Table 2.
Summary of data stratified by presence of steatosis, use of modulator (lumacaftor/ivacaftor), and diabetes status
| Normal Range | All subjects (n = 20) | Steatosis (n = 6) | No Steatosis (n = 14) | P value | CFTR modulator (n = 9) | No CFTR modulator (n = 11) | P value | CFRD (n = 12) | NGT (n = 8) | P value | |
| Hepatic Fat Fraction | -- | 3.0 | 9.5 | 2.4 | 0.01 | 2.0 | 4.1 | 0.002 | 2.2 | 4.9 | 0.06 |
| 0.0-21.0 | 6.0-21.0 | 0-4.1 | 0.0-6.4 | 2.7-21 | 0.0-14.5 | 2.4-21 | |||||
| Age | -- | 22.3 | 16.7 | 26.0 | 0.08 | 26.4 | 21.9 | 0.29 | 28.2 | 18.0 | 0.04 |
| 11.3-39.0 | 13.4-36.1 | 11.3-39.0 | 16.3-39.0 | 11.3-36.1 | 17.0-39.0 | 11.3-30.6 | |||||
| Sex (male) | -- | 16 | 5 | 11 | 1.00 | 7 | 9 | 1.00 | 8 | 8 | 0.12 |
| 80% | 83% | 78% | 78% | 82% | 67% | 100% | |||||
| BMI | -- | 21.0 | 20.5 | 21.0 | 0.6 | 22.4 18.8-25.7 | 20.0 16.9-32.4 | 0.05 | 21.0 18.8-32.4 | 19.6 16.9-22.4 | 0.05 |
| 16.9-32.4 | 16.9-32.4 | 18.8-25.7 | |||||||||
| BMI percentile | -- | 39 | 50 | 30 | 0.77 | 51 | 23 | 0.21 | 39 | 37.5 | 0.62 |
| 2-96 | 2-96 | 3-77 | 3-77 | 2-96 | 3-96 | 2-73 | |||||
| FEV1 % | -- | 77 | 86 | 74.5 | 0.22 | 73 | 77 | 0.24 | 63.5 | 88 | 0.001 |
| 33-105 | 62-105 | 33-97 | 33-89 | 48-105 | 33-105 | 65-97 | |||||
| CFTR modulator | -- | 9 | 1 | 8 | 0.16 | 9 | 11 | -- | 7 | 2 | 0.20 |
| 45% | 17% | 57% | 100% | 100% | 58% | 25% | |||||
| Hemoglobin A1C (%) | < 5.8 | 5.8 | 5.7 | 6.3 | 0.30 | 6.3 | 5.7 | 0.21 | 6.4 | 5.6 | 0.002 |
| 5.2-8.2 | 5.4-6.4 | 5.2-8.2 | 5.3-8.2 | 5.2-7.4 | 5.4-8.2 | 5.2-5.7 | |||||
| AST (U/L) | 9-39 | 23 | 27 | 23 | 0.93 | 20 | 27 | 0.39 | 20 | 26 | 0.87 |
| 11-45 | 11-45 | 11-42 | 11-42 | 11-45 | 11-42 | 11-45 | |||||
| ALT (U/L) | 10-52 | 22 | 24.5 | 22 | 0.57 | 18 | 29 | 0.19 | 18 | 26 | 0.46 |
| 10-58 | 12-58 | 10-45 | 10-45 | 12-58 | 10-58 | 14-47 | |||||
| GGT (U/L) | 5-64 | 14 | 19 | 13.5 | 0.10 | 12 | 19 | 0.07 | 13.5 | 19 | 0.30 |
| 9-29 | 14-25 | 9-29 | 9-24 | 10-29 | 9-29 | 11-24 | |||||
| Alk Phos (U/L) | 33-120 | 110 | 172 | 90 | 0.01 | 66 | 155 | 0.01 | 90 | 157 | 0.04 |
| 44-310 | 146-310 | 44-234 | 44-178 | 103-310 | 44-178 | 71-310 | |||||
| Alk Phos SD | -2-2 | 1.2 | 3.1 | -0.3 | 0.03 | -0.3 | 1.8 | 0.07 | 0.6 | 1.4 | 0.65 |
| -1.5-4.8 | -0.6-4.8 | -1.5-4.7 | -1.5-4.7 | -0.6-4.8 | -1.5-4.8 | -0.6-3.2 | |||||
| Total Bili (µmol/L) | 0-20.5 | 6.8 | 8.5 | 5.1 | 0.47 | 5.1 | 8.6 | 0.003 | 5.1 | 8.6 | 0.06 |
| 3.4-20.5 | 3.4-15.4 | 3.4-20.5 | 3.4-6.8 | 3.4-20.5 | 3.4-18.8 | 5.1-20.5 | |||||
| Triglyceride (mmol/L) | < 1.7 | 1.02 | 1.25 | 0.68 | 0.07 | 0.9 | 1.25 | 0.79 | 0.68 | 1.28 | 0.15 |
| 0.36-2.55 | 0.69-2.55 | 0.36-1.54 | 0.64-2.55 | 0.36-2.35 | 0.64-2.35 | 0.36-2.55 | |||||
| LDL (mmol/L) | < 3.4 | 1.7 | 1.4 | 1.7 | 0.05 | 1.6 | 1.7 | 0.53 | 1.7 | 1.6 | 0.71 |
| 0.8-2.4 | 0.9-1.6 | 0.8-2.4 | 0.8-2.2 | 0.9-2.4 | 0.9-2.4 | 0.8-2.0 | |||||
| HDL (mmol/L) | > 1.0 | 1.03 | 0.84 | 1.09 | 0.02 | 1.04 | 0.98 | 0.31 | 1.01 | 1.05 | 0.73 |
| 0.53-2.03 | 0.53-1.41 | 0.91-2.03 | 0.86-2.03 | 0.53-1.54 | 0.53-2.03 | 0.84-1.53 |
Data is stratified by steatosis (MRI proton density fat fraction >5%) or no steatosis (MRI proton density fat fraction <5%), use of CFTR modulator lumacaftor/ivacaftor, and diabetes status. Data are presented as median and range. To convert total bilirubin from μmol/L to mg/dL multiply by 0.0585.To convert triglycerides from mmol/L to mg/dL multiply by 88.5. To convert LDL from mmol/L to mg/dL multiply by 38.7. To convert HDL from mmol/L to mg/dL multiply by 38.7. CFRD: cystic fibrosis related diabetes. NGT: normal glucose tolerance; AST: Aspartate transaminase; ALT: Alanine transaminase; GGT: Gamma-glutamyltransferase; LDL: Low density lipoprotein; HDL: High density lipoprotein; BMI: Body mass index.