منابع مشابه
Pathophysiology of Cystic Fibrosis
Understanding the development of lung disease in cystic fibrosis (CF) has been a sought after goal for several decades. With the cloning of the CF transmembrane conductance regulator (CFTR) gene and other advances in the study of airway epithelial biology, a much clearer picture of disease pathophysiology has emerged. Our best available evidence points to dysregulated ion transport as the under...
متن کاملPathophysiology and management of pulmonary infections in cystic fibrosis.
This comprehensive State of the Art review summarizes the current published knowledge base regarding the pathophysiology and microbiology of pulmonary disease in cystic fibrosis (CF). The molecular basis of CF lung disease including the impact of defective cystic fibrosis transmembrane regulator (CFTR) protein function on airway physiology, mucociliary clearance, and establishment of Pseudomona...
متن کاملEpidemiology, Pathophysiology, and Prognostic Implications of Cystic Fibrosis–Related Diabetes
C ystic fibrosis–related diabetes (CFRD) is the most common comorbidity in subjects with cystic fibrosis (CF). A consensus conference on CFRD was cosponsored by the Cystic Fibrosis Foundation (CFF), the American Diabetes Association (ADA), and the Pediatric Endocrine Society (PES) in September 2009. The committee’s evidencebased recommendations for clinical management of CFRD are published in t...
متن کاملPathophysiology of gene-targeted mouse models for cystic fibrosis.
Pathophysiology of Gene-Targeted Mouse Models for Cystic Fibrosis. Physiol. Rev. 79, Suppl.: S193-S214, 1999. - Mutations in the gene causing the fatal disease cystic fibrosis (CF) result in abnormal transport of several ions across a number of epithelial tissues. In just 3 years after this gene was cloned, the first CF mouse models were generated. The CF mouse models generated to date have pro...
متن کاملPathophysiology of cystic fibrosis: implications for critical care nurses.
Lisa C. Grossman is a first-year medical student at Georgetown University,Washington, DC. in patients who have 10% or more CFTR function. Patients with 1% or less functioning of CFTR generally have a more classic type of cystic fibrosis; however, no definitive correlation exists between the genotype and the phenotype. In patients with 1% or less functioning of the gene, the patients’ cells have...
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ژورنال
عنوان ژورنال: Annales Nestlé (English ed.)
سال: 2006
ISSN: 1661-4011,0517-8606
DOI: 10.1159/000095374