Table 3.

Integration of clinical and laboratory information for patients with classical CF (N = 300), CFTR-RD (N = 19), and CRMS (N = 57)

Disease type	Symptoms	Sweat chloridea	IRT	CFTR mutation class	Pancreatic status	FTT	Colonizationb
CF	+	Majority >60 mmol/l	↑	I–IIIc	>80% insufficient	>25%	>80% PA > 20% MRSA
CFTR-RD	+	Majority <60 mmol/l	↑	IV–V	Sufficient	~20%	<35% PA 0% MRSA
CRMS	−	All <60 mmol/l	↑	IV–V	Sufficient	~2%	<40% PA < 5% MRSA

CF, cystic fibrosis; CFTR-RD, cystic fibrosis transmembrane regulator-related disease; CRMS, cystic fibrosis transmembrane regulator-related metabolic syndrome; FTT, failure to thrive; IRT, immunoreactive trypsinogen; MRSA, methicillin-resistant Staphylococcus aureus; PA, Pseudomonas aeruginosa.

^aSweat chloride measurements are report.

^bMicrobiological growth on oropharyngeal, sputum, and/or bronchoalveolar lavage specimens from the time poinst closest to diagnosis.

^cExceptions noted in manuscript text.