1 Introduction Newborn screening (NBS) for Cystic Fibrosis (CF) has improved outcomes but introduces new diagnostic conundrums [1, 2]. The designation Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) -related metabolic syndrome (CRMS), known in the UK and Europe as CF Screen Positive, Inconclusive Diagnosis (CFSPID) is used for infants with a positive NBS for whom follow-up tests neither confirm, nor rule out, a CF diagnosis.