Table 1. Patient characteristics.

Patient characteristics of a full cohort of 23 patients. For numerical data, the median and range are reported. For categorical data, the number of patients, with the percentage of the full cohort, are reported. Age, gender, pancreatic status, mutation, culture results, antibiotics at the time of bronchoscopy, and pathology cell counts are acquired retrospectively from medical records of the patients. PRAGMA-CF %Dis and %Bx are measured quantitatively using the PRAGMA-CF scoring methods. CFTR mutations and individual data are listed in Table S1. PRAGMA-CF scores and pathology cell counts do not differ significantly when grouped based on gender, mutation, pancreas status, culture results, or antibiotic treatment. *“PRAGMA-CF after two years” contained 21 patients. Two patients did not receive a chest CT scan.

Patient characteristics n=23
Age		1 year	5	22%
		3 year	18	78%
Gender		Male	8	34.78%
		Female	15	65.22%
Pancreas status		Insufficient	19	82.61%
		Sufficient	4	17.39%
Mutation		F508Del homozygote	12	52.17%
		F508Del heterozygote	10	43.48%
		Other mutation	1	4.35%
Culture result	Bacterial	Positive	8	34.78%
		Negative	15	65.22%
	Fungi	Positive	1	4.35%
		Negative	22	95.65%
Antibiotics at time of bronchoscopy		Yes	21	91.30%
		No	2	8.70%
Pulmozyme at time of bronchoscopy		Yes	7	30.40%
		No	16	69.60%
PRAGMA-CF		%Disease score	1.75%	(0.16–5.39)
		%Bronchiectasis	0.07%	(0–0.62)
PRAGMA-CF after two years*		%Disease score	1.27%	(0–9.09)
		%Bronchiectasis	0.43%	(0–2.77)
Pathology cell count		%Neutrophils	24%	(4–71)
		%Macrophages	62%	(26–91)
		%Lymphocytes	5%	(2–20)
		%Eosinophils	0%	(0–3)