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Ultrastructural ciliary defects in children with recurrent infections of the lower respiratory tract
Author(s) -
Barlocco Ezio G.,
Valletta Enrico A.,
Canciani Mario,
Lungarella Giuseppe,
Gardi Concetta,
Margherita De Santi M.,
Mastella Gianni
Publication year - 1991
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/ppul.1950100104
Subject(s) - primary ciliary dyskinesia , medicine , bronchiectasis , situs inversus , respiratory tract , sinusitis , chronic sinusitis , respiratory system , respiratory disease , cilium , kartagener syndrome , respiratory tract infections , pathology , aplasia , otitis , lung , surgery , biology , microbiology and biotechnology
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. Pediatr Pulmonol 1991; 10:11–17.