SciELO - Scientific Electronic Library Online

 
vol.127 issue8Horizontal gastroplasty with Roux en Y anastomosis in morbidly obese patients. Preliminary resultsAssociation of Down syndrome and hyperthyroidism.: Report of three cases author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand

Journal

Article

Indicators

Related links

Share


Revista médica de Chile

Print version ISSN 0034-9887

Abstract

MOYA D, Guisela et al. Primary ciliary dyskinesia. : A clinical series. Rev. méd. Chile [online]. 1999, vol.127, n.8, pp.961-966. ISSN 0034-9887.  http://dx.doi.org/10.4067/S0034-98871999000800010.

Background: Primary ciliary dyskinesia is characterized by a congenital alteration of the ciliary ultrastructure and function. As a consequence, their respiratory tract sweeping action is lost and recurrent respiratory infections ensue. Aim: To analyze a clinical series of patients with primary ciliary dyskinesia, their clinical and laboratory features. Patients and methods: A retrospective review of patients with primary ciliary dyskinesia seen a University Hospital, between 1994 and 1998. Bronchial biopsies were obtained with 3.6 mm diameter Olympus fibrobronchoscope, using a cayman type forceps. Ultrastructural alterations of respiratory tract ciliated cells were recorded. Results: Six patients (four male) aged 9 months to 13 years old were reviewed. Three patients had situs inversus. All had repeated bouts of obstructive bronchitis and pneumonia, five had sinusitis, four atelectasis, three recurrent otitis and three had bronchiectasis. Cystic fibrosis and immunological alterations were ruled out in five children. Ultrastructural analysis revealed absence of dynein arms in three cases, absence of the internal dynein arm in one, additional peripheral microtubules and absence of dynein arms in one case. Conclusions: Primary ciliary dyskinesia must be considered in the differential diagnosis of recurrent respiratory infections. Ultrastructural analysis of ciliary structure can be done in bronchial biopsies obtained through bronchoscopy.

Keywords : Bronchial diseases; Bronchoscopy; Ciliary motility disorders.

        · text in Spanish

 

Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License