New test could lead to personalized treatments for cystic fibrosis

The test combines high-speed video microscopy with a novel video analysis algorithm to measure the coordinated movement of cilia in the lungs.
Source: Yale Science and Health News - Category: Universities & Medical Training Source Type: news

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Publication date: Available online 14 July 2020Source: The Journal of Allergy and Clinical Immunology: In PracticeAuthor(s): Karen M. Anstey, Michael Lee, Diana Dawson, Vicki Jue, Jonathan M. Budzik, Michelle Yu, Mary Ellen Kleinhenz, Iris M. Otani
Source: The Journal of Allergy and Clinical Immunology: In Practice - Category: Allergy & Immunology Source Type: research
Conditions:   Chronic Rhinosinusitis (Diagnosis);   Cystic Fibrosis Intervention:   Sponsors:   University of Colorado, Denver;   Cystic Fibrosis Foundation Recruiting
Source: ClinicalTrials.gov - Category: Research Source Type: clinical trials
Authors: Teri A, Sottotetti S, Arghittu M, Girelli D, Biffi A, D'Accico M, Daccò V, Gambazza S, Pizzamiglio G, Trovato A, Tortoli E, Colombo C, Cariani L Abstract Mycobacterium abscessus (MABS) infection represents significant management challenge in cystic fibrosis (CF) patients. This retrospective study (2005-2016) aims to determine the prevalence of the subspecies of MABS isolated from CF patients, to evaluate the persistence over the years of a single subspecies of MABS and to correlate mutations responsible for macrolides and amikacin resistance with MIC values. We investigated 314 strains (1 isolate/pa...
Source: New Microbiologica - Category: Microbiology Tags: New Microbiol Source Type: research
CONCLUSION: Crystalluria is a common consequence of cystic fibrosis in childhood. The prevailing crystalluric finding includes hypercalciuria followed by hyperuricosuria, and hyperoxaluria. During disease GFR may be decreased due to several reasons such as nephrotoxic drugs usage. PMID: 32655022 [PubMed - in process]
Source: Iranian Journal of Kidney Diseases - Category: Urology & Nephrology Tags: Iran J Kidney Dis Source Type: research
CONCLUSIONS: The MexXY-OprM efflux pump is confirmed as the resistance determinant involved most frequently in P. aeruginosa aminoglycoside resistance of CF lung infections, followed by the ant(2″)-Ia-encoded adenylyltransferase. The latter may prove to be a novel target for new antimicrobial combinations against P. aeruginosa. PMID: 32654097 [PubMed - as supplied by publisher]
Source: Advances in Experimental Medicine and Biology - Category: Research Tags: Adv Exp Med Biol Source Type: research
In individuals with cystic fibrosis (CF), lung hyper-inflammation starts early in life and is perpetuated by mucus obstruction and persistent bacterial infections. The continuous tissue damage and scarring caused by non-resolving inflammation leads to bronchiectasis and, ultimately, respiratory failure. Macrophages (MΦs) are key regulators of immune response and host defense. We and others have shown that, in CF, MΦs are hyper-inflammatory and exhibit reduced bactericidal activity. Thus, MΦs contribute to the inability of CF lung tissues to control the inflammatory response or restore tissue homeostasis. The no...
Source: Frontiers in Pharmacology - Category: Drugs & Pharmacology Source Type: research
AbstractHuman primary bronchial epithelial cells differentiatedin vitro represent a valuable tool to study lung diseases such as cystic fibrosis (CF), an inherited disorder caused by mutations in the gene coding for the Cystic Fibrosis Transmembrane Conductance Regulator. In CF, sphingolipids, a ubiquitous class of bioactive lipids mainly associated with the outer layer of the plasma membrane, seem to play a crucial role in the establishment of the severe lung complications. Nevertheless, no information on the involvement of sphingolipids and their metabolism in the differentiation of primary bronchial epithelial cells are...
Source: Glycoconjugate Journal - Category: Biochemistry Source Type: research
Patients with cystic fibrosis (CF) have a two- to four-fold higher sodium chloride sweat content compared with healthy controls. This high sweat salt loss increases the risk for electrolyte disturbances, associated with subacute or chronic complications. Sodium status therefore needs to be adequately monitored and salt intake adjusted to individual needs. The lack of current evidence to formulate specific recommendations and assess sodium status is reflected in a variability of recommendations in international guidelines.
Source: Journal of the American Dietetic Association - Category: Nutrition Authors: Tags: Research Source Type: research
As CF lung disease is a progressive condition and there is heterogeneity among patients in the impact of disease that cannot be attributed solely to their CFTR genotype, we have long sought to understand factors that predict clinical outcomes in infants and children with CF. In this issue of the Journal of Cystic Fibrosis, three articles from three prospective cohort studies offer new insights, while raising key questions.
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Tags: Editorial Source Type: research
Cystic fibrosis (CF) is caused by loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, a cAMP-activated chloride channel expressed in the lungs, pancreas and other tissues [1, 2]. More than 2000 CFTR gene variants have been identified, with>300 associated with CF of varying severity [1, 3]. Although F508del is the most prevalent mutation in Europe and the US, there is considerable regional variability in many CFTR mutations [4]. The c.3700A>G mutation is a relatively common CF-causing mutation in the Middle East [5 –8], present in nearly all CF subjects in Qatar ...
Source: Journal of Cystic Fibrosis - Category: Respiratory Medicine Authors: Source Type: research
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