Probiotic Supplementation and Pulmonary Exacerbations in Patients with Cystic Fibrosis

Tzippora Shalem, Batia Weiss

Research output: Chapter in Book/Report/Conference proceedingChapterpeer-review


The major cause of morbidity and mortality in cystic fibrosis (CF) is a progressive inflammatory lung disease that results in end-stage respiratory failure in 90% of CF patients. In time, most children become colonized with Pseudomonas aeruginosa. Patients colonized with P. aeruginosa undergo a more rapid clinical course with progressive impairment of respiratory function. Therefore, colonization with Pseudomonas identifies a subset of patients at increased risk of pulmonary exacerbations and is associated with a decrease in survival rate. In an attempt to reduce the rate and severity of pulmonary exacerbations, children with CF need preventive antibiotic treatment and broad-spectrum antibiotics during exacerbation. There is a growing amount of evidence that chronic intestinal inflammation exists in CF patients. Intestinal inflammation may negatively affect the nutritional status of patient with CF, which adversely affects pulmonary function and survival. Approximately 85% of the patients with CF have pancreatic insufficiency. Despite adequate pancreatic enzyme replacement therapy in pancreatic-sufficed patients, most patients with CF have gastrointestinal symptoms, including abdominal pain, steatorrhea, and dysmotility. It has been suggested that nonpancreatic factors (increased permeability, abnormal microflora, and dysregulated innate immune mediators) are likely to contribute to the pathophysiology of these symptoms. Probiotics are live bacteria administered orally that have been successfully used to decrease the severity of acute gastroenteritis and to prevent atopic diseases in children. In addition, probiotics have been used as adjuvant therapy in patients with pouchitis and inflammatory bowel diseases. The mechanism of action of probiotics may be through improvement of intestinal barrier function and through modification of immune response. These, together with the chronic lung inflammation, intestinal inflammation, and increased use of antibiotics, lead to the assumption that probiotic treatment may be beneficial for CF patients.

Original languageEnglish
Title of host publicationDiet and Exercise in Cystic Fibrosis
PublisherElsevier Inc.
Number of pages4
ISBN (Electronic)9780128005880
ISBN (Print)9780128000519
StatePublished - 2015


  • Cystic fibrosis
  • Innate immunity
  • Intestinal inflammation
  • Microbiota
  • Probiotics


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