Cystic fibrosis. 2. Lung injury in cystic fibrosis.

Maintaining Respiratory Health in Cystic Fibrosis Patients

Cystic fibrosis (CF) is an inherited disease that primarily affects the lungs and the digestive system, however, it also affects a number of other organs and systems. More than 90% of mortality of  CF patients is due to lung complications.  Healthy lungs are important for a long life for people with CF, We will discuss two important topics for maintaining respiratory health. Chronic use of drug...

Prevalence of Cystic Fibrosis Trans-membrane Conductance Regulator Gene common mutations in children with cystic fibrosis in Isfahan, Iran

Background: Cystic fibrosis (CF) is the most common lethal genetic disorder of Cystic Fibrosis Trans-membrane Conductance (CFTR) Regulator gene mutations. We aimed to investigate common mutations in CF patients and to assess its possible relationship with clinical presentations. Materials and Methods: This cross sectional study was conducted on 36 CF patients who were referred to a tertiary ped...

Lung transplantation in cystic fibrosis.

Lung transplantation has become a viable option for those cystic fibrosis (CF) patients with end-stage lung disease. Despite the challenges that the CF patients present, the survival seen after lung transplantation is more favorable than seen in patients with chronic obstructive pulmonary disease and pulmonary fibrosis. Although the CF patients with severe respiratory disease usually are infect...

Lung abscess in cystic fibrosis.

Almost all patients with cystic fibrosis eventually develop chronic pulmonary infection with specific bacterial pathogens, notably Pseudomonas aeruginosa and Staphylococcus aureus. Despite this, the development of large pulmonary abscess cavities in patients with cystic fibrosis is exceedingly uncommon. Similarly, bacteraemia in cystic fibrosis is surprisingly rare. We describe a patient with c...

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