Ultrastructural ciliary defects in children with recurrent infections of the lower respiratory tract.

Abstract:

:One hundred fifty-four children with recurrent or chronic infections of the lower respiratory tract compatible with the diagnosis of primary ciliary dyskinesia (PCD) were evaluated for the presence of ultrastructural ciliary abnormalities. Studies were performed on multiple samples of respiratory mucosa obtained by nasal and bronchial brushing. Twenty-eight children showed ultrastructural ciliary defects compatible with the diagnosis of PCD: Twenty-four presented dynein arm deficiency (either as isolated defect or in association with microtubular abnormalities), two had ciliary aplasia, and two showed microtubular abnormalities. Eleven patients with PCD had situs viscerum inversus, bronchiectasis, and chronic sinusitis (Kartagener's syndrome); one child with Kartagener's syndrome had normal ciliary structure. The appearance of respiratory symptoms within the first month of life, the colonization by Haemophilus influenzae, and a history of recurrent rhinitis and otitis were characteristically present in children with PCD. The clinical status of those patients who reached adolescence was, in our experience, remarkably good. An early diagnosis with adequate prevention and therapy of respiratory infections may have an important role in minimizing irreversible lung damage.

journal_name

Pediatr Pulmonol

journal_title

Pediatric pulmonology

authors

Barlocco EG,Valletta EA,Canciani M,Lungarella G,Gardi C,De Santi MM,Mastella G

doi

10.1002/ppul.1950100104

subject

Has Abstract

pub_date

1991-01-01 00:00:00

pages

11-7

issue

1

eissn

8755-6863

issn

1099-0496

journal_volume

10

pub_type

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