Primary Ciliary Dyskinesia (Kartagener Syndrome) Questions & Answers

Updated: Jun 12, 2020
  • Author: Elena B Willis, MD; Chief Editor: John J Oppenheimer, MD  more...
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Questions & Answers

Overview

What is primary ciliary dyskinesia (Kartagener syndrome)?

How was ciliary dyskinesia (Kartagener syndrome) first described?

What is the pathophysiology of primary ciliary dyskinesia (Kartagener syndrome)?

What causes primary ciliary dyskinesia (Kartagener syndrome)?

What is the prevalence of primary ciliary dyskinesia (Kartagener syndrome) in the US?

What is the sexual predilection of primary ciliary dyskinesia (Kartagener syndrome)?

Which age groups have the highest prevalence of primary ciliary dyskinesia (Kartagener syndrome)?

What is the prognosis of primary ciliary dyskinesia (Kartagener syndrome)?

What is the morbidity associated with upper airway primary ciliary dyskinesia (Kartagener syndrome)?

What is the morbidity associated with lower respiratory tract primary ciliary dyskinesia (Kartagener syndrome)?

What possible complications of primary ciliary dyskinesia (Kartagener syndrome)?

Presentation

Which clinical history findings are characteristic of primary ciliary dyskinesia (Kartagener syndrome)?

What is the clinical triad characteristic of primary ciliary dyskinesia (Kartagener syndrome)?

Which upper respiratory findings are characteristic of primary ciliary dyskinesia (Kartagener syndrome)?

Which lower respiratory tract findings are characteristic of primary ciliary dyskinesia (Kartagener syndrome)?

What other findings may be seen in primary ciliary dyskinesia (Kartagener syndrome)?

DDX

What are the differential diagnoses for Primary Ciliary Dyskinesia (Kartagener Syndrome)?

Workup

What is the role of lab testing in the workup of primary ciliary dyskinesia (Kartagener syndrome)?

What is the role of imaging studies in the workup of primary ciliary dyskinesia (Kartagener syndrome)?

Which tests are performed for primary ciliary dyskinesia (Kartagener syndrome) screening?

What pulmonary function studies are used in the workup of primary ciliary dyskinesia (Kartagener syndrome)?

What is the role of biopsy in the workup of primary ciliary dyskinesia (Kartagener syndrome)?

What is the role of nasal endoscopy in the workup of primary ciliary dyskinesia (Kartagener syndrome)?

Which histologic findings are characteristic of primary ciliary dyskinesia (Kartagener syndrome)?

Treatment

How is primary ciliary dyskinesia (Kartagener syndrome) treated?

How does smoking affect the treatment of primary ciliary dyskinesia (Kartagener syndrome)?

What is the role of surgery in the treatment of primary ciliary dyskinesia (Kartagener syndrome)?

Which specialist consultations are beneficial to patients with primary ciliary dyskinesia (Kartagener syndrome)?

Which activity modifications are needed during the treatment of primary ciliary dyskinesia (Kartagener syndrome)?

Medications

Which medications are used for treatment of primary ciliary dyskinesia (Kartagener syndrome)?

Which medications in the drug class Expectorants are used in the treatment of Primary Ciliary Dyskinesia (Kartagener Syndrome)?

Which medications in the drug class Antibiotics are used in the treatment of Primary Ciliary Dyskinesia (Kartagener Syndrome)?