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Risk factors associated with pulmonary exacerbations in pediatric patients with cystic fibrosis

Silvina Lubovich1, Silvina Zaragoza2, Viviana Rodríguez2

  • 1Centro Respiratorio, Unidad 3, Hospital de Niños Ricardo Gutiérrez, Buenos Aires. slubovich@gmail.com.

Archivos Argentinos De Pediatria
|September 28, 2019
PubMed

Insights

Cystic fibrosis pulmonary exacerbations are linked to the p.F508del mutation, poor nutrition, and chronic infections. A significant portion of patients struggle to regain lung function after these events.

Area of Science:

  • Pulmonology
  • Infectious Diseases
  • Genetics

Background:

  • Pulmonary exacerbations (PEs) are a major cause of morbidity in cystic fibrosis (CF) patients, often necessitating intensive treatment.
  • Identifying risk factors for PEs is crucial for timely intervention and improved patient outcomes.

Purpose of the Study:

  • To identify risk factors associated with pulmonary exacerbations in cystic fibrosis patients.
  • To determine the percentage of CF patients who do not recover baseline lung function after a PE.

Main Methods:

  • Retrospective cohort study analyzing medical records of 117 cystic fibrosis patients.
  • Patients were categorized into groups with and without PEs.
  • Data collected included demographics, genetic mutations (p.F508del), nutritional status (BMI Z-score), and microbial colonization (Pseudomonas aeruginosa, MRSA, Bcc).

Main Results:

  • Pulmonary exacerbations were significantly associated with a lower BMI Z-score (RR: 1.45), the p.F508del mutation (RR: 3.23), and chronic colonization with Burkholderia cepacia complex (RR: 3.69), Pseudomonas aeruginosa (RR: 1.89), and methicillin-resistant Staphylococcus aureus (RR: 2.32).
  • Twenty-four percent of patients failed to recover their baseline lung function post-exacerbation.

Conclusions:

  • The p.F508del mutation, poor nutritional status, and chronic microbial colonization are key risk factors for pulmonary exacerbations in cystic fibrosis.
  • A substantial proportion of cystic fibrosis patients experience persistent lung function impairment following exacerbations.
Abstract

Keywords:
cystic fibrosispulmonary exacerbations

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