cystic fibrosis
A genetic disorder affecting multiple organ systems, with characteristic chest imaging findings requiring specialized pediatric radiological interpretation.
Full Definition
Cystic fibrosis (CF) is an autosomal recessive genetic disorder that primarily affects the respiratory and digestive systems. In pediatric radiology, CF patients require frequent chest imaging to monitor disease progression and complications. Characteristic radiological findings include bronchiectasis, mucus plugging, hyperinflation, and eventually pulmonary fibrosis. The Brasfield scoring system is used to grade disease severity on chest radiographs. CF patients also may develop complications such as pneumothorax, hemoptysis, and allergic bronchopulmonary aspergillosis (ABPA). High-resolution CT is increasingly used for detailed assessment of airways disease.
Usage
Usage note: Acceptable to abbreviate as CF after first use in a document.
In Context
- "The chest radiograph showed typical cystic fibrosis changes with increased bronchial markings." — Pulmonology clinic note
- "High-resolution CT demonstrated early bronchiectasis consistent with cystic fibrosis progression." — Radiology report