Magnetic resonance imaging as a non-invasive tool to investigate pulmonary and renal fibrosis in small rodents : method and readout validation, animal model optimization, and substance testing [PDF]
Pulmonary fibrosis, an end result of various types of lung damage including interstitial pneumonia and respiratory bronchiolitis (Gross and Hunninghake, 2001; Katzenstein and Myers, 1998; Lazenby et al., 1990) is a lethal disease characterized by ...
Egger, Christine
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IntroductionOrganisms of the Burkholderia cepacia complex (BCC) are important pathogens in cystic fibrosis (CF). The majority of those who acquire BCC develop chronic infection but it can also result in rapid decline in a significant minority.
Alex eHorsley +7 more
doaj +1 more source
Endomyocardial fibrosis (EMF) is a neglected cardiovascular disease of poverty which carries a poor prognosis with no specific treatment affecting mainly children and young adults. Here, we report our 10-year experience in the therapeutic management and surgical treatment for EMF.From February 2009 to 2019 March, 55 patients diagnosed with EMF from our
Chukwuemeka Daniel, Iroegbu +4 more
openaire +3 more sources
ABSTRACT Background Shwachman–Diamond syndrome (SDS) is a rare autosomal recessive ribosomopathy characterized by bone marrow failure and multisystem involvement, with emerging evidence of associated neurocognitive impairment. Methods We conducted a retrospective study of 240 individuals with biallelic Shwachman–Bodian–Diamond syndrome (SBDS) mutations
Jane Koo +11 more
wiley +1 more source
Toll-like receptor signaling and SIGIRR in renal fibrosis upon unilateral ureteral obstruction. [PDF]
Innate immune activation via IL-1R or Toll-like receptors (TLR) contibutes to acute kidney injury but its role in tissue remodeling during chronic kidney disease is unclear.
Skuginna Veronika +41 more
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Validating the Data Completeness and Accuracy of the Canadian Cystic Fibrosis Registry
Conclusion: Our validation of the CCFR demonstrated high accuracy for clinical and demographic variables used in clinical research.
Ranjani Somayaji +9 more
doaj +1 more source
Phosphatidylinositol 4‐kinase as a target of pathogens—friend or foe?
This graphical summary illustrates the roles of phosphatidylinositol 4‐kinases (PI4Ks). PI4Ks regulate key cellular processes and can be hijacked by pathogens, such as viruses, bacteria and parasites, to support their intracellular replication. Their dual role as essential host enzymes and pathogen cofactors makes them promising drug targets.
Ana C. Mendes +3 more
wiley +1 more source
Cystic fibrosis mice carrying the missense mutation G551D replicate human genotype phenotype correlations [PDF]
We have generated a mouse carrying the human G551D mutation in the cystic fibrosis transmembrane conductance regulator gene (CFTR) by a one-step gene targeting procedure, These mutant mice show cystic fibrosis pathology but have a reduced risk of fatal ...
Wainwright, Brandon J. +23 more
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An epithelial GPR35 isoform supports tumor‐associated transcriptional and metabolic phenotypes
GPR35 generates two functionally distinct isoforms with previously unresolved roles. GPR35‐short mediates immune‐cell chemotaxis, while GPR35‐long is enriched in colorectal cancer epithelium, where it supports increased metabolism, proliferation, and tumor‐associated transcriptional programs.
Jørgen D. Rønneberg +14 more
wiley +1 more source
How are the ancient cystic fibrosis patients? Cystic fibrosis diagnosed over 60 years-old
Background and aims: To specify the prevalence of patients diagnosed with CF at age of ≥60 year-old and to analyze their characteristics. Patients and methods: Observational study of CF patients which were diagnosed at age ≥60 year-old.
C. Prados +10 more
doaj +1 more source

