Williams—Campbell’s syndrome in children

Abstract

Results of follow-up in 27 patients with Williams—Campbell’s syndrome was present in the article. It was shown, that disease defining anatomical defect leads in children to formation of chronic obstructive bronchopulmonary process with suppurative endobronchitis, diffuse located sacculative bronchiectasia, and pneumosclerosis. The clinical symptoms manifestate in 96.3% of patients during first three years after birth and are characterised by intermittent cases of pneumonia and bronchoobstructive syndrome.

During Williams—Campbell’s syndrome course, diffuse suppurative bronchitis or bronchial suppurative catarrh with chronic course, as well as great number of tracheobronchial tree abnormalities, are character for endoscopic view. The coupling of anamnestic data, clinical signs, and typical changes in bronchogramms and spirogramms allow to diagnose lighter Williams—Campbell’s syndrome. The disease prognosis is serious. It is defined directly by extent of bronchopulmonary process and depends on pulmonary hypertension and cor pulmonale developing.