Disquinesia ciliar primaria: experiencia en 6 pacientes / Primary ciliary dyskinesia: a experience in 6 patients
Rev. méd. Chile
; 127(8): 961-6, ago. 1999. ilus, tab
Article
em Es
| LILACS
| ID: lil-253163
Biblioteca responsável:
CL1.1
RESUMO
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 andmethods:
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
Texto completo:
1
Coleções:
01-internacional
Base de dados:
LILACS
Assunto principal:
Broncoscopia
/
Transtornos da Motilidade Ciliar
Tipo de estudo:
Diagnostic_studies
/
Etiology_studies
Limite:
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
Idioma:
Es
Revista:
Rev. méd. Chile
Assunto da revista:
MEDICINA
Ano de publicação:
1999
Tipo de documento:
Article
País de publicação:
Chile