Wolfgang Hirsch (Halle)  

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15 Years  

N/A  

Thorax-Lung  

congenital  

15 year-old patient, illness known since 2 years of age. Chronic cough. At the moment, exertion dyspnea, a hint of drumstick fingers. Proof of pseudomonas aeruginosa in broncial secretions.  

Defect of the chloride canal's membrane proteins. Autosomal recessive genetic point mutation on chromosome 7. Many different courses of disease, probably due to the fact that the mutations present themselves in different variations (>700).

In this case, a very severe pulmonary course as well as pronounced extrapulmonary symptoms.  

<- view X-Ray 1

X-ray 1: Multiple "dotty" confluent opacities particularly in the middle lobe of both sides. Hilary lymph node swelling on both sides. In the upper lobes, there is an increase of transparency (emphysema). In the lower lobes, track-like stripe pattern (bronchiectasias). Generalized emphasis of the interstital pulmonary structure. The Chrispin-Norman score is 20 (lateral image not shown, obviously over-inflated.)




<- view CT 1

CT 1: Slice just above the carina: multiple round infiltrates. Old scar tissue to the right, ventral. Even here, one sees bronchiectasias (Seal-ring configuration: Wide bronchus, thin corresponding vessel).




<- view CT 2

CT 2: Slice in the level of the atria. There are multiple bronchiectasias and infiltrates to be seen.




<- view CT 3

CT 3: Bronchiectasias in the enlargened image: Not only the widening of the brochi can be easily identified, but also their obvious thickened walls and their unevenness and peribronchial infiltrates.




<- view MRI 1

MRI 1: FLASH 3D-image coronal in the level of the trachea. As seen in the CT image, the infiltrates and the brochiectasias are emphasized dorsally, so that there are less infiltrates seen ventrally in the upper and middle lobes.




<- view MRI 2

MRI 2: Mucous retention and the multiple pulmonary infiltrates are seen very well further dorsally. The bronchiectasias themselves (as in X-ray) are less obvious, but rather the thickened bronchial walls can be seen. One sees the aorta to the left of the spine.




<- view MRI 3

MRI 3: Another coronal slice, right below the slice of MRI 2. Through sequential analysis of the 2-3 mm slicese, the lung can be sufficiently assessed in MRI in patients with cystic fibrosis. Obstructive changes are hard to see in MRI; they can be seen in MPR-reconstruction through the larger p.a. diamter of the thorax.

 

Moleculary genetics  

chronic pulmonary infections, Wegner granulomatosis.  

N/A  

The goal of reducing radiation exposition in children is accomplished by substituting CT examinations with toher radiation-free procedures. In cystic fibrosis patients, this is possible (however, accepting losses of information when compared to CT images). In more consistent and comparable MRI-investigations, the thoracic x-ray follow-ups can be reduced in frequency or avoided, since the information recieved by the MRI regarding the lung is higher.  

N/A  

N/A  

Cystic fibrosis, bronchiectasis, infiltrate, lung diagnostics with MRI, exertion dyspnea, drumstick fingers, Pseudomonas Aeruginosa, child, childhood, pediatric radiology  

Wolfgang Hirsch. Cystic fibrosis / Lung diagnostic with MRI. PedRad [serial online] vol 2, no. 11.
URL: www.PedRad.info/?search=20021127102045