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Nanocomposite Microparticles (nCmP) for Pulmonary Drug Delivery Applications
Published in Raj K. Keservani, Anil K. Sharma, Rajesh K. Kesharwani, Drug Delivery Approaches and Nanosystems, 2017
Zimeng Wang, Elisa A. Torrico-Guzmán, Sweta K. Gupta, Samantha A. Meenach
Cystic Fibrosis (CF) is a genetic disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Current U.S. guidelines suggest treating all CF patients older than 6 years with chronic pulmonary infections with inhaled antibiotics, first tobramycin, then aztreonam, and finally colistin. Aerosolized antibiotic therapy is traditionally given through a nebulizer; however, in March 2013 the TOBI Podhaler was approved as a DPI alternative for inhaled tobramycin. A trial comparing the tobramycin-inhaled powder (TIP) versus nebulized solution found that TIP was generally well tolerated, provided a significantly more convenient treatment option, and resulted in a higher discontinuation rate (Konstan et al., 2011a, 2011b; Parkins and Elborn, 2011). Colistin, while considered third-line by U.S. guidelines, is frequently used as first choice in Europe for CF patients (Smyth et al., 2014). The DPI form of colistin (Colobreathe) is approved for use in Europe.
Clinical Applications of Immunoassays
Published in Richard O’Kennedy, Caroline Murphy, Immunoassays, 2017
Cystic fibrosis (CF) is one of the commonest life-threatening autosomal recessive conditions primarily affecting Caucasian populations. It is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene on chromosome 7, which results in the expression of a dysfunctional chloride channel in exocrine tissue. This defect leads to abnormal transport of chloride and sodium across the epithelium leading to the formation of thick viscous secretions affecting the lung, pancreas, liver, intestine and the reproductive tract. Ultimately it is progressive lung disease, which is the major cause of morbidity and mortality in CF patients, as increased viscous secretions in the lung promote bacterial colonisation [40]. Diagnosis of CF is based on both biochemical and genetic testing. The sweat chloride test is the mainstay of laboratory confirmation. This test is performed using pilocarpine iontophoresis followed by chemical analysis of the secretions. A chloride concentration >60 mmol L−1 is required for a diagnosis of CF. A small quantity of a dried blood sample is now routinely used in screening newborns for CF and can identify approximately 95% of CF cases. This test is based on the detection of elevated immunoreactive trypsin (IRT) levels and can be quantified by either radioimmunoassay or by an enzyme-linked immunoassay [41].
Compensatory changes in physical activity and sedentary time in children and adolescents with cystic fibrosis
Published in Journal of Sports Sciences, 2019
Kelly A. Mackintosh, Nicola D. Ridgers, Melitta A. McNarry
Cystic fibrosis (CF), currently affecting over 10,000 people in the UK (Cystic Fibrosis Trust, 2017), is the most prevalent inherited genetic disorder in the Caucasian population (Quinton, 1990). CF is a multi-system disease, which primarily affects the lungs and digestive system through mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene that lead to malfunctioning or absent CFTR proteins and impaired mucosal clearance mechanisms (Davies, Alton, & Bush, 2007; National Institute for Health and Care Excellence, 2017; Ratjen, 2009). Despite therapeutic advances and an increased life expectancy to 47 years for those born in 2017 (Cystic Fibrosis Trust, 2017), CF remains incurable, highlighting the need to enhance patient well-being.