (BMI) ratio, Cole's BMI cut-off points, severity of mutation type, and survival rate of CF patients. We found that the mean BMI was strongly associated with the severity of mutation type and was significantly lower in patients with severe mutations of grade I and II...
July 19, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28711222/retrospective-observational-study-of-french-patients-with-cystic-fibrosis-and-a-gly551asp-cftr-mutation-after-1-and-2years-of-treatment-with-ivacaftor-in-a-real-world-setting
#13
Dominique Hubert, Clémence Dehillotte, Anne Munck, Valérie David, Jinmi Baek, Laurent Mely, Stéphane Dominique, Sophie Ramel, Isabelle Danner Boucher, Sylvaine Lefeuvre, Quitterie Reynaud, Virginie Colomb-Jung, Prissile Bakouboula, Lydie Lemonnier
BACKGROUND: Ivacaftor has been shown to improve lung function and body weight in patients with CF and a gating mutation. Real-world evaluation is warranted to examine its safety and effectiveness over the long term. METHODS: A retrospective observational multicentre study collected clinical data in the year before and the 2years after ivacaftor initiation in patients with CF and a Gly551Asp-CFTR mutation. RESULTS: Fifty-seven patients were included...
July 12, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28697970/cystic-fibrosis-priorities-and-progress-for-future-therapies
#14
REVIEW
Eitan Kerem
Significant improvement in the survival of patients with CF has been achieved in the last decades. The improved clinical status of the patients is mainly the result of a better understanding of the natural course of infection and inflammation in CF that has led to the implementation of strategies that increase the life expectancy and quality of life of the patients. These strategies include prompt diagnosis, timely and aggressive nutritional support, augmentation of MCC and improved mucous drainage, initiation of antimicrobial and anti-inflammatory therapy as soon as possible, early treatment of acute exacerbations, implementation of effective hygienic measures in and outside CF centers and prompt identification and treatment of CF-related complications...
June 12, 2017: Paediatric Respiratory Reviews
https://www.readbyqxmd.com/read/28646623/pregnancy-outcome-in-women-with-cystic-fibrosis-related-diabetes
#15
Quitterie Reynaud, Stéphanie Poupon-Bourdy, Muriel Rabilloud, Lina Al Mufti, Christine Rousset Jablonski, Lydie Lemonnier, Raphaële Nove-Josserand, Sandrine Touzet, Isabelle Durieu
With increasing life expectancy more women with cystic fibrosis and diabetes mellitus become pregnant. We investigated how pre-gestational diabetes (cystic fibrosis related diabetes) influenced pregnancy outcome and the clinical status of these women MATERIAL AND METHODS: We analyzed all pregnancies reported to the French cystic fibrosis registry between 2001 and 2012, and compared forced expiratory volume (FEV1 ) and body mass index before and after pregnancy in women with and without pre-gestational diabetes having a first delivery RESULTS: A total 249 women delivered 314 infants...
June 24, 2017: Acta Obstetricia et Gynecologica Scandinavica
https://www.readbyqxmd.com/read/28627192/-excess-weight-in-patients-with-cystic-fibrosis-is-it-always-beneficial
#16
David González Jiménez, Rosana Muñoz-Codoceo, María Garriga-García, Manuel Molina-Arias, Marina Álvarez-Beltrán, Ruth García-Romero, Cecilia Martínez-Costa, Silvia María Meavilla-Olivas, Luis Rodrigo Peña-Quintana, Silvia Gallego Gutiérrez, José Manuel Marugán de Miguelsanz, Lucrecia Suárez Cortina, Esperanza Natividad Castejón Ponce, Rosaura Leis Trabazo, Fayna Martín Cruz, Juan José Díaz Martín, Carlos Bousoño García
INTRODUCTION: The primary objective of this study was to find out the prevalence of overweight and obese status, as well as their association to pulmonary function, total cholesterol and vitamin D in patients with cystic fibrosis (CF). MATERIALS AND METHODS: This is a multicenter descriptive and cross-sectional study. Twelve Spanish hospitals participated. 451 patients with CF were included. Adults were classified according to body mass index (BMI) and children were classified according to BMI percentiles (WHO tables)...
June 5, 2017: Nutrición Hospitalaria: Organo Oficial de la Sociedad Española de Nutrición Parenteral y Enteral
https://www.readbyqxmd.com/read/28601425/the-changing-face-of-nutrition-in-cystic-fibrosis
#17
EDITORIAL
Susan P Wolfe, Clare Collins
No abstract text is available yet for this article.
July 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28584399/immunonutrition-in-patients-with-cyctic-fibrosis-leads-to-drop-of-serum-amyloid-a-and-increase-of-oxidative-stress
#18
Ondrej Hloch, Jiri Charvat, Libor Fila, Havlin Jan
The aim of the present study is to evaluate of the impact of immunonutrition on parameters of oxidative stress and inflammation in patients with cystic fibrosis and malnutrition. In the 30 patients with cystic fibrosis and long-term enteral nutrition support for malnutrition the effect of standard and immunonutrion sipping on oxidative stress and inflammatory activity parameters was compared. Malonyldialdehyde (MDA) as parameter of oxidative stress and serum amyloid A (SAA), interleukin 1 and 6, hsCRP, IgM, IgA, IgG as parameters of inflammatory activity were examined...
May 2017: Journal of Clinical Biochemistry and Nutrition
https://www.readbyqxmd.com/read/28579360/complications-of-long-and-intermediate-term-venous-catheters-in-cystic-fibrosis-patients-a-multicenter-study
#19
Teresa L May, Alex H Gifford, Thomas Lahiri, Adam Black, Janet Trang, Alexandra G Cornell, Karyll Gonzalez, Scott Morin, Mark Napier, Christine W Duarte, Jonathan B Zuckerman
BACKGROUND: Totally implantable venous access devices (TIVADs) or peripherally inserted central venous catheters (PICCs) are commonly used in the care of patients with cystic fibrosis (CF), but they are associated with various complications, including thrombosis, infection, and insertion site symptoms. METHODS: We conducted a retrospective review of PICC and TIVAD use in adults and children with CF over an 8-year period at 3 accredited care centers. Patient attributes included CFTR genotype, comorbidities, lung function, body mass index, use of anticoagulation, and respiratory tract microbiology...
June 1, 2017: Journal of Cystic Fibrosis: Official Journal of the European Cystic Fibrosis Society
https://www.readbyqxmd.com/read/28508087/the-vitamin-d-for-enhancing-the-immune-system-in-cystic-fibrosis-disc-trial-rationale-and-design-of-a-multi-center-double-blind-placebo-controlled-trial-of-high-dose-bolus-administration-of-vitamin-d3-during-acute-pulmonary-exacerbation-of-cystic-fibrosis
#20
Vin Tangpricha, Ellen M Smith, Jose Binongo, Suzanne E Judd, Thomas R Ziegler, Seth Walker, Rabindra Tirouvanziam, Susu M Zughaier, Moon Jeong Lee, Supavit Chesdachai, Wendy A Hermes, James F Chmiel, Amit Gaggar, Ruth E Grossmann, Patricia M Joseph, Jessica A Alvarez
Vitamin D deficiency is highly prevalent in children and adults with cystic fibrosis (CF). Recent studies have found an association between vitamin D status and risk of pulmonary exacerbations in children and adults with CF. The ongoing Vitamin D for enhancing the Immune System in Cystic fibrosis (DISC) study is a multi-center, double-blind, randomized, placebo-controlled trial that will test the hypothesis of whether high dose vitamin D given as a single oral bolus of 250,000 IU to adults with CF during a pulmonary exacerbation followed by a maintenance dose of vitamin D will improve time to next pulmonary exacerbation and re-hospitalization, improve survival and lung function compared to placebo and reduce the rates of pulmonary exacerbation,...
June 2017: Contemporary Clinical Trials Communications
keyword
keyword
108809
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"