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Table 1 When and how rule out CF according to existing bronchiectasis guidelines

From: When and how ruling out cystic fibrosis in adult patients with bronchiectasis

Guidelines

When

How

ERS 2017 [14]

Selected adults including those with any of the following: young adults; upper lobe predominance of bronchiectasis on chest CT; the presence of nasal polyposis and/or chronic rhinosinusitis; recurrent pancreatitis; male primary infertility and/or malabsorption

Sweat chloride, other biomarkers of CFTR activity and CFTR mutation analysis

Pulmonology Portoguese Society, 2016 [16]

All children and selected adults (no further specified)

Two measurements of sweat chloride and CFTR mutation analysis for

British Thoracic Society, 2010 [22]

All children and selected adults including those with any of the following: adults up to the age of 40; age at presentation > 40 years and no other identified cause; persistent isolation of Staphylococcus aureus in the sputum; features of malabsorption; male primary infertility; upper lobe bronchiectasis; a history of childhood steatorrhoea.

Two measurements of sweat chloride and CFTR mutation analysis

Normativa SEPAR, 2008 [23]

Selected adults

Sweat chloride, nasal potential difference, CFTR mutation analysis test

Thoracic Society of Australia and New Zealand, 2015 [24]

All children and selected adults (no further specified)

Sweat test