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Primary Ciliary Dyskinesia

 Syn. Immotile-cilia syndrome , Kartagener syndrome , Kartageners syndrome

Signs & Symptoms

 Facial pressure
Note: due to Rhinosinusitis
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Persistent mucopurulent drainage
Note: due to Rhinosinusitis
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Nasal blockage
Note: due to Rhinosinusitis
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Nasal obstruction
Note: due to Rhinosinusitis
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]

Physical Examination

 Tachypnea
Note: Seen in Newborns
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Mild hypoxemia
Note: Seen in Newborns
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Respiratory distress
Note: Seen in Newborns
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]

Lab / Serology

 Absent nasal nitric oxide
Note: A useful screening test for patients age 5 years or older
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Very low nasal nitric oxide
Note: A useful screening test for patients age 5 years or older
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]

Imagin Study

 Atelectasis
Note: HRCT and chest radiograph
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Bronchiectasis
Note: HRCT and chest radiograph, Seen in in all adults and approximately 50 percent of children
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Small centrilobular nodules
Note: due to bronchiolitis
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]

Complication

 Infertility Gr. Female
Note: due to Impaired ciliary function in the fallopian tubules
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Infertility Gr. Male
Note: due to Dyskinetic spermatozoa
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]
Conductive hearing loss
Note: due to Chronic secretory otitis media
Ref: Uptodate [ Primary ciliary dyskinesia (immotile-cilia syndrome)]

Risk Factors

Cause

Other