[Clinical and ultrastructural features of ciliary dyskinesia]. / Disquinesia ciliar: diagnóstico ultraestructural, evolución clínica y alternativas de tratamiento.
Rev Med Chil
; 135(9): 1147-52, 2007 Sep.
Article
em Es
| MEDLINE
| ID: mdl-18064369
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.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Transtornos da Motilidade Ciliar
Tipo de estudo:
Diagnostic_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Adolescent
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Adult
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Child
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Child, preschool
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Female
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Humans
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Infant
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Male
Idioma:
Es
Revista:
Rev Med Chil
Ano de publicação:
2007
Tipo de documento:
Article
País de afiliação:
Chile
País de publicação:
Chile