HIRT-Nl | HIRT-CF | ||
NPD diagnosis score | >0.27 | ≤0.27 | p Value |
No. of patients | 8 | 13 | |
At birth | |||
IRT (day 3) | 105 (25) | 81 (69) | 0.43 |
Birth weight (g) | 2970 (1357) | 3380 (517) | 0.14 |
At evaluation | |||
Age (years) | 1.7 (2.9) | 2 (1.8) | 0.56 |
Sweat Cl– (mmol/l) | 46(17) | 33 (9) | 0.03 |
Recurrent lower tract infection* | 3/8 | 8/13 | 0.38 |
Transient isolation of S. aureus | 1/8 | 4/13 | 0.6 |
BMI Z score | −1.6 (1.5) | −1 (2.3) | 0.06 |
At follow-up | |||
Age (years) | 3.4 (3) | 4.1 (1.4) | 0.88 |
Sweat test >60 mmol/l | 0/5 | 6/9 | 0.03 |
Sweat Cl– (mmol/l) | 18 (14.5); n=5 | 64 (36); n=9 | 0.004 |
Recurrent lower tract infection* | 0/8 | 8/13 | 0.007 |
Chronic P aeruginosa | 0/8 | 1/13 | 0.9 |
Chronic S aureus | 0/8 | 3/13 | 0.25 |
Transient isolation of P aeruginosa | 0/8 | 2/13 | 0.5 |
Transient isolation of S aureus | 3/8 | 4/13 | 0.9 |
Airway obstruction | 1/8 | 4/13 | 0.6 |
Bronchiectasis | 0/8 | 3/13 | 0.25 |
BMI Z-score | −1.2 (1.6) | 0 (2) | 0.04 |
Pancreatic insufficiency | 0/8 | 1/13 | 0.99 |
Alternative diagnosis | 6/8 | 0/13 | 0.0005 |
Results are presented as median (IQR) or number of observations. Comparison with Fisher exact test for qualitative variables and Mann–Whitney test for quantitative variables.
Patients with HIRT-Nl have a diagnostic score >0.27, patients with HIRT-CF have a diagnostic score <0.27.
↵* At least four episodes per week requiring modification of basal treatment.
BMI, body mass index; CF, cystic fibrosis; IRT, immunoreactive trypsinogen; NPD, nasal potential difference.