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Updated: Jul 20, 2026

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
[Thoracic findings in pediatric patients with cystic fibrosis]
1Abteilung Radiologie, Vestische Kinder- und Jugendklinik, Universität Witten/Herdecke, Datteln. R.Wunsch@kinderklinik-datteln.de
Insights
Cystic fibrosis (CF) is a genetic disorder affecting fluid balance, leading to lung disease. Improved diagnostics and treatments have increased life expectancy for CF patients.
Area of Science:
- Genetics and Molecular Biology
- Respiratory Medicine
- Medical Imaging
Context:
- Cystic fibrosis (CF) is the most prevalent autosomal recessive metabolic disease in Europe.
- Lung disease severity is the primary determinant of morbidity and mortality in CF patients.
- Advances in diagnostics and therapeutics have significantly improved CF patient life expectancy.
Purpose:
- To summarize the underlying pathology of cystic fibrosis.
- To review current and emerging radiological diagnostic tools for CF lung disease.
Summary:
- CF results from a defect on chromosome 7, impacting cell membrane fluid balance (chloride and sodium transport).
- This defect causes viscous mucus buildup in exocrine glands, obstructing airways and leading to lung infections and parenchymal destruction.
- While chest X-ray remains standard, CT offers earlier and more precise morphological changes, and MRI shows promise for functional assessment.
Impact:
- Enhanced understanding of CF pathophysiology.
- Highlights the evolving role of medical imaging in CF diagnosis and management.
- Informs future research directions for CF imaging modalities.
Abstract:
Cystic fibrosis is the most common autosomal recessive metabolic disease in Europe with an incidence of 1:2500. The severity of the lung disease is the most important factor of morbidity and mortality in CF-patients. Because of the better diagnostic and therapeutic modalities life expectancy has increased significantly. The underlying pathology is a defect of chromosome 7, which encodes the regulation of the fluid balance across the cell membrane which effects chloride as well as sodium. The exocrine glands produce a viscous mucus which obstructs the airways and promotes infections. The result is the destruction of lung parenchyma. In daily routine, chest x-ray is still the most important radiological tool, although computed tomography depicts changes in morphology earlier and more exactly. Recent research studies show that MRI has-because of its additional functional options-interesting aspects for the future.
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Assessment:
1. Clinical Evaluation:
History:

