Bronchiectasis, Kartagener Xn

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Step 1: View clinicals

A 35 year old woman from South Asia presents with fever and a cough productive of yellowish, viscid sputum for one week. She gives a history of similar episodes dating from childhood, which were usually managed on an outpatient basis. Her medical history is otherwise unremarkable She does not smoke, and has been married for 5 years, with no children.


Step 2: Order all relevant investigations

X-Ray Chest

There are cystic shadows in the lower zones of both lungs. Dextrocardia is present.

Echocardiogram

Dextrocardia with situs inversus is noted. Atrioventricular concordance is present. No valvular or structural defects are seen. The great arteries and veins appear structurally normal.

CT Chest

There is bronchiectasis of the right middle and lower lobes and the left lower lobe. The positions of the thoracic viscera are reversed.

Ultrasound Abdomen

The positions of the abdominal viscera are reversed.


Step 3: Select appropriate management

Antibiotics
Dornase alfa
Pulmonary resection
Physiotherapy


Score: ★★☆