Management of primary ciliary dyskinesia: the lower airways.
Paediatr Respir Rev
; 10(2): 55-7, 2009 Jun.
Article
en En
| MEDLINE
| ID: mdl-19410202
ABSTRACT
Primary ciliary dyskinesia (PCD) is a genetic disease associated with defective ciliary structure and function. Chronic oto-sino-pulmonary infection is the most common clinical presentation. Patients should be monitored in centres with expertise in PCD. Regular respiratory monitoring consists of lung function tests, adapted to the patient's age, and cough swab or sputum cultures. Chest X-rays are insensitive but the use of high-resolution computed tomography should be considered carefully to avoid excessive radiation. Treatment of the chronic suppuration of the lower airways relies on antibiotics to which the isolated bacteria are sensitive, together with respiratory physiotherapy and exercise to clear excessive bronchial secretions. Bronchodilators and anti-inflammatory agents have no proven benefit and should be prescribed, if at all, on an individual basis. Treatment of the lower airways should always be in conjunction with that of the upper airways. Importantly in PCD, respiratory disease may be controlled with the use of early, adequate and aggressive management.
Texto completo:
1
Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Infecciones del Sistema Respiratorio
/
Síndrome de Kartagener
/
Modalidades de Fisioterapia
/
Antibacterianos
Tipo de estudio:
Diagnostic_studies
/
Etiology_studies
Límite:
Humans
Idioma:
En
Revista:
Paediatr Respir Rev
Asunto de la revista:
PEDIATRIA
Año:
2009
Tipo del documento:
Article
País de afiliación:
Francia