Problems in the differential diagnosis of Kartagener's syndrome and ATP-ase deficiency.
Two cases of Kartagener's syndrome with a partial loss of the dynein arms and the central spokes within the cilia are presented. In light microscopic examination the predominant cell types were mast cells within the stroma and the epithelium and plasma cells and lymphocytes in the stroma. The reaction for the magnesium-activated ATP-ase located in the apical cytoplasm of ciliated cells was negative in one case and slightly positive in the same case on year later; in our second case, the ATP-ase reaction was variable, slightly to moderately positive, in the same biopsy. Besides the dynein arm defect this is a second defect of another ATP-ase, located in the apical region of the ciliated and mucus producing epithelial cells. Based on histological examination of nasal mucosal biopsies a differential diagnosis has been established for Kartagener's syndrome, the more common cystic fibrosis and the relative frequent allergic rhinitis.
D000251:Adenosine Triphosphatases / Q000172:deficiency*; D000328:Adult; D002648:Child; D002923:Cilia / Q000648:ultrastructure; D003550:Cystic Fibrosis / Q000473:pathology; D004398:Dyneins / Q000378:metabolism; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000175:diagnosis* / Q000473:pathology; D008854:Microscopy, Electron; D009297:Nasal Mucosa / Q000473:pathology; D012221:Rhinitis, Allergic, Perennial / Q000473:pathology
D000251:Adenosine Triphosphatases; D004398:Dyneins