Please enable JavaScript.
Coggle requires JavaScript to display documents.
Primary ciliary dyskinesia (Clinical presentation (Recurrent productive…
Primary ciliary
dyskinesia
Definition
Congenital disorder of cilia
affecting the respiratory tract
Epidemiology
Rare
Pathophysiology
Defect
Mutation affecting structure/function of cilia
Cilia needed for clearance of resp tract, also involved in normal organ status during foetal development
Mechanism
Impaired mucociliary clearance and infections
Can lead to severe bronchiectasis
May have Kartagener syndrome (major organs in mirror position of normal)
Clinical
presentation
Recurrent productive cough
Purulent nasal discharge
Chronic ear infections
Diangosis
Examination
Resp
Clubbing, bilateral widespread crackles,
productive cough (bronchiectasis)
Cardio
Dextrocardia (Kartagener syndrome)
Investigations
Bedside
Obs
Swabs
Nasal (microscopy of cilia)
Sputum (MCS)
History
DH
Meds, allergies
FH
Resp disorders
PMH
Growth and development
Vaccinations
Known medical conditions
POH
Bloods, scans, growth, gestation,
delivery, weight, complications
PC/HPC
Recurrent infections
SH
Living arrangements, school/nursery,
family smoking status
Management
Conservative
Information, advice, support
Daily physiotherapy
Medical
Abx
Indication: RTI