Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento

Background: Ciliary dyskinesia (CD) is a low incidence genetic illness, that presents with a wide clinical spectrum. Also, there are transitory conditions that present with ciliary anomalies, secondary to infectious diseases of the airways. Aim: To descube clinical and ultrastructural findings and c...

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Autores principales:	Iñiguez C,Rodrigo, Fonseca A,Ximena, Hernández C,Jury, González B,Sergio, Sánchez D,Ignacio
Lenguaje:	Spanish / Castilian
Publicado:	Sociedad Médica de Santiago 2007
Materias:	Ciliary motility disorders Otitis Rhinitis Sinusitis
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007000900008
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spelling	oai:scielo:S0034-988720070009000082008-05-30Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamientoIñiguez C,RodrigoFonseca A,XimenaHernández C,JuryGonzález B,SergioSánchez D,Ignacio Ciliary motility disorders Otitis Rhinitis Sinusitis Background: Ciliary dyskinesia (CD) is a low incidence genetic illness, that presents with a wide clinical spectrum. Also, there are transitory conditions that present with ciliary anomalies, secondary to infectious diseases of the airways. Aim: To descube clinical and ultrastructural findings and clinical and therapeutic evolution of these patients. Patients and Methods: Retrospective review of medical records and electron microscopy findings of 33 patients (aged 1 to 21 years, 14 females) with ultrastructural diagnosis of CD. To obtain follow up information, a telephone survey was done. Results: In 30 patients (90%) the inner dynein arm (IDA) was absent in 50 or more percent of the cilia. Twenty two (66%) had absence of the outer dynein arm. Before diagnosis of CD, 19 patients (57%) presented recurrent otitis media, 25 patients (77%), three or more episodes of rhinosinusitis and 18 patients (56%) had recurrent pneumonia. Middle ear ventilation tubes were placed in 19 patients (57%), and during its use, 12 (68%) remained without othorrea. Sixteen patients (48%) with recurrent episodes of rhinosinusitis required adenoidectomy Seven (21%) required a functional endoscopic sinus surgery (FESS), and 6 (86%) improved after FESS. Conclusions: Our patients with CD presented recurrent infections in different airway locations. In those with a diagnosis of CD and recurrent otológica! and rhinosinusal infections, IDA was absent in a high percentage of cilia. FESS and the use of ventilation tubes may have a beneficial role in a subgroup of patients with CDinfo:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.135 n.9 20072007-09-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007000900008es10.4067/S0034-98872007000900008
institution	Scielo Chile
collection	Scielo Chile
language	Spanish / Castilian
topic	Ciliary motility disorders Otitis Rhinitis Sinusitis
spellingShingle	Ciliary motility disorders Otitis Rhinitis Sinusitis Iñiguez C,Rodrigo Fonseca A,Ximena Hernández C,Jury González B,Sergio Sánchez D,Ignacio Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento
description	Background: Ciliary dyskinesia (CD) is a low incidence genetic illness, that presents with a wide clinical spectrum. Also, there are transitory conditions that present with ciliary anomalies, secondary to infectious diseases of the airways. Aim: To descube clinical and ultrastructural findings and clinical and therapeutic evolution of these patients. Patients and Methods: Retrospective review of medical records and electron microscopy findings of 33 patients (aged 1 to 21 years, 14 females) with ultrastructural diagnosis of CD. To obtain follow up information, a telephone survey was done. Results: In 30 patients (90%) the inner dynein arm (IDA) was absent in 50 or more percent of the cilia. Twenty two (66%) had absence of the outer dynein arm. Before diagnosis of CD, 19 patients (57%) presented recurrent otitis media, 25 patients (77%), three or more episodes of rhinosinusitis and 18 patients (56%) had recurrent pneumonia. Middle ear ventilation tubes were placed in 19 patients (57%), and during its use, 12 (68%) remained without othorrea. Sixteen patients (48%) with recurrent episodes of rhinosinusitis required adenoidectomy Seven (21%) required a functional endoscopic sinus surgery (FESS), and 6 (86%) improved after FESS. Conclusions: Our patients with CD presented recurrent infections in different airway locations. In those with a diagnosis of CD and recurrent otológica! and rhinosinusal infections, IDA was absent in a high percentage of cilia. FESS and the use of ventilation tubes may have a beneficial role in a subgroup of patients with CD
author	Iñiguez C,Rodrigo Fonseca A,Ximena Hernández C,Jury González B,Sergio Sánchez D,Ignacio
author_facet	Iñiguez C,Rodrigo Fonseca A,Ximena Hernández C,Jury González B,Sergio Sánchez D,Ignacio
author_sort	Iñiguez C,Rodrigo
title	Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento
title_short	Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento
title_full	Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento
title_fullStr	Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento
title_full_unstemmed	Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento
title_sort	disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento
publisher	Sociedad Médica de Santiago
publishDate	2007
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872007000900008
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Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento

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