[Primary ciliary dyskinesia].
The immotile cilia syndrome is a genetic disease characterized by chronic disease of the upper and lower respiratory tract, male infertility and situs viscerum inversus in 50% of affected patients. Abnormalities of ciliary structure are the cause of abnormalities in mucociliary clearance and chronic inflammation consecutively. The boy aged 13 years with clinical manifestations (chronic rhinorrhea, sinuitis, recurrent otitis media, recurrent bronchial inflammation, pneumonia and finally bronchiectasis) has been presented. The boy had a normal visceral status. The diagnosis was confirmed by electron microscopy analysis of cross section of bronchial cilia: numerous abnormalities in microtubules positioning and lack of radiar arms have been found.
Raos
M
M
Specijalna bolnica za bolesti disnog sustava djece i mladezi Srebrnjak, Zagreb.
Bela-Klancir
S
S
Dodig
S
S
Koncul
I
I
Sćukanec-Spoljar
M
M
D000293:Adolescent; D006801:Humans; D007619:Kartagener Syndrome / Q000175:diagnosis*; D008297:Male