A 27-year-old man is seen with a history of chronic sinus and pulmonary infections. He works as a salesperson in a retail outlet and denies any specific occupational exposure. He lives with his wife of 4 years and has no children. Family and travel history is noncontributory. On examination, he is in no acute distress. Lung exam reveals crackles in both lower lung zones and extremities show no clubbing. CXR is shown below.The most likely diagnosis is
This x-ray shows a bilateral lower zone hazy density with small cystic-appearing shadows. There is dextrocardia with situs inversus totalis, i.e., a right aoic arch, the stomach bubble on the right side, and the left diaphragm higher than the right. The chest x-ray shows dextrocardia and bilateral bronchiectasis as seen in immotile cilia syndrome. Kaagener syndrome is the immotile cilia syndrome with situs inversus totalis.This is an autosomal recessive disease characterized by structural and functional abnormalities of the cilia resulting in impaired mucus clearance, recurrent infection, chronic sinobronchial infections, and infeility. Frontal sinuses are often absent or hypoplastic. Chronic mucus impaction and infections lead to bronchiectasis.
IgA deficiency
Kaagener syndrome
Aspiration pneumonia
Cystic fibrosis
Respiratory system