ReviewThe pathology of cystic fibrosis
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Cited by (33)
Autophagy markers as mediators of lung injury-implication for therapeutic intervention
2020, Life SciencesCitation Excerpt :Experiment proves that the pro-inflammatory cytokines IL-1β directly regulates the initiation of acute and chronic inflammation making it a valuable target of IPF [88]. Cystic fibrosis is characterized as a most common autosomal recessive genetic disorder caused by the mutation in CFTR gene (transmembrane protein gene-cystic fibrosis transmembrane conductance receptor) [89]. Pathology of the disease involves bronchiolitis, obstruction of pathways, endobronchiolar infection, impaired ciliary actions, atelectasis finally leading to secondary alveolar injury.
Disruption of Aspergillus fumigatus biofilm by Streptococcus pneumoniae: Mycelial fragmentation by hydrogen peroxide
2020, Journal of Infection and ChemotherapyCitation Excerpt :The affected area of a patient would host various interactions among pathogens. A. fumigatus and Pseudomonas aeruginosa are often isolated from cystic fibrosis (CF) patients [47,48]. CF is an autosomal recessive genetic disorder, which is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene, and primarily affects the digestive system and lungs [49].
Dose administration maneuvers and patient care in tobramycin dry powder inhalation therapy
2018, International Journal of PharmaceuticsCitation Excerpt :Cystic fibrosis (CF) is a genetic rare disease caused by mutations in the gene coding the CF trans-membrane conductance regulator protein leading to viscous mucus presence in the airways (Moskowitz et al., 2005; Sheppard and Nicholson, 2002).
Vitamin E status and its determinants in patients with cystic fibrosis
2018, Advances in Medical SciencesCitation Excerpt :The membrane protein is a product of CFTR gene transcription and functions as an ion channel for sodium, chloride and water [3]. Dysfunctional protein leads to the accumulation of mucus on epithelial surfaces in many organs of respiratory, digestive and reproduction systems [4]. Accordingly, pancreatic insufficiency is one of the many consequences occurring in 85–90% CF patients and leads to fat-soluble vitamins deficiency (A, D, E, K) [5,6].
Inhalable DNase I microparticles engineered with biologically active excipients
2013, Pulmonary Pharmacology and TherapeuticsCitation Excerpt :Cystic fibrosis (CF) is the most common autosomal, recessive, life-span shortening disease in Caucasians. Development of chronic pulmonary diseases is the main cause of mortality in CF patients [1]. Currently, DNase I (Mw 33 KDa) is delivered as a nebulising solution.
Enhanced properties of discrete pulmonary deoxyribonuclease I (DNaseI) loaded PLGA nanoparticles during encapsulation and activity determination
2011, International Journal of PharmaceuticsCitation Excerpt :Cystic fibrosis (CF) is a common autosomal recessive disorder in the Caucasian population, affecting 1 in 2000 of live births (Sheppard and Nicholson, 2002).
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Correspondence to: MNS. E-mail: [email protected]