With newer tools for gene editing, is it time to revisit genetic therapy for cystic fibrosis?

Publication date: Available online 22 January 2020Source: Genes &DiseasesAuthor(s): Anuj Gupta, Ankita Gupta, Larissa Boyd
Source: Genes and Diseases - Category: Genetics & Stem Cells Source Type: research

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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
Emerging findings indicate there is a vital cross-talk between gut microbiota and the lungs, which is known as gut–lung axis. The gut disturbances in lung diseases including allergy, asthma, chronic obstructive pulmonary disease, cystic fibrosis and lung cancer were observed by extensive studies. Investigating how gut microbiota impact other distant organs is of great interest in recent years. Although it has not been fully understood whether the disturbance is the cause or effect of lung diseases, alterations in the gut microbial species and metabolites have been linked to changes in immune responses and inflammatio...
Source: Frontiers in Microbiology - Category: Microbiology Source Type: research
Stenotrophomonas maltophilia is a Gram-negative opportunistic pathogen that can chronically colonize the lungs of people with cystic fibrosis (CF) and is associated with lethal pulmonary hemorrhage in immunocompromised patients. Its secreted virulence factors include the extracellular serine proteases StmPR1, StmPR2, and StmPR3. To explore the impact of secreted virulence determinants on pulmonary mucosal defenses in CF, we examined the secretome of human CFBE41o- bronchial epithelial cells in response to treatment with S. maltophilia K279a cell culture supernatant (CS) using a liquid-chromatography-tandem mass spectrometr...
Source: Frontiers in Immunology - Category: Allergy & Immunology Source Type: research
To examine contraceptive use, pregnancy intention and the association of hormonal contraceptive type with adverse health outcomes among women with cystic fibrosis (CF).
Source: Contraception - Category: OBGYN Authors: Tags: Original Research Article Source Type: research
f M Abstract The planar cell polarity (PCP) signaling pathway is a potent developmental regulator of directional cell behaviors such as migration, asymmetric division and morphological polarization that are critical for shaping the body axis and the complex three-dimensional architecture of tissues and organs. PCP is considered a noncanonical Wnt pathway due to the involvement of Wnt ligands and Frizzled family receptors in the absence of the beta-catenin driven gene expression observed in the canonical Wnt cascade. At the heart of the PCP mechanism are protein complexes capable of generating molecular asymmetries...
Source: Biochemical Society Transactions - Category: Biochemistry Authors: Tags: Biochem Soc Trans Source Type: research
Patients with Cystic Fibrosis-related diabetes (CFRD) have poorer lung function than those who do not have CFRD [1], and their lung function begins to decline several years prior to the development and diagnosis of fulminant diabetes [2]. CFRD is conventionally diagnosed using the Oral Glucose Tolerance Test (OGTT) but even when the OGTT is normal, patients with CF may still demonstrate elevated glucose levels on continuous glucose monitoring (CGM) [3,4]. Using a subcutaneous probe, CGM devices record interstitial fluid glucose levels over several days.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Short Communication Source Type: research
ConclusionsOur data suggest that fosfomycin has good effect against mucoid and non-mucoid strains of P. aeruginosa and automated systems can be implemented in clinical microbiology laboratories to assess fosfomycin with rapid and reproducible results.
Source: Journal of Global Antimicrobial Resistance - Category: Infectious Diseases Source Type: research
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