Primary ciliary dyskenesia syndrome. (Kartagener syndrome =subset med dextrocardi/situs inversus. 50% av dessa patienter. 20% av dextrocarida has kartageners).
assos: pyl stenos, hypospadi, post cricoid web. defective neutrophil chemotaxis.
autosomal res. drabbade cilia o spermier---> dextrocarida pga ingen rotation in utero.
chronic sinusitis, bronchiektasier(cylindrisk, vaircose,cystic) i mittlapp/underlapper.
brochiolektasier=tree in bud
air trapping pga small airway disease
mosaic perfusion pga obliterative bronchiolit
HRCT centrilobular small nodules up to 2 cm
hyperinfaltion, återkommande penumonier
diff: CF upper llobe bronchiektasier, mucoid impacions, polyper och kronisk sinusit, svett test. inte ciliær immotilitet.