Ciliary ultrastructure in two sisters with Kartagener's syndrome.
Kartagener's syndrome (KS) is a clinical variant of primary ciliary dyskinesia involving situs inversus associated with chronic airway infections. We studied two sisters; the elder one had dextrocardia and scoliosis, and the younger one had situs inversus of the lung, liver, and stomach as well as dextrocardia. Both patients had chronic sinusitis and chronic bronchitis with bronchiectasis. In both cases, the ciliary defect associated with this syndrome is the absence of inner dynein arms.
Tanaka
Kayoko
K
Division of Morphological Science, Biomedical Research Center, Saitama Medical University, 38 Moroyama, Iruma-gun, Saitama 350-0495, Japan.
Sutani
Akihisa
A
Uchida
Yuka
Y
Shimizu
Yoshihiko
Y
Shimizu
Michio
M
Akita
Masumi
M
D000328:Adult; D001987:Bronchiectasis / Q000473:pathology; D029481:Bronchitis, Chronic / Q000473:pathology; D002923:Cilia / Q000648:ultrastructure*; D003914:Dextrocardia / Q000473:pathology; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000473:pathology*; D008099:Liver / Q000473:pathology; D008168:Lung / Q000473:pathology / Q000648:ultrastructure; D046529:Microscopy, Electron, Transmission; D020545:Respiratory Mucosa / Q000473:pathology / Q000648:ultrastructure*; D012600:Scoliosis / Q000473:pathology; D035781:Siblings; D012852:Sinusitis / Q000473:pathology; D012857:Situs Inversus / Q000473:pathology; D013270:Stomach / Q000473:pathology