PRimary ciliary dyskinesia

Question 1

triad of kartagener

Answer 1

bronchiectasis
chronic sinusitis
situs inversus totalis (50%)

Question 2

averenge length of cilia

Answer 2

6 micron

Question 3

mature respi ciliated cell has how many cilia

Answer 3

200 cilia
uniform in size

Question 4

contains ATPase providing energy for movemenr

Answer 4

dynein heavy chains

Question 5

2 phases of ciliary motion

Answer 5

Stroke
Recovery

Question 6

Phase wherein it sweeps forward
tips of cilia contacting mucus
to propel forward

Answer 6

Stroke phase

Question 7

Bends Backward
EXtend into starting position

lose contact with mucus

Answer 7

recovery phase

Question 8

normal beat frequency

Answer 8

8 to 14 Hz

Question 9

normal beat rapidity is quicker in the

Answer 9

PROXImal airways
faster in young children (13 beats per second)

Question 10

80% of px with pcd has this abnormality

Answer 10

dynein abnormalities

Question 11

absent outer dynein arms

Answer 11

immotile
slightly flickering

Question 12

radial spoke defect
isolated inner dynein arm

Answer 12

slow
stiff

Question 13

ciliary transposition defects

Answer 13

lacks directional bend

normal frequency

Question 14

NO level in pcd is

Answer 14

extremely low

Question 15

compared to CF, PCD features

Answer 15

neonatal respi distress
otitis media
laterality defects
congenital heart defects
hydroceph
blindness

Question 16

CF vs pcd

Answer 16

pancreatic insufficiency
diabetes

Question 17

both cf and pcd

Answer 17

bronchitis/bronchiectasis
chronic sinusitis
nasal polyposis
male infertility
biliary cirrhosis

Question 18

usual lobe affected in pcd neonates with respi distress 12-24 hrs of life

Answer 18

UPPER and MIDDLE lobe atelectasis

Question 19

PCD high likely

Answer 19

neonatal distress with situs anomalies

Question 20

distinctive features

Answer 20

Daily
year round productive (wet) cough
begins infancy

Question 21

CT Scan findings in PCD

Answer 21

BRONCHIAL Wall thickening and mucus plugging

more than air trapping and hyperinflation

Question 22

bronchiectasis in pcd usually affects what lobe

Answer 22

middle
lingual
lower lobes

Question 23

PFT findings in PCD

Answer 23

normal during early childhood
Obstructive in adulthood

Reduced mmef
Reduced fev1

increased rv
elevated rv/tlc ratio

Question 24

solid evidence for diagnosis

Answer 24

identification of impaired motility and specific ultrastructural defects

clinical:
daily productive cough
non seasonal rhinosinusitis
laterality defects
neonatal distress