[Are ciliary abnormalities always present in Kartagener's syndrome? A study of 16 patients].
Kartagener's syndrome is defined as the combination of bronchiectasis, sinusitis and situs inversus. Assessment of characteristic ultrastructural changes and of their clinical expression was possible by examining the ciliated cells in the airways of 16 patients (12 children). Respiratory cilia of 11/13 patients were found to have abnormal motility. Quantitative ultrastructural data were obtained for 15 patients. Findings were absolutely normal in 2 cases. In 13 cases, ciliary abnormalities affected all the cilia, the majority of them (70-90%) or some of them (20-40%) (n = 7, n = 4, n = 2, respectively). The most frequently encountered aberration was a lacking external dynein arm. Summing it up: 1) ultrastructural ciliary abnormalities do not appear consistently in Kartagener's syndrome; 2) the lack of external dynein arms of cilia is the most frequent anomalous finding: 3) the clinical expression of ciliary dysfunction varies and the exact correlation between ultrastructural data and the intensity of the clinical manifestations remains to be established.
Escudier
E
E
Département d'Histologie-Embryologie et Biologie Cellulaire, Faculté de Médecine, Université Paris XII, Créteil.
Boucherat
M
M
Pinchon
M C
MC
Bernaudin
J F
JF
Peynègre
R
R
Fleury
J
J
D000328:Adult; D001980:Bronchi / Q000473:pathology; D002648:Child; D002675:Child, Preschool; D002923:Cilia / Q000648:ultrastructure*; D002925:Ciliary Motility Disorders / Q000209:etiology; D004398:Dyneins / Q000032:analysis; D005260:Female; D006801:Humans; D007223:Infant; D007619:Kartagener Syndrome / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009297:Nasal Mucosa / Q000473:pathology
D004398:Dyneins
Des anomalies ciliaires sont-elles toujours présentes dans le syndrome de Kartagener? Une étude chez 16 patients.