Cystic fibrosis Research Paper

Cystic fibrosis Research Paper

Melissa, a 12-year-old girl with cystic fibrosis comes to the primary care office with complaints of increased cough and productive green sputum over the last week. She also complains of increasing shortness of breath. She denies sore throat or nasal congestion. On physical examination her temperature is 101 and she has inspiratory wheezes bilaterally. Negative lymphadenopathy noted. Posterior pharynx is pink without exudate. BP 112/72 HR 96 RR 28. 1. In cystic fibrosis, the airway microenvironment favors bacterial colonization. Explain the pathophysiological reason for this occurrence

Due to the complicated nature of cystic fibrosis transmembrane conductance regulator (CFTR), several physiological defects occur in cystic fibrosis. CFTR defect in secretory epithelial cells leads to blockages in the airway and pancreatic ducts as a significant pathological result. In cystic fibrosis patient’s respiratory tract, CFTR impairment causes the formation of unusually thick, sticky mucus in the airway, which hinders clearance of bacterial mucociliary and encourages pathogenic microbes to colonize the airways (Bhagirath et al., 2016).  Cystic fibrosis Research Paper.The most common pathogenic microbes include Staphylococcus aureus, Pseudomonas aeruginosa, Burkholderia cepacia, and Haemophilus influenzae, with P. aeruginosa being the most prevalent respiratory infection in cystic fibrosis (Bhagirath et al., 2016). Prevalent chronic inflammation is attributed to the absence of microbial release and the development of a harmful pro-inflammatory local microenvironment that destroys the lungs and inherent immune function thus promoting infections.


The respiratory tract of a patient with cystic fibrosis is an intensely complex and diverse environment that presents a myriad of problems to occupying pathogens in the process. These problems encompass nutrients and oxygen limitation, hyperactive immune response, changing lung pathophysiology, competing microorganisms, and antibiotics. CFTR dysfunction in cystic fibrosis alters the airway ecosystem and the structural variables in the lower respiratory tract. The lower airway of a patient with cystic fibrosis contains numerous pathogens and microorganisms that are normally missing in healthy persons. Additionally, pathogens, as well as microorganisms, develop under these factors to cause mutators and persisters capable of long-term survival and colonization of potentially harsh lung ecosystem (López-Causapé et al., 2015). It is conceivable that the diverse physiological and anatomical conditions make the cystic fibrosis lung a unique environment with different areas ultimately filled by compatible microorganisms. This is reinforced by the various microbiota found in various areas of the respiratory tract and by the intrapulmonary dissemination of pathogens to previously untouched areas (Boutin et al., 2015). It is suggested that the existence of active bacterial subpopulations in specific niches of the airways may be correlated with pulmonary exacerbations in cystic fibrosis. Cystic fibrosis Research Paper. 


Bhagirath, A. Y., Li, Y., Somayajula, D., Dadashi, M., Badr, S., & Duan, K. (2016). Cystic fibrosis lung environment and Pseudomonas aeruginosa infection. BMC pulmonary medicine16(1), 1-22.

Boutin, S., Graeber, S. Y., Weitnauer, M., Panitz, J., Stahl, M., Clausznitzer, D., … & Mall, M. A. (2015). Comparison of microbiomes from different niches of upper and lower airways in children and adolescents with cystic fibrosis. PloS one10(1), e0116029.

López-Causapé, C., Rojo-Molinero, E., Macia, M. D., & Oliver, A. (2015). The problems of antibiotic resistance in cystic fibrosis and solutions. Expert review of respiratory medicine9(1), 73-88. Cystic fibrosis Research Paper.