6533b828fe1ef96bd12890aa
RESEARCH PRODUCT
Ultrastructural patterns of primary ciliar dyskinesia syndrome.
Miguel ArmengotAmando PeydróCarmen CardaAmparo Escribanosubject
AdultMalePathologymedicine.medical_specialtyAdolescentMucociliary clearanceBiologyPathology and Forensic MedicineDiagnosis DifferentialMicroscopy Electron TransmissionStructural BiologymedicineHumansCiliaChildPrimary ciliary dyskinesiaKartagener SyndromeKartagener SyndromeDyneinsInfantAnatomyMiddle Agedmedicine.diseaseSitus inversusNasal MucosaDyskinesiaHomogeneousMucociliary ClearanceUltrastructureFemalemedicine.symptomCiliary ultrastructuredescription
Clinical presentation, ciliary ultrastructure, and nasal mucociliary transport by a radioisotopic technique were analyzed in 14 Kartagener syndrome patients. In this study the most common pattern was the absence of outer and inner dynein arms in 57% of cases. Also reported are 14% patients with short inner dynein arms. A total of 29% of the patients showed normal dynein arms. Mucociliary stasis was observed in 13 cases. Primary ciliary dyskinesia syndrome and Kartagener syndrome are clinically homogeneous and morphologically heterogeneous. The authors conclude that a typical clinical presentation with an altered mucociliary transport obtained by radioisotopic technique is diagnostic although ciliary ultrastructure is normal.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2005-06-04 | Ultrastructural pathology |