[Ultrastructural changes of the nasal mucosa in primary ciliary dyskinesia].
Primary ciliary dyskinesia syndrome (PCD) is a rare, autosomal receive disorder. Kartagener's syndrome is a subgroup of the PCD with situs inversus, bronchiectasis, and sinusitis. The symptoms results from an abnormal ultrastructural morphology of the cilia such as absence of dynein arms and other changes. As a consequence ciliary motility is disturbed. A 25-year-old man was examined because he suffered from recurrent severe pneumonia and Aspergillus infections of the lungs. On electron micrographs, ciliary abnormalities including deficiency of inner and outer dynein arms, dysmorphic outer dynein arms, and disorientation of the cilia were demonstrated. The diagnosis of PCD requires electron-microscopic investigations of the ciliated mucosa. Special attention should be given to ultrastructural changes of nasal or bronchial mucosa if a young patient suffers from recurrent severe respiratory infections.
Knipping
S
S
Universitätsklinik und Poliklinik für Hals-, Nasen-, Ohrenheilkunde, Kopf- und Halschirurgie, Martin-Luther-Universität Halle-Wittenberg, Magdeburger Strasse 12, 06097 Halle/Saale.
Holzhausen
H J
HJ
Wollschläger
B
B
Mir Salim
P A
PA
Berghaus
A
A
D000328:Adult; D001228:Aspergillosis / Q000235:genetics / Q000473:pathology*; D001996:Bronchopneumonia / Q000235:genetics / Q000473:pathology*; D002869:Chromosome Aberrations; D002925:Ciliary Motility Disorders / Q000235:genetics / Q000473:pathology*; D005808:Genes, Recessive / Q000235:genetics; D006801:Humans; D008172:Lung Diseases, Fungal / Q000235:genetics / Q000473:pathology*; D008297:Male; D008854:Microscopy, Electron; D009079:Mucociliary Clearance / Q000235:genetics / Q000502:physiology*; D009297:Nasal Mucosa / Q000473:pathology*; D012008:Recurrence; D022142:Secretory Vesicles / Q000648:ultrastructure; D014057:Tomography, X-Ray Computed