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PubMed1985

Electron microscopy in rhinology.

In rhinology, electron microscopy has been a useful research tool for the past 15 years, but provided only a few direct clinical applications. In this review, the author's work on the human nasal mucosa and the studies of other investigators are discussed, with the emphasis on allergic reactions and disturbances of the autonomous nervous system as well as the immotile cilia syndrome.

Autonomic Nervous System↗
PubMed1984

[Immotile cilia disease with neonatal disclosure. Ultrastructural study].

Recurrent bronchopulmonary and E.N.T. infections in a 2-month old child with complete situs inversus suggested an immotile cilia syndrome. Electron microscopy of the respiratory epithelium cilia demonstrated an ultra structural abnormality (defective radial spokes) typical of this recently discovered syndrome. This case is similar to 3 other cases in infants reported in the literature. It shows that the clinical manifestations of ciliary dysfunction may occur soon after birth and that early detection is desirable for optimal treatment. The type of abnormality detected and the percentage of cilia affected demonstrate that the syndrome is congenital and not acquired and provide information of the degree of ciliary dyskinesia. The genetic aspects are discussed. A diagnosis of "immotile cilia" syndrome should systematically be envisaged in infants with recurrent pneumonia or otitis of unknown aetiology, or when the clinical context (situs inversus, family history) is suggestive of the conditions.

Bronchi↗
PubMed1984

[Transmission electron microscopy study of the nasal cilia].

The authors draw the attention on the existence of dyskinetic and immotile cilia in patients with chronic respiratory infections of unknown origin. The study of the ultrastructure of the ciliae is done by a transmission electron microscope. It is very important to make a difference between primary and acquired anomalies. Furthermore, the authors emphasize the importance of early diagnosis in view of a treatment.

Adult↗
PubMed1984

[How useful is the ultrastructural study of the cilia of the respiratory tract in the diagnosis of an immotile cilia syndrome?].

The immotile cilia syndrome (ICS) comprises a range of congenital defects of the ciliary apparatus most probably transmitted by autosomal recessive inheritance. Because cilia occur mainly in the respiratory and genital tract, the clinical symptoms of ICS are most commonly chronic sinusitis, bronchitis, bronchiectasis and male sterility. The syndrome can be associated with a situs inversus and is then called Kartagener's syndrome. We studied the ciliary ultrastructure in airway biopsies of 5 patients suffering from chronic upper and lower respiratory tract infections. With the single exception of one female patient with confirmed ICS diagnosis (Kartagener's syndrome) the etiology of the recurrent infections was unknown. The following ciliary defects were observed: missing dynein arms, radial spoke defects, missing nexin links, microtubular transpositions, compound cilia, supernumerary, absent, or incomplete microtubules, lack of ciliary orientation and various abnormal patterns of microtubular arrangement. In no instance did a patient show only a single anomaly; defects were always combined. Missing dynein arms, radial spoke defects and microtubular transpositions have frequently been described as lesions specific for ICS. Whenever these lesions were found simultaneously in both the respiratory and genital tracts, their genetic origin cannot be doubted. In our confirmed ICS patient the outer dynein arms were not missing but were reduced in number and length in a large number of cilia. The biopsy was, however, obtained from the heavily infected maxillary sinus and it is known that inflammation can lead to a loss of dynein arms. In the light of our investigations and of a review of the published cases of ciliary anomalies, it is concluded that none of the above defects in itself is specific for ICS. They may all occur as secondary lesions or sporadically as varieties in otherwise healthy subjects. It therefore appears questionable whether ICS can be diagnosed from the ciliary ultrastructure of a single airway biopsy. Assessment of ICS cannot be based simply on the ultrastructural demonstration of a particular ciliary defect, but necessitates additional considerations particularly regarding the origin of the biopsy, the sampling procedures and quantitation of defects. It appears necessary to investigate samples from different parts of the airways and quantitatively analyze the prominent lesions.

Abnormalities, Multiple↗