CF mucus defect present at birth

(University of Iowa Health Care) New research by University of Iowa scientists shows that cystic fibrosis (CF), a life-shortening, inherited condition that affects about 30,000 Americans, causes abnormalities in airway mucus that impairs the ability to clear particles and germs out of the airway.
Source: EurekAlert! - Medicine and Health - Category: Global & Universal Source Type: news

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Burkholderia cenocepacia adheres to the respiratory epithelium of cystic fibrosis patients and causes chronic inflammation and disease. Our findings uncover the transcriptional alteration ofBCAM2418 gene induced by the physical contact of the bacterium with bronchial epithelial cells. We found that overexpression ofBCAM2418 gene augmented the bacterial cell adhesion to host cells, and it is dependent on recognition of O-linked glycans from the host cell membranes. AbstractCell ‐to‐cell early contact between pathogens and their host cells is required for the establishment of many infections. Among various surface factor...
Source: MicrobiologyOpen - Category: Microbiology Authors: Tags: ORIGINAL ARTICLE Source Type: research
Cystic fibrosis (CF) is the most common, life-threatening, autosomal-recessive disorder among Caucasians. To date, approximately 2000 mutations in the CFTR gene have been reported. Some of these mutations are ...
Source: BMC Pediatrics - Category: Pediatrics Authors: Tags: Case report Source Type: research
Since 2000, more than 400 000 babies worldwide have died of congenital diaphragmatic hernia (CDH), a condition that is occurring as frequently as cystic fibrosis and characterised by underdeveloped lungs (pulmonary hypoplasia), persistent pulmonary hypertension and a diaphragmatic defect [1]. CDH can be diagnosed prenatally with ultrasound and fetal MRI, but outcome prediction and diagnostic accuracy remain imperfect [2]. The observed over expected lung-to-head (O/E LHR) ratio at 22–23 and 32–33 weeks of gestation is currently used to predict CDH outcomes [3]. A prenatal biomarker for the assessment of dis...
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Original Articles: Research letters Source Type: research
Nurhayat Yakut, Eda Kepenekli Kadayifci, Ela Erdem Eralp, Yasemin GokdemirLung India 2020 37(2):161-163 Allergic bronchopulmonary aspergillosis (ABPA) is recognized as a rare, progressive, allergic disorder in patients with cystic fibrosis (CF) and asthma. Treatment of ABPA mainly includes systemic corticosteroids (CSs) and antifungal agents. Here, we report posaconazole treatment in a 9-year-old male child with ABPA and also review the literature on antifungal management of ABPA. The child with CF was admitted to the emergency room with complaints of fever, productive cough, and acute dyspnea. Auscultation of the lungs r...
Source: Lung India - Category: Respiratory Medicine Authors: Source Type: research
While the quality of life and survival of individuals with cystic fibrosis (CF) are improving, advanced CF lung disease (ACFLD) remains common and the most frequent cause of death. CF Foundation Patient Registry data show that the forced expiratory volume in one second (FEV1) is less than 40 percent predicted in 18% of patients who are age 30 years, and nearly 25% of those age 45 years. ACFLD is associated with reduced quality of life, worsening clinical symptoms, increased exacerbations, and increased healthcare utilization [1,2].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
Cystic fibrosis (CF) is classically characterized by a progressive obstructive pulmonary disease and pancreatic exocrine insufficiency [1]. It is primarily the progressive respiratory decline that leads to an early mortality for those with CF. There are many other co-morbidities, such as cystic fibrosis-related diabetes (CFRD), that accelerates the lung decline [1]. The onset of CFRD portends a much worse prognosis and is associated with a more rapid loss of lung function, more frequent pulmonary exacerbations and ultimately an increase in mortality due to respiratory failure [2,3].
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Original article Source Type: research
Nature, Published online: 26 February 2020; doi:10.1038/s41586-020-2047-9Metabolomics data from germ-free and specific-pathogen-free mice reveal effects of the microbiome on host chemistry, identifying conjugations of bile acids that are also enriched in patients with inflammatory bowel disease or cystic fibrosis.
Source: Nature AOP - Category: Research Authors: Source Type: research
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
ConclusionThere was a significant association of antibiotic therapy and age on S. aureus carriage profiles in CF patients indicating that antibiotic therapy prevents acquisition of new clones, while during aging of patients with persisting S. aureus, dominant clones were selected and mutations in the spa-repeat region accumulated.
Source: Frontiers in Microbiology - Category: Microbiology Source Type: research
Authors: Sibanda D, Singleton R, Clark J, Desnoyers C, Hodges E, Day G, Redding G Abstract Recent literature has highlighted the importance of transition from paediatric to adult care for children with chronic conditions. Non-cystic fibrosis bronchiectasis is an important cause of respiratory morbidity in low-income countries and in indigenous children from affluent countries; however, there is little information about adult outcomes of childhood bronchiectasis. We reviewed the clinical course of 31 Alaska Native adults 20-40 years of age from Alaska's Yukon Kuskokwim Delta with childhood bronchiectasis. In pa...
Source: International Journal of Circumpolar Health - Category: International Medicine & Public Health Tags: Int J Circumpolar Health Source Type: research
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