Radiology – Integrated Training Initiative (R-ITI) | Paediatrics | Cystic Fibrosis

Cystic Fibrosis
Session Overview
Description
This session is designed to show the various clinical and radiological manifestations of cystic fibrosis and the role of imaging.

Learning Objectives
By the end of this session you will be able to:

• Describe the spectrum of radiological manifestations of cystic fibrosis
• Interpret radiographs and computed tomography (CT) of the chest in cystic fibrosis
• Describe the role of imaging in the management of cystic fibrosis

Prerequisites
Before commencing this session you should:

• Completed session in Module 5 Paediatrics/How to Interpret the Paediatric Chest X-ray (300-0441)

Cystic fibrosis (Fig 1) is an autosomal recessive disorder in which there is mutation of the cystic fibrosis transmembrane conductance regulator gene (CFTR). This leads to a lack of chloride ion secretion, increased sodium retention and fluid absorption.

You should be aware of the following facts regarding cystic fibrosis:

• Its incidence
• The course of the disease
• Associated abnormalities
• Treatment