Learning objectives
Cystic fibrosis (CF) is a relatively common disease.
Radiologists should be familiar with it and the acute and chronic complications of this disorder.
The purpose of this paper is to exhibit the imaging findings that allow us to identify common and less frequent acute complications of patients with CF.
Background
CF is one of the most common fatal genetic diseases in Caucasians,
affecting approximately 1 in every 2000-4000 children born yearly.
It is an autosomal recessive disorder and it is estimated that 1/25 people are carriers of the defective gene.
The CF gene and its product,
the CF transmembrana conductance regulator (CFTR),
cause abnormal chloride ion transport on the apical surface of exocrine gland epithelial cells.
This results in abnormally thickened,
viscous secretions,
which affect multiple organ systems (lungs,
pancreas,
intestine,
liver…).
Over 1500 different...
Imaging findings OR Procedure details
Imaging findings in Cystic Fibrosis:
- Chest X-ray: It may be normal in early stages Fig. 5or may show nonspecific linear images in the lower lobes.
As the disease progresses,
thickening of these linear images and even tubular lesions with the appearance of "rail station",
characteristic of cylindrical bronchiectasis Fig. 6 are identified.
In advanced stages saccular bronchiectasis as annular images in both lungs,
sometimes full of mucous or fluid levels inside Fig. 7 Fig. 8 Fig. 9 are found.
Others common findings are hyperinflation...
Conclusion
Because CF is a chronic and multisystem disease,
these patients are recurrent candidates to the Hospital Services including the Emergency Services.
It is important to know the typical imaging findings of these patients,
both in acute and chronic complications for a proper interpretation of the images
References
- Review of the Abdominal Manifestations of Cystic Fibrosis in the Adult Patient.
M B Robertson,
K A Choe,
P M Joseph.
RadioGraphics 2006; 26: 679-690.
- Hepatobiliary and Pancreatic Manifestations of Cystic Fibrosis: MR Imaging Appearances.
L King,
E Scurr,
N Murugan,
S Williams,
D Westaby,
J Healy.
RadioGraphics 2000; 20: 767-777.
- Cystic Fibrosis: CT Assessment of Lung Involvement in Children and Adults.
T H Helbich,
G Heinz-Peer,
I Eichler,
P Wunderbaldinger,
M Götz,
C Wojnarowski,
R C Brasch,
Ch J Herold.
Radiology...