Table 3

Evaluation and follow-up investigations of CRMS/CFSPID infants

Initial assessment6 months12 months2 years3 years4 years5 years6 years
Sweat test+++/-++/-+/-+/-+
Extended CFTR gene analysis++/-
Check https://cftr2.org++++++++
Stool for faecal elastase++/-++/-+/-+/-+/-+/-
Respiratory assessment++++++++
Abdominal assessment++++++++
Weight, height/length, BMI++++++++
Respiratory culture+/-+/-+/-+/-+/-+/-+/-+/-
Chest imaging+/-+/-+/-+/-+/-+/-+/-+
LCI+
Spirometry+

+: undertake at this visit; +/-: consider only if clinically indicated. BMI: body mass index; LCI: lung clearance index. Reproduced and modified from [3] with permission.