Which syndrome is characterized by ciliary dyskinesia and situs inversus?

Prepare for the ACAAI Board Exam. Utilize flashcards and comprehensive multiple-choice questions, equipped with hints and detailed explanations. Ace your allergy and clinical immunology exam efficiently.

Kartagener's syndrome is characterized by a triad of symptoms: bronchiectasis, chronic sinusitis, and situs inversus. The underlying issue in this condition is primary ciliary dyskinesia, which refers to the impaired movement of cilia due to a genetic defect, leading to difficulty in clearing mucus from the respiratory tract and resulting in chronic respiratory infections. The association with situs inversus occurs because the dysfunction in ciliary movement affects left-right asymmetry during embryonic development, leading to the mirror-image placement of organs.

While primary ciliary dyskinesia also describes the ciliary dysfunction, it doesn't specifically encompass the additional features of situs inversus and bronchiectasis, which are definitive elements of Kartagener's syndrome. Cystic fibrosis, on the other hand, involves a different pathophysiological mechanism related to CFTR gene mutations and primarily affects the lungs and pancreas without the situs inversus characteristic. Churg-Strauss syndrome involves an autoimmune component affecting small to medium-sized blood vessels and has no association with ciliary dyskinesia or situs inversus. Thus, the clinical picture of Kartagener's syndrome aligns perfectly with the symptoms described in the question, making it the correct choice.

Subscribe

Get the latest from Examzify

You can unsubscribe at any time. Read our privacy policy