Related Experiment Video

Updated: Jun 18, 2026

Long Term Chronic Pseudomonas aeruginosa Airway Infection in Mice
15:43

Long Term Chronic Pseudomonas aeruginosa Airway Infection in Mice

Published on: March 17, 2014

Long-term pulmonary outcome after meconium ileus in cystic fibrosis

Matthias Kappler1, Maria Feilcke, Christian Schröter

  • 1Children's University Hospital of the Ludwig-Maximilians-University, Munich, Germany.

Pediatric Pulmonology
|November 14, 2009
PubMed

Insights

Cystic fibrosis (CF) patients with meconium ileus (MI) experienced earlier Pseudomonas aeruginosa infections but had similar long-term lung function outcomes compared to those without MI. Early diagnosis and treatment in CF patients with MI contribute to comparable long-term results.

Area of Science:

  • Pediatric Pulmonology
  • Genetics and Genetic Diseases

Background:

  • Meconium ileus (MI) is a common early manifestation of cystic fibrosis (CF).
  • Understanding the long-term outcomes of CF patients with a history of MI is crucial for optimizing care.

Purpose of the Study:

  • To identify CF patients with a history of MI.
  • To compare the long-term outcomes, including lung function and Pseudomonas aeruginosa infection, between CF patients with and without a history of MI.

Main Methods:

  • Retrospective analysis of 370 CF patients treated at a Munich CF center.
  • Classification of patients based on a history of MI.
  • Primary outcome variables included age at diagnosis, lung function (FEV% predicted), and time to first P. aeruginosa detection.

Main Results:

  • 16.2% of CF patients had a history of MI.
  • No significant difference in age or lung function (FEV1) was observed between groups in long-term follow-up.
  • CF patients with MI were diagnosed significantly earlier and had an earlier first detection of P. aeruginosa.

Conclusions:

  • While MI is associated with earlier P. aeruginosa infection, long-term lung function outcomes are comparable between CF patients with and without MI.
  • Earlier diagnosis and initiation of therapy in CF patients with MI may mitigate potential long-term disadvantages.
  • This suggests that early intervention is key to achieving similar long-term outcomes in CF patients with a history of meconium ileus.
Abstract

More Related Videos

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis05:56

Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

Published on: August 29, 2025

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections06:57

The WinCF Model - An Inexpensive and Tractable Microcosm of a Mucus Plugged Bronchiole to Study the Microbiology of Lung Infections

Published on: May 8, 2017

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, but...
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 sinusitis...
Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features01:24

Chronic Obstructive Pulmonary Disease III: Chronic Bronchitis Features

Chronic bronchitis is a key phenotype of chronic obstructive pulmonary disease (COPD), characterized by airway-centered inflammation and mucus overproduction. It develops from long-term exposure to harmful particles or gases, most commonly cigarette smoke, which triggers a persistent inflammatory response.Cellular and Structural ChangesInflammation initially affects the large bronchi and later the smaller airways, with infiltration by immune cells, including neutrophils, macrophages, and...
Chronic Obstructive Pulmonary Disease II: Emphysema01:23

Chronic Obstructive Pulmonary Disease II: Emphysema

Emphysema, a major phenotype of chronic obstructive pulmonary disease (COPD), is characterized by irreversible destruction of alveolar walls and permanent enlargement of distal airspaces. Unlike chronic bronchitis, which primarily affects the airways, emphysema predominantly involves the lung parenchyma, where structural damage leads to airflow limitation.PathophysiologyIt most commonly results from prolonged exposure to cigarette smoke and other toxic gases, particularly cigarette smoke.
Atelectasis II: Pathophysiology01:10

Atelectasis II: Pathophysiology

Atelectasis develops when alveoli lose their air and collapse inward. Because lung tissue is naturally elastic, these air sacs shrink rather than remaining open. Collapsed alveoli are no longer ventilated, reducing their role in gas exchange. Blood flow may continue in these regions, creating a ventilation–perfusion mismatch. Clinical findings include decreased breath sounds, dullness to percussion, reduced chest expansion, and decreased tactile fremitus as sound transmission through collapsed...
Pulmonary Cycle: Exhalation01:17

Pulmonary Cycle: Exhalation

In terms of human respiration, the act of expelling air, known as exhalation (or expiration), operates on the principle of pressure gradients. During expiration, the pressure within the lungs exceeds that of the surrounding atmosphere. Under normal conditions, quiet breathing involves passive exhalation and is free of muscular contractions. This is because the exhalation process is driven by the natural elastic recoil of the lungs and chest wall, both of which have an inherent tendency to...