Cystic Fibrosis from Childhood to Adulthood

This article reviews the specific imaging features of CF using conventional imaging modalities (chest radiographs and high-resolution computed tomography [HRCT]) as well as emerging imaging technologies (digital chest tomosynthesis and MR imaging). In addition, the authors review the CF-specific HRCT imaging findings that are essential in the evaluation of these patients in the pre –lung transplant and post–lung transplant settings.
Source: Radiologic Clinics of North America - Category: Radiology Authors: Source Type: research

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Authors: Szczesniak R, Rice JL, Brokamp C, Ryan P, Pestian T, Ni Y, Andrinopoulou ER, Keogh RH, Gecili E, Huang R, Clancy JP, Collaco JM Abstract Introduction: Natural, social, and constructed environments play a critical role in the development and exacerbation of respiratory diseases. However, less is known regarding the influence of these environmental/community risk factors on the health of individuals living with cystic fibrosis (CF), compared to other pulmonary disorders.Areas Covered: Here, we review current knowledge of environmental exposures related to CF, which suggest that environmental/community risk f...
Source: Expert Review of Respiratory Medicine - Category: Respiratory Medicine Tags: Expert Rev Respir Med Source Type: research
AbstractAims/hypothesisThe aim of this study was to determine the mechanism(s) for hypoglycaemia occurring late following oral glucose loading in patients with cystic fibrosis (CF).MethodsA 3  h 75 g OGTT was performed in 27 non-diabetic adults with CF who were classified based on this test as experiencing hypoglycaemia (glucose
Source: Diabetologia - Category: Endocrinology Source Type: research
Introduction: The responses of cystic fibrosis (CF) airway epithelial cells (AEC) to rhinovirus (RV) infection are likely to contribute to early pathobiology of lung disease with increased neutrophilic inflammation and lower apoptosis reported. Necrosis of AEC resulting in airway inflammation driven by IL-1 signaling is a characteristic finding in CF detectable in airways of young children. Being the most common early-life infection, RV-induced epithelial necrosis may contribute to early neutrophilic inflammation in CF via IL-1 signaling. As little is known about IL-1 and biology of CF lung disease, this study assessed cel...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
Almost all bacteria secrete spherical membranous nanoparticles, also referred to as membrane vesicles (MVs). A variety of MV types exist, ranging from 20 to 400 nm in diameter, each with their own formation routes. The most well-known vesicles are the outer membrane vesicles (OMVs) which are formed by budding from the outer membrane in Gram-negative bacteria. Recently, other types of MVs have been discovered and described, including outer-inner membrane vesicles (OIMVs) and cytoplasmic membrane vesicles (CMVs). The former are mainly formed by a process termed endolysin-triggered cell lysis in Gram-negative bacteria, the la...
Source: Frontiers in Microbiology - Category: Microbiology Source Type: research
ConclusionsEnvironmental surveillance is required to investigate the aetiology and mode of transmission of theB.cepacia outbreak. Systematic management of nosocomial outbreaks, particularly in resource limited regions will mitigate transmission and improve patients ’ outcomes.
Source: PLoS Neglected Tropical Diseases - Category: Tropical Medicine Authors: Source Type: research
Purpose of review Liver disease in cystic fibrosis (CF) usually develops before puberty, is often asymptomatic and slowly progressive. Multilobular cirrhosis develops in approximately 5–10% of patients by the age of 18, and is a significant contributor to the morbidity and mortality. No therapy, including ursodeoxycholic acid and cystic fibrosis transmembrane conductance regulator correctors or potentiators, has proven effective to prevent or halt the progression of liver disease towards cirrhosis and portal hypertension. This review provides the current knowledge in the epidemiology of CF liver disease and develop...
Source: Current Opinion in Gastroenterology - Category: Gastroenterology Tags: LIVER: Edited by Don C. Rockey Source Type: research
Baker Discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene was the long-awaited scientific advance that dramatically improved the diagnosis and treatment of cystic fibrosis (CF). The combination of a first-tier biomarker, immunoreactive trypsinogen (IRT), and, if high, DNA analysis for CF-causing variants, has enabled regions where CF is prevalent to screen neonates and achieve diagnoses within 1–2 weeks of birth when most patients are asymptomatic. In addition, IRT/DNA (CFTR) screening protocols simultaneously contribute important genetic data to determine genotype, prognosticate,...
Source: Genes - Category: Genetics & Stem Cells Authors: Tags: Review Source Type: research
Cystic fibrosis (CF) is today the second most common primary indication for bilateral lung transplantation in adults and the third most common indication for lung transplantation in adults overall according to the most recent Thoracic Transplant Registry Report of the International Society for Heart and Lung Transplantation (ISHLT) (1). In children and adolescents, CF is the commonest reason for transplantation reaching 2/3rd in children older than 11 years (2). In general, lung transplantation is considered a life-saving therapy option in well selected patients with respiratory failure due to advanced CF lung disease (3).
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Editoria Source Type: research
Abstract We have demonstrated previously that intracellular transport is impaired in cystic fibrosis (CF) epithelial cells. This impairment is related to both growth and inflammatory regulation in CF cell and animal models. Understanding how transport in CF cells is regulated and identifying means to manipulate that regulation are key to identifying new therapies that can address key CF phenotypes. It was hypothesized that resveratrol could replicate these benefits since it interfaces with multiple pathways identified to affect microtubule regulation in CF. It was found that resveratrol treatment significantly res...
Source: American Journal of Physiology. Lung Cellular and Molecular Physiology - Category: Cytology Authors: Tags: Am J Physiol Lung Cell Mol Physiol Source Type: research
Yale School of Medicine experts offer critical COVID-19 guidance for people with chronic pulmonary conditions like asthma, COPD, and cystic fibrosis.
Source: Yale Science and Health News - Category: Universities & Medical Training Source Type: news
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