Disquinesia ciliar primaria.: Experiencia en 6 pacientes

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 cili...

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Autores principales:	Moya D,Guisela, Caussade L,Solange, González B,Sergio, Navarro M,Héctor, Sánchez D,Ignacio
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 1999
Materias:	Bronchial diseases Bronchoscopy Ciliary motility disorders
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98871999000800010
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spelling	oai:scielo:S0034-988719990008000102005-11-14Disquinesia ciliar primaria.: Experiencia en 6 pacientesMoya D,GuiselaCaussade L,SolangeGonzález B,SergioNavarro M,HéctorSánchez D,Ignacio Bronchial diseases Bronchoscopy Ciliary motility disorders 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.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.127 n.8 19991999-08-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98871999000800010es10.4067/S0034-98871999000800010
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Bronchial diseases Bronchoscopy Ciliary motility disorders
spellingShingle	Bronchial diseases Bronchoscopy Ciliary motility disorders Moya D,Guisela Caussade L,Solange González B,Sergio Navarro M,Héctor Sánchez D,Ignacio Disquinesia ciliar primaria.: Experiencia en 6 pacientes
description	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.
author	Moya D,Guisela Caussade L,Solange González B,Sergio Navarro M,Héctor Sánchez D,Ignacio
author_facet	Moya D,Guisela Caussade L,Solange González B,Sergio Navarro M,Héctor Sánchez D,Ignacio
author_sort	Moya D,Guisela
title	Disquinesia ciliar primaria.: Experiencia en 6 pacientes
title_short	Disquinesia ciliar primaria.: Experiencia en 6 pacientes
title_full	Disquinesia ciliar primaria.: Experiencia en 6 pacientes
title_fullStr	Disquinesia ciliar primaria.: Experiencia en 6 pacientes
title_full_unstemmed	Disquinesia ciliar primaria.: Experiencia en 6 pacientes
title_sort	disquinesia ciliar primaria.: experiencia en 6 pacientes
publisher	Sociedad Médica de Santiago
publishDate	1999
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98871999000800010
work_keys_str_mv	AT moyadguisela disquinesiaciliarprimariaexperienciaen6pacientes AT caussadelsolange disquinesiaciliarprimariaexperienciaen6pacientes AT gonzalezbsergio disquinesiaciliarprimariaexperienciaen6pacientes AT navarromhector disquinesiaciliarprimariaexperienciaen6pacientes AT sanchezdignacio disquinesiaciliarprimariaexperienciaen6pacientes
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Disquinesia ciliar primaria.: Experiencia en 6 pacientes

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