Presentation
Carrier of William-Campbell syndrome and heterozygous for CFTR. On oxygen therapy.
Patient Data
Age: 54
Gender: Female
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Mid to upper zones ring shadows suggestive of bronchiectasis.
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Cystic and varicose bronchiectasis in the upper lobes and in the apical segments of the lower lobes.
Case Discussion
Williams-Campbell syndrome is characterized by complete absence or insufficient development of the cartilaginous bronchial rings of the third-eighth bronchial orders. The trachea and main bronchi have a normal structure.
An autosomal recessive inheritance is presumed.
The lungs are usually hyperinflated.
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