A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: A tertiary care experience.
Respir Med
; 137: 35-39, 2018 04.
Article
em En
| MEDLINE
| ID: mdl-29605210
ABSTRACT
INTRODUCTION:
Non-cystic fibrosis(CF) bronchiectasis has been recognized in children for the past 200 years. Early childhood pneumonia and underlying conditions such as immunodeficiency, primary ciliary dyskinesia(PCD), and congenital lung pathology should be considered in the etiology. The aim of our study was to describe the clinical characteristics, laboratory, and radiological findings of a large population of patients with non-CF bronchiectasis at a tertiary center.METHODS:
We analyzed the clinical findings of 187 patients diagnosed with non-CF bronchiectasis over a period of 10 years (January 2005-December 2015) at the Hacettepe University Faculty of Medicine Department of Pediatric Pulmonology.RESULTS:
The median age at the time of diagnosis of non-CF bronchiectasis was 8 years (1-18 years). Consanguinity was positive in 59.4% (nâ¯=â¯111) of patients and 19.8% (nâ¯=â¯37) of patients had a positive family history for non-CF bronchiectasis. Common causes were PCD in 51.3% (nâ¯=â¯96), immunodeficiency in 15% (nâ¯=â¯28), history of tuberculosis in 5.9% (nâ¯=â¯11), post-infectious complication in 3.2% (nâ¯=â¯6) and other anomalies in 2.1% (nâ¯=â¯4) of patients. The frequency of pulmonary lobe involvement was as follows 71.1% left-lower lobe, 59.4% right lower lobe, 54% right-middle lobe, 26.8% left lingula, 13.9% right upper lobe, and 9.6% left upper lobe.CONCLUSIONS:
Diagnosis of non-CF bronchiectasis is often delayed because of a failure to recognize the significance of symptoms. Through clinical investigation, including a HRCT scan of the chest, sweat test, studies of immune function, and ciliary function in a child with a prolonged suppurative cough, remains important. In Turkey, the most common causes of non-CF bronchiectasis are PCD and immunodeficiency, related to a high frequency of consanguinity.Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Bronquiectasia
/
Transtornos da Motilidade Ciliar
/
Tosse
/
Agamaglobulinemia
/
Doenças Genéticas Ligadas ao Cromossomo X
/
Pneumopatias
Tipo de estudo:
Etiology_studies
/
Observational_studies
/
Risk_factors_studies
Limite:
Adolescent
/
Adult
/
Child
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Child, preschool
/
Female
/
Humans
/
Male
País/Região como assunto:
Asia
Idioma:
En
Ano de publicação:
2018
Tipo de documento:
Article