Change in lung clearance index with microbiological status in children with cystic fibrosis

Katarzyna Walicka-Serzysko1,2, Magdalena Postek1,2, Justyna Milczewska1,2

  • 1Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland.

Pediatric Pulmonology
|March 7, 2019
PubMed

Insights

Lung clearance index (LCI) in cystic fibrosis patients is linked to specific infections. Chronic Aspergillus fumigatus and Pseudomonas aeruginosa increase LCI, highlighting the importance of early pathogen eradication for lung health.

Area of Science:

  • Respiratory Medicine
  • Pediatric Pulmonology
  • Clinical Microbiology

Background:

  • Cystic Fibrosis (CF) lung disease progression is significantly impacted by bacterial infections, particularly Gram-negative bacteria.
  • Pulmonary function decline in CF often begins in early childhood.
  • Lung clearance index (LCI) is a sensitive marker for small airway disease in CF, potentially more so than FEV1.

Purpose of the Study:

  • To examine the relationship between the microbiological status of pediatric cystic fibrosis patients and their lung clearance index (LCI).
  • To compare LCI with traditional spirometry measures (FEV1, FVC) in relation to various CF-related infections.
  • To assess the impact of different pathogens, including Staphylococcus aureus, Pseudomonas aeruginosa, and Aspergillus fumigatus, on LCI.

Main Methods:

  • A cohort of 136 pediatric CF patients (aged 5-18) were enrolled over one year.
  • Patients were categorized based on microbiological findings: Staphylococcus aureus, Pseudomonas aeruginosa (new, intermittent, chronic), Aspergillus fumigatus, or no pathogenic flora.
  • Lung clearance index (LCI) was measured using the multiple breath washout (MBW) technique, alongside spirometry (FEV1, FVC).

Main Results:

  • Patients with Aspergillus fumigatus infections showed significantly higher LCI compared to those with normal flora (P < 0.05).
  • Significant differences in LCI were observed between patients with chronic Pseudomonas aeruginosa infection and those with first-time infection (P < 0.05).
  • Distinct LCI values were noted between chronic Pseudomonas aeruginosa infected patients with FEV1 > 70% and FEV1 < 70% (P < 0.05).

Conclusions:

  • Lung clearance index (LCI) is demonstrably associated with the microbiological landscape in cystic fibrosis patients.
  • Chronic lung infections, specifically by Aspergillus fumigatus and Pseudomonas aeruginosa, correlate with elevated LCI.
  • Timely eradication of pathogenic microbial flora is crucial for maintaining lower LCI and potentially mitigating small airway disease progression in CF.

Related Concept Videos

Cystic Fibrosis: Pathogenesis01:23

Cystic Fibrosis: Pathogenesis

Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
864
Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
516
Lung Capacity01:47

Lung Capacity

The air in the lungs is measured in volumes and capacities. Lung volume measures reflect the amount of air taken in, released, or left over after a lung function, like a single inhalation. Lung capacity measures are sums of two or more lung volume measures.
56.2K
History of Microbiology01:28

History of Microbiology

Microbiology, a scientific field dedicated to the study of microorganisms, has undergone profound development since its inception in the 17th century. Its history is marked by key discoveries and technological advancements that have shaped our understanding of life at the microscopic level and transformed medicine, agriculture, and industry.Early Foundations of MicrobiologyThe early foundations of microbiology were built on groundbreaking observations and the development of pioneering...
9.0K
Three-Dimensional Microscopy in Microbiology01:28

Three-Dimensional Microscopy in Microbiology

Three-dimensional imaging techniques are essential in cell biology, allowing researchers to visualize intricate cellular structures with high resolution. Two prominent methods, Differential Interference Contrast Microscopy (DIC) and Confocal Scanning Laser Microscopy (CSLM), provide distinct advantages for imaging live and thick specimens, respectively.Differential Interference Contrast MicroscopyDIC microscopy enhances contrast in transparent, unstained samples by converting phase...
800
Hepatic Drug Clearance: Restrictive and Nonrestrictive Clearance01:09

Hepatic Drug Clearance: Restrictive and Nonrestrictive Clearance

Hepatic clearance refers to the volume of blood cleared of a drug by the liver per unit of time. It plays a crucial role in drug metabolism and elimination. While hepatic clearance is commonly estimated by subtracting renal clearance from total body clearance, other pathways, such as pulmonary or biliary clearance, may also contribute. However, these pathways are generally less significant than hepatic and renal clearance.
Most drugs undergo restrictive clearance, which is proportional to the...
507