Complex Relation Between Diet and Phospholipid Fatty Acids in Children With Cystic Fibrosis
Conclusions: The fatty acid composition of plasma PC and PE is altered in CF. Fatty acid differences between children with and without CF are inconsistent between PC and PE and are not explained by dietary fat.
Cystic Fibrosis (CF) is a monogenic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, resulting in defective CFTR-mediated chloride and bicarbonate transport, with dysregulation of epithelial sodium channels (ENaC). These changes alter fluid and electrolyte homeostasis and result in an exaggerated proinflammatory response driven, in part, by infection. We tested the hypothesis that NLRP3-inflammasome activation and ENaC upregulation drives exaggerated innate-immune responses in this multisystem disease. We identify an enhanced proinflammatory signature, as evidenced by incr...
In conclusion, IL-1α and IL-1β are upstream components of a signaling pathway, including IL-1R1 and downstream SPDEF and ERN2, that generate a positive feedback cycle capable of producing persistent mucus hyperconcentration and IL-1α and/or IL-1β–mediated neutrophilic inflammation in the absence of infection in CF airways. Targeting this pathway therapeutically may ameliorate mucus obstruction and inflammation-induced structural damage in young CF children.
Mucus obstruction is a hallmark of cystic fibrosis (CF) airway disease, leading to chronic infection, dysregulated inflammation, and progressive lung disease. As mucus hyperexpression is a key component in the initiation and perpetuation of airway obstruction, the triggers underlying mucin release must be identified and understood. In this issue of the JCI, Chen et al. sought to delineate the mechanisms that allow IL-1α/IL-1β to perpetuate the mucoinflammatory environment characteristic of the CF airway. The authors demonstrated that IL-1α and IL-1β stimulated non-CF human bronchial epithelial (HBE) c...
Key findings of this national survey of non-cystic fibrosis bronchiectasis epidemiology were that its prevalence, incidence and mortality have all increased over recent years; we estimate that around 212,000 people are currently living with bronchiectasis in the UK, very much higher than commonly quoted figures. Bronchiectasis is more common in females than males; 60% of diagnoses are made in the over-70 age group. Regional differences in prevalence, incidence, mortality, and hospital admission were identified.
Condition: Bronchiectasis Intervention: Drug: Roflumilast Sponsor: Assiut University Not yet recruiting
The family of Ayda Louden, from Carlisle, say moving to Scotland is now a serious consideration after the government there approved the life-extending drug Orkambi for NHS use.
Numerous issues must be addressed when developing standard operating procedures for clinical research studies involving chest computed tomography of lung disease in patients with cystic fibrosis (CF). Study success depends on the provision of adequate funding and the identification of personnel with the necessary expertise to conduct the study, along with clear guidelines that detail the CT operating procedure at each site, including breathing maneuvers, and image reconstruction. Close coordination of the quality assurance process between sites and the central review organization is required to maintain protocol adherence.
Publication date: Available online 13 September 2019Source: Journal of Ginseng ResearchAuthor(s): Do-Yeon Cho, Daniel Skinner, Shaoyan Zhang, Ahmed Lazrak, Dong Jin Lim, Christopher G. Weeks, Catherine G. Banks, Chang Kyun Han, Si-Kwan Kim, Guillermo J. Tearney, Sadis Matalon, Steven M. Rowe, Bradford A. WoodworthAbstractBackgroundAbnormal chloride (Cl−) transport has a detrimental impact on mucociliary clearance in both cystic fibrosis (CF) and non-CF chronic rhinosinusitis (CRS). Ginseng is a medicinal plant noted to have anti-inflammatory and antimicrobial properties. The present study aims to assess the capabilit...
ConclusionViral infections contributed to the increase in morbidity and mortality of CF patients in our population, and rhinovirus was the most common causative agent. Viral infections and viral recurrence increased the prevalence of bacterial infection of specific pathogens such as H. influenza and S. aureus. Physicians should be aware to prevent progressive lung damage in CF patients by treating the concomitant viral and bacterial infections. Viral infection may be associated with some common CFTR mutations.