PRimary ciliary dyskinesia Flashcards
triad of kartagener
bronchiectasis
chronic sinusitis
situs inversus totalis (50%)
averenge length of cilia
6 micron
mature respi ciliated cell has how many cilia
200 cilia
uniform in size
contains ATPase providing energy for movemenr
dynein heavy chains
2 phases of ciliary motion
Stroke
Recovery
Phase wherein it sweeps forward
tips of cilia contacting mucus
to propel forward
Stroke phase
Bends Backward
EXtend into starting position
lose contact with mucus
recovery phase
normal beat frequency
8 to 14 Hz
normal beat rapidity is quicker in the
PROXImal airways
faster in young children (13 beats per second)
80% of px with pcd has this abnormality
dynein abnormalities
absent outer dynein arms
immotile
slightly flickering
radial spoke defect
isolated inner dynein arm
slow
stiff
ciliary transposition defects
lacks directional bend
normal frequency
NO level in pcd is
extremely low
compared to CF, PCD features
neonatal respi distress
otitis media
laterality defects
congenital heart defects
hydroceph
blindness
CF vs pcd
pancreatic insufficiency
diabetes
both cf and pcd
bronchitis/bronchiectasis
chronic sinusitis
nasal polyposis
male infertility
biliary cirrhosis
usual lobe affected in pcd neonates with respi distress 12-24 hrs of life
UPPER and MIDDLE lobe atelectasis
PCD high likely
neonatal distress with situs anomalies
distinctive features
Daily
year round productive (wet) cough
begins infancy
CT Scan findings in PCD
BRONCHIAL Wall thickening and mucus plugging
more than air trapping and hyperinflation
bronchiectasis in pcd usually affects what lobe
middle
lingual
lower lobes
PFT findings in PCD
normal during early childhood
Obstructive in adulthood
Reduced mmef
Reduced fev1
increased rv
elevated rv/tlc ratio
solid evidence for diagnosis
identification of impaired motility and specific ultrastructural defects
clinical:
daily productive cough
non seasonal rhinosinusitis
laterality defects
neonatal distress
