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A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: A tertiary care experience.

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% (n = 111) of patients and 19.8% (n = 37) of patients had a positive family history for non-CF bronchiectasis. Common causes were PCD in 51.3% (n = 96), immunodeficiency in 15% (n = 28), history of tuberculosis in 5.9% (n = 11), post-infectious complication in 3.2% (n = 6) and other anomalies in 2.1% (n = 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.

journal_name

Respir Med

journal_title

Respiratory medicine

authors

Satırer O,Mete Yesil A,Emiralioglu N,Tugcu GD,Yalcın E,Dogru D,Kiper N,Ozcelik U

doi

10.1016/j.rmed.2018.02.013

subject

Has Abstract

pub_date

2018-04-01 00:00:00

pages

35-39

eissn

0954-6111

issn

1532-3064

pii

S0954-6111(18)30042-8

journal_volume

137

pub_type

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A review of the etiology and clinical presentation of non-cystic fibrosis bronchiectasis: A tertiary care experience.