11 yo with cystic fibrosis. Severe bronchiectasis affecting | Figure 1

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Case Summary

11 yo with cystic fibrosis. Severe bronchiectasis affecting entirety of right upper lobe with much less disease in other lobes. FEV1 30% and frequent pulmonary exacerbations at time of right upper lobectomy.

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Follow up to last chest X-ray - that showed #Bronchiectasis. This CT shows dilated airways and the signet ring sign (red arrow) - the airway accompanying a pulmonary arterial branch should be the same diameter but in #Bronchiectasis , the airway is wider. The underlying disorder is #Cystic-fibrosis.

A case about a 3 year old child, Caucasian, with normal growth and good social status, presents to the clinic with cough and fever for 3 days. During the past year he was diagnosed with pneumonia for three times. Blood count and markers for inflammation were normal - crp 8 mg/l. On auscultation - wheezing and crackles bilaterally. On X-ray - perihilar right pneumonia. We found out that this infiltrated is the same as on an X-ray a year ago. A CT showed an consolidation/subsegmental atelectasis 40/20mm, thickened dorsal pleura, areas with air trapping, stain glass opacities bilaterally. Mantoux test - 0 mm, adenosine deaminase slightly elevated 18.6 u/l. The child remained in a good overall condition during the treatment and no wheezing and crackles.

Coronal image from a chest CT shows a tracheal bronchus (arrow). A tracheal bronchus is an anatomic variant where an accessory bronchus or displaced bronchus arises from the trachea. The tracheal bronchus almost always supplies the right upper lobe. Patients with a tracheal bronchus are usually asymptomatic but may present with recurrent right upper lobe pneumonia or recurrent right upper lobe atelectasis if intubated. #ThoraxThursday #ThoracicThursday

This is a follow up from a previous case. #CFTR-gene deletion/duplication test has been ordered but no results yet. Any thoughts? We've found conflicting information on if this mutation may have any relevance considering her symptoms. A brief review, pt is a 32YOF who has SOB, frequent upper and lower respiratory infections, multiple sinus surgeries, idiopathic pancreatitis, etc. If further testing does not reveal any more mutations then CF is not the cause. However, based on symptoms this may be an atypical case of CF.