Adult bronchiectasis as diagnosis of hereditary ciliary dyskinesia

Citation
E. Hazouard et al., Adult bronchiectasis as diagnosis of hereditary ciliary dyskinesia, REV MAL RES, 16(5), 1999, pp. 839-841
Citations number
18
Categorie Soggetti
Cardiovascular & Respiratory Systems
Journal title
REVUE DES MALADIES RESPIRATOIRES
ISSN journal
07618425 → ACNP
Volume
16
Issue
5
Year of publication
1999
Pages
839 - 841
Database
ISI
SICI code
0761-8425(199911)16:5<839:ABADOH>2.0.ZU;2-H
Abstract
We report a case of bronchiectasis in a 26-year-old man associated with the following congenital abnormalities: deafness, purulent bronchorrhea, nasal polyps, dysmorphic physical pattern and chronic sinusitis. Situs inversus was absent, A sampling was performed on the posterior nasal mucous membrane and displayed structural ciliary abnormality: a deficiency of the intern d ynein-arm. The patient's bother was affected find had similar features: con genital bronchiectasis, deafness, mental deficiency and sinusitis. Young's syndrome was relevant in this case, Hereditary ciliary dyskinesia should be considered in adults with bronchiectasis together with rhinologic and alim entary canal disorders. Nasal biopsies are safe and allow cilia examination .