NURS 530 Discussion Cystic Fibrosis Pathophysiology of Lung Disease
NURS 530 Discussion Cystic Fibrosis: Pathophysiology of Lung Disease
DQ1 Select one of the following discussion prompts to address:
Discuss the pathophysiology, clinical manifestations, evaluation, and treatment of cystic fibrosis. Discuss the potential impact of gene therapy on CF and the ethical dimensions in treating this life-limiting disease.
Describe the pathophysiology, clinical manifestations, evaluation, and treatment of asthma (include the National Asthma Education and Prevention Program guidelines).
Describe the pathophysiology, clinical manifestations, evaluation, and treatment of tuberculosis or pneumonia.
DQ2 Select one of the following discussion prompts to address:
Summarize acute kidney injury (AKI), include the RIFLE criteria for acute renal dysfunction/failure, and describe the pathophysiology, clinical manifestations, evaluation, and treatment.
Describe the stages of chronic kidney disease, and summarize the pathophysiology, clinical manifestations, evaluation, and treatment.
Discuss common causes of acute pyelonephritis, and describe the pathophysiology, clinical manifestations, evaluation, and treatment.
Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive disorder. In the last few years, giant steps have been made with regard to the understanding of CF pathophysiology, allowing the scientific community to propose mechanisms that cause the myriad of CF clinical manifestations. Following the discovery of the cystic fibrosis transmembrane conductance regulator (CFTR) gene in 1989, the structure and function of the CFTR protein were described. Since then, more than 2,000 variants of the CFTR gene and their impact on the amount and function of the CFTR protein have been reported. The role of the CFTR protein as an ion channel transporting chloride and bicarbonate and its repercussions on different epithelial cell-lined organs and mucus are now better understood. Mechanisms behind susceptibility to infection in CF have also been proposed and include abnormalities in the composition, volume and acidity of the airway surface liquid, changes in the submucosal gland’s anatomy and function, and deficiencies in the mucociliary clearance system. Numerous hypotheses explaining the excessive inflammatory response in CF are also debated and involve impaired mucociliary clearance, persistent hypoxia, lipid abnormalities, protease and antiprotease disproportion, and oxidant and antioxidant imbalance. The purpose of this review is to summarize our current knowledge of CF pathophysiology, including significant historic discoveries and most recent breakthroughs, and to improve understanding and awareness of this fatal disease.
