CYSTIC-FIBROSIS - EFFECTS OF SERUM FACTORS ON MUCUS SECRETION

  • 1 January 1976
    • journal article
    • research article
    • Vol. 35  (6) , 588-595
Abstract
The effects of serum from children with cystic fibrosis and from normal children on the mucus-secreting, ciliated epithelium were investigated in vitro using explanted tissue from rabbit lung. By optical and scanning electron microscopy, a sequence of structural changes is observed after incubation with cystic fibrosis serum; this sequence does not occur with normal serum. The earliest changes involve swelling of the goblet cells, with subsequent discharge of mucus onto the epithelial surface. This is followed by disruption of the normally rapid and synchronized ciliary activity. Mucus gradually extends over the surface entangling cilia. Finally, some shedding of ciliated cells occurs from the epithelium. Factors in cystic fibrosis serum apparently cause discharge of mucus leading to a disturbance of the normal ciliary activity in the rabbit lung. Such changes may result in dysfunction of the mucociliary clearance mechanism, and this dysfunction may be a contributory factor to the pathogenesis of lung disease.

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