(Cystic fibrosis) articles in PubMed
- Oral Supplementation with a Special Additive of Retinyl Palmitate and Alpha Tocopherol Reduces Growth Retardation in Young Pancreatic Duct Ligated Pigs Used as a Model for Children Suffering from Exocrine Pancreatic Insufficiency. [Journal Article]
- Int J Mol Sci 2016; 17(10)IJ
- Pancreatic exocrine insufficiency (PEI) is a disease of diverse aetiology-e.g., majority of patients suffering from cystic fibrosis (CF) show PEI congenitally. Malnutrition and malabsorption of nutri...
Pancreatic exocrine insufficiency (PEI) is a disease of diverse aetiology-e.g., majority of patients suffering from cystic fibrosis (CF) show PEI congenitally. Malnutrition and malabsorption of nutrients impair growth and nutritional status. As reduced fat digestion leads to a deficiency of fat-soluble vitamins the supplementation is standard, but absorption is a critical point in PEI-patients. The pancreatic duct ligated (PL) pig is an established model for PEI in humans and has been proven to be a suitable model to compare different vitamin additives for supplementation. In a former study, PEI caused distinct growth retardation in young piglets, but did not affect growth in older ones. Our study hypothesised that this age-dependent effect is caused by exhausted body reserves of fat-soluble vitamins and, therefore, extra supply reduces growth retardation. PEI was induced by PL at the age of seven (PL-7) or 16 weeks (PL-16). Controls (C) underwent a sham surgery. Some PL-7 pigs (PL-7 + Vit) were fed a special vitamin additive. PEI reduced the mean final body weight (kg) at 26 weeks of age significantly with lower effect in PL-16-pigs (C:117; PL-7:49.5; PL-7 + Vit:77.1; PL-16:96.4). Extra vitamin supply resulted in an increased growth and normalised serum concentration of alpha-tocopherol, underlining the importance of special supplementation in PEI-patients.
- Of Pigs, Mice, and Men: Understanding Early Triggers of Cystic Fibrosis Lung Disease. [Journal Article]
- Am J Respir Crit Care Med 2016 Oct 1; 194(7):784-785AJ
- miR-146a, miR-155, miR-370 and miR-708 are CFTR-dependent, Predicted FOXO1 Regulators and Change at Onset of CFRDs. [Journal Article]
- J Clin Endocrinol Metab 2016 Sep 30; :jc20162431JC
- CONCLUSIONS: The data showed significant changes in miRNAs dependent on genotype and glucose tolerance state in CF patients, and highlighted some miRNAs of importance in CFRD at onset. MiRNAs could explain some of the variability observed in cystic fibrosis.
- Population pharmacokinetics of mycophenolic acid and its glucuronide metabolite in lung transplant recipients with and without cystic fibrosis. [Journal Article]
- Xenobiotica 2016 Aug 10; :1-8X
- 1. Cystic fibrosis (CF) is a disease affecting multiple organs that may reduce the systemic exposure of some drugs. The objective of this work was to characterize and compare the population pharmacok...
1. Cystic fibrosis (CF) is a disease affecting multiple organs that may reduce the systemic exposure of some drugs. The objective of this work was to characterize and compare the population pharmacokinetics (PK) of the immunosuppressant mycophenolic acid (MPA), and its glucuronide metabolite (MPAG) in adult lung transplant recipients with and without CF (NCF) following repeated oral administration of the prodrug mycophenolate mofetil (MMF). 2. A population PK model was developed, with simultaneous modeling of MPA and MPAG, using nonlinear mixed effects modeling. MPA and MPAG serum concentration-time data were adequately described by a compartmental model including enterohepatic recirculation (EHR). Both MPA and MPAG apparent clearance values were significantly elevated (>65%) in patients with CF (24.1 and 1.95 L/h, respectively) compared to the values in the NCF patients (14.5 and 1.12 L/h, respectively), suggesting a notable influence of CF on MPA absorption and disposition. 3. The population PK model developed from our study successfully characterized the absorption, distribution, elimination and EHR of MPA and the metabolite MPAG in lung transplant recipients with or without CF. This model may help to further understand the impact of CF to the overall clinical effects of MPA therapy including immunosuppression and gastrointestinal side effects.
- Update of literature from cystic fibrosis registries 2012-2015. Part 6: Epidemiology, nutrition and complications. [Review]
- Pediatr Pulmonol 2016 Sep 29PP
- Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics...
Patient registries provide useful information to afford more knowledge on rare diseases like Cystic Fibrosis (CF). Twenty-two studies originating from national CF registries, focusing on demographics, survival, genetics, nutritional status, and non-pulmonary complications, were published between December 2011 and March 2015. The purpose of this review article is to examine these reports, aiming attention to the clinical characteristics of CF patients included in the registries, current, and estimated future epidemiological data, the role of gender gap, the increasing survival in different countries. Some studies offer insights into pubertal growth and non-pulmonary complications, such as liver disease, nephropathy, and cancer. Pediatr Pulmonol. 2016; 9999:XX--XX. © 2016 Wiley Periodicals, Inc.
- Constrictive Bronchiolitis in Adolescents with Cystic Fibrosis with Refractory Pulmonary Decline. [Journal Article]
- Ann Am Thorac Soc 2016 Sep 29AA
- CONCLUSIONS: Constrictive bronchiolitis is widely present in lung tissue from CF adolescents with advanced disease, and may contribute to impaired lung function that is refractory to conventional therapy (antibiotics, anti-inflammatories, and mucolytics). TGF-β dependent myofibroblast differentiation is prominent in areas of active fibrogenesis and may foster small airway remodeling in CF lung disease.
- Burkholderia cenocepacia K56-2 trimeric autotransporter adhesin BcaA binds TNFR1 and contributes to induce airway inflammation. [Journal Article]
- Cell Microbiol 2016 Sep 29CM
- Chronic lung disease caused by persistent bacterial infections is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). CF pathogens acquire antibiotic resistance, overcame ...
Chronic lung disease caused by persistent bacterial infections is a major cause of morbidity and mortality in patients with cystic fibrosis (CF). CF pathogens acquire antibiotic resistance, overcame host defenses and impose uncontrolled inflammation that ultimately may cause permanent damage of lungs' airways. Among the multiple CF-associated pathogens, Burkholderia cenocepacia and other B. cepacia complex (Bcc) bacteria have become prominent contributors of disease progression. Here, we demonstrate that BcaA, a trimeric autotransporter adhesin (TAA) from the epidemic strain B. cenocepacia K56-2, is a TNFR1-interacting protein able to regulate components of the TNF signaling pathway and ultimately leading to a significant production of the proinflammatory cytokine IL-8. Notably, this study is the first to demonstrate that a protein belonging to the TAA family is involved in the induction of the inflammatory response during B. cenocepacia infections contributing to the success of the pathogen. Moreover our results reinforce the relevance of the TAA BcaA as a multifunctional protein with a major role in B. cenocepacia virulence. This article is protected by copyright. All rights reserved.
- A Life Course Perspective on Health Trajectories and Transitions [BOOK]
- Springer: Cham (CH)BOOK
- This chapter describes our longitudinal registry studies examining the effect of social deprivation on longitudinal clinical outcomes, healthcare use and employment opportunities in people with cysti...
- A Life Course Perspective on Health Trajectories and Transitions [BOOK]
- Springer: Cham (CH)BOOK
- This open access book examines health trajectories and health transitions at different stages of the life course, including childhood, adulthood and later life. It provides findings that assess the r...
This open access book examines health trajectories and health transitions at different stages of the life course, including childhood, adulthood and later life. It provides findings that assess the role of biological and social transitions on health status over time. The essays examine a wide range of health issues, including the consequences of military service on body mass index, childhood obesity and cardiovascular health, socio-economic inequalities in preventive health care use, depression and anxiety during the child rearing period, health trajectories and transitions in people with cystic fibrosis and oral health over the life course. The book addresses theoretical, empirical and methodological issues as well as examines different national contexts, which help to identify factors of vulnerability and potential resources that support resilience available for specific groups and/or populations. Health reflects the ability of individuals to adapt to their social environment. This book analyzes health as a dynamic experience. It examines how different aspects of individual health unfold over time as a result of aging but also in relation to changing socioeconomic conditions. It also offers readers potential insights into public policies that affect the health status of a population.
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- Parent & Child Perceptions of Child Health after Sibling Death. [Journal Article]
- Int J Nurs Clin Pract 2016; 3IJ
- CONCLUSIONS: Children's responses to a sibling's death may not be visually apparent or become known by asking parents. Parents often perceive their children as healthier than children perceive themselves at 2 and 4 months after sibling death, so talking with children separately is important. Children's perceptions of their health may be influenced by depression, fathers' perceptions by children's anxiety, and mother's perceptions by the cause of sibling death.