Table II.
Characteristics | CF (n = 29) |
Controls∗ (n = 18) | ||
---|---|---|---|---|
Entry (n = 28) | 2-wk visit (n = 11) | 12-wk visit (n = 26) | ||
Age, y | 6.9 (2.8-10.9) | 3.2 (2.4-9.3) | 9.3 (3.0-11.7) | 13.3 (11.3-15.9) |
Male/female | 18 (64)/10 (36) | 7 (64)/4 (36) | 17 (65)/9 (35) | 5 (28)/13 (72) |
Distance from CF center,† km | 111 (53-217) | 53 (51-63) | 76 (53-212) | |
Modulator(s) | 19 (68) | 7 (64) | 18 (69) | |
Elexacaftor/tezacaftor/ivacaftor | 11 (39) | 3 (27) | 12 (46) | |
Ivacaftor | 3 (11) | 2 (18) | 3 (12) | |
Lumacaftor/ivacaftor | 1 (4) | 1 (9) | 2 (8) | |
Tezacaftor/ivacaftor | 1 (4) | 1 (9) | 1 (4) | |
Pancreatic insufficiency‡ | 25 (89) | 9 (82) | 23 (88) | |
Proton-pump inhibitor | 20 (71) | 9 (82) | 17 (65) | |
Long-term oral azithromycin | 8 (29) | 1 (9) | 8 (31) | |
Weight-for-age z score§ | 0.12 (−0.45 to 0.67) | 0.48 (0.12-1.35) | 0.24 (−0.51 to 0.76) | 0.255 (−0.6 to 0.69) |
Height-for-age z score§ | 0.01 (−0.65 to 0.47) | 0.42 (−0.12 to 0.53) | −0.01 (−0.30 to 0.37) | −0.48 (−1.00 to 0.32) |
Weight-for-length/height z score§ | 0.59 (0.09-0.86) | 0.73 (0.30-0.88) | 0.23 (−0.12 to 0.8) | n/a |
BMI z score§ | 0.20 (−0.09 to 0.97) | 1.05 (0.76-1.61) | 0.38 (−0.47 to 1.33) | 0.04 (−0.73 to 0.82) |
IV antibiotics in past 3 mo | 0 (0) | 0 (0) | 1 (4) | |
Oral antibiotics in past week | 4 (14) | 1 (9) | 1 (4) | |
Diagnosis of CF liver disease | 4 (14) | 2 (18) | 4 (15) | |
Abdominal pain in past month | ||||
None | 13 (50) | 6 (55) | 14 (70) | |
Once a week | 8 (31) | 3 (27) | 2 (10) | |
2-3 times per week | 4 (15) | 1 (9) | 4 (20) | |
4-5 times per week | 1 (4) | 1 (9) | 0 (0) | |
Stool provided | 25 (89) | 10 (91) | 22 (85) | 12 (75) |
Blood provided | 28 (100) | 10 (91) | 22 (85) | 16 (89) |
IV, intravenous; n/a, not applicable.
Values expressed as median (IQR) or n (percent).
Calculated as shortest driving distance from street address of primary residence to St Louis Children’s Hospital in Google Maps in May 2023.
Pancreatic insufficiency defined as those who have low concentrations of fecal elastase, 2 severe mutations, or who are prescribed pancreatic enzyme replacements.
As published in Kuczmarski et al.23