TABLE 1.
Characteristics of the cohort according to the presence of cystic fibosis-related liver disease (CFLD)
| Characteristic | All patients (n=127) | With CFLD (n=18) | Without CFLD (n=109) | P |
|---|---|---|---|---|
| Demographics | ||||
| Age, years | 27 (22–37) | 28 (18–32) | 27 (22–37) | 0.22 |
| Male sex | 60 (47) | 10 (56) | 50 (42) | 0.46 |
| Body mass index†, kg/m2 | 21 (19–23) | 21 (19–23) | 21 (19–23) | 0.96 |
| CF-related variables | ||||
| Diabetes mellitus | 24 (19) | 4 (22) | 20 (17) | 0.75 |
| Pancreatic insufficiency | 107 (84) | 17 (94) | 90 (76) | 0.31 |
| Lung transplantation | 15 (12) | 3 (17) | 12 (10) | 0.45 |
| Ursodeoxycholic acid use | 32 (25) | 15 (83) | 17 (14) | <0.00005 |
| CFTR-genotype ΔF508 mutation | 114 (90) | 16 (89) | 98 (90) | 1.00 |
| CFTR-genotype ΔF508 homozygous | 67 (53) | 12 (67) | 55 (51) | 0.31 |
| Spirometry | ||||
| FEV1, L | 2.55 (1.71–3.28) | 2.98 (1.86–3.26) | 2.46 (1.70–3.28) | 0.42 |
| FEV1, % predicted (%) | 70 (52.5–88) | 74 (58–80) | 69 (50–89) | 0.92 |
| FVC, L | 3.86 (2.97–4.73) | 4.06 (3.01–4.58) | 3.82 (3.02–4.75) | 0.85 |
| FVC, % predicted (%) | 92 (78–105) | 95 (77–103) | 92 (78–106) | 0.74 |
| FEV1/FVC (%) | 73 (64–84) | 74 (73–85) | 73 (62–84) | 0.28 |
| Biochemistry | ||||
| Albumin†, g/L | 38 (35–41) | 38 (36–40) | 39 (35–41) | 0.48 |
| Total bilirubin†, μmol/L | 6 (4–8) | 8 (6–10) | 6 (4–8) | 0.03 |
| Alanine aminotransferase (ALT)†, U/L | 21 (17–29) | 31 (24–48) | 21 (16–27) | 0.001 |
| Aspartate aminotransferase (AST)†, U/L | 23 (19–28) | 28 (22–45) | 22 (19–26) | 0.004 |
| Gamma-glutamyl transferase (GGT)†, U/L | 18 (13–28) | 41 (26–68) | 17 (13–25) | 0.0001 |
| Alkaline phosphatase (ALP)†, U/L | 94 (78–123) | 157 (125–205) | 89 (77–108) | <0.00005 |
| Platelets, ×109/L | 272 (224–332) | 218 (112–332) | 274 (236–330) | 0.053 |
| International normalized ratio† | 1.0 (1.0–1.1) | 1.1 (1.0–1.1) | 1.0 (1.0–1.1) | 0.01 |
| Creatinine, μmol/L† | 66 (55–77) | 60 (52–70) | 67 (56–77) | 0.23 |
| Glucose, mmol/L‡ | 4.9 (4.3–6.6) | 4.9 (4.4–6.9) | 4.9 (4.2–6.4) | 0.74 |
Data presented as n (%) or median (interquartile range).
†
Data missing for five patients (four without CFLD and one with CFLD).
‡
Albumin, total bilirubin, ALT, AST, GGT, ALP, international normalized ratio and creatinine missing in 1, 1, 1, 5, 1, 1, 4 and 1 patients (all in the group without CFLD), respectively;
‡
Glucose data missing in 11 patients (one with CFLD). FEV1 Forced expiratory volume in 1 s; FVC Forced vital capacity