[Ciliary disorders of the bronchi in children].

Abstract

The present study relates to 39 children, 24 boys and 15 girls, aged 1 to 17 (mean age, 6 1/2 years) suffering from chronic airflow obstruction without muco-viscidosis. The search for a ciliary anomaly was motivated either by the coexistence of situs inversus (group I: 12 cases) or by the negative or scanty aetiological history (group II: 27 cases). The investigation of the cilia consists of a study of ciliary motility and an ultrastructural study of biopsy specimens using the electron microscope. Groups I and II were similar as regards severity of disease, assessed by the incidence of bronchiectasis and chronic hypoxia at rest; a higher incidence of neonatal respiratory distress was noted in group I (7 cases against 4 in group II). Ciliary immotility was particularly noted in group I (7 cases) and one case of weak motility in group II. The major ultrastructural anomalies of the axonemal complex were seen as well in both groups I and II; it was noted that all the structures may be totally or partially missing with the exception of the peripheral microtubules. The discussion centred on three points: 1. In the first analysis, the discordance between the existance of major ultrastructural anomalies of the axonemal complex, a priori incompatible with the conservation of ciliary motility on light microscopy; the link seems to lie in the percentage of cilia affected, the existence of ciliary dyskinesia and finally the disorientation of the basal corpuscles. 2. The immunologic abnormalities sometimes associated with a syndrome of ciliary immotility: that is the anomalies of leucokyte migration under the control of microtubular structures in the peri-centriolar region. 3. The practical consequences. Current treatment rests on daily respiratory physiotherapy and antibiotics adapted for cases of superinfection; also there are drugs stimulating ciliary activity perhaps by the effect on their residual motility; some substances carrying ATP and ATP-ase may re-establish ciliary motility anulled by the absence of dynein arms; also the observations of major ultrastructural abnormalities ought to lead to better genetic counselling than at present, where the mode or modes of transmission are uncertain.