[Incomplete ciliary axonema: anther cause of ciliary dysmotility syndrome?].
Immotile cilia syndrome is associated with different ciliary defects, although the clinical presentation is similar in every case. A study was made of a 36-year-old woman with recurrent respiratory infections since birth, chronic sinusitis and chronic bronchitis with bronchiectasias. Her medical history included a tubaric pregnancy and two miscarriages. Nasal mucociliary transport was investigated on two occasions at a 1-year interval using an isotopic technique. Ciliary ultrastructure was studied by electron microscopy after obtaining two biopsies from the inferior and middle turbinates separated by a 1-year interval. The sweat test and blood immunoglobulins were normal. The absence of mucociliary transport was verified on both occasions. An abnormality was observed in 30% of the cilia in the form of semicircular ciliary cross-sections, with only 7 pairs of peripheral microtubules. The central pair was normal. We termed this anomaly "incomplete ciliary axonema" and believe that it could be another cause of immotile cilia syndrome.
Armengot
M
M
Servicio de ORL, Hospital General Universitario, Valencia.
Carda
C
C
Basterra
J
J
D000328:Adult; D001706:Biopsy; D002925:Ciliary Motility Disorders / Q000209:etiology* / Q000473:pathology*; D005260:Female; D006801:Humans; D008854:Microscopy, Electron; D009297:Nasal Mucosa / Q000473:pathology / Q000648:ultrastructure*; D013577:Syndrome