Table 1.
Patient 1 | Patient 2 | Patient 3 | Patient 4 | Patient 5 | |
---|---|---|---|---|---|
Age at presentation (years) | 11 | 10 | 12 | 11 | 11.5 |
Sex | Male | Male | Male | Male | Female |
Body mass index (kg/m2) | 26 | 31 | 28 | 26 | 21.1 |
Body mass index centile | >95th | >95th | >95th | >95th | 85th–90th |
Length of follow up (years) | 4 | 1.3 | 5 | 0.5 | 0.5 |
ALT (u/l) | 171 | 183 | 218 | 100 | 86 |
AST (u/l) | 90 | 68 | 73 | 40 | 42 |
Albumin (g/dl) | 3.6 | 4.1 | 4.4 | 4.0 | 4.6 |
INR | 1 | 1.2 | 1 | 1.17 | 1 |
Hepatitis screeninga | Negative | Negative | Negative | Negative | Negative |
Autoimmune screeningb | Negative | Negative | Negative | Negative | Negative |
Ceruloplasmin (μmol/l) | 1.8 | 2 | 2.6 | 2.2 | 2.3 |
Urinary Cu (μmol/24 h) | 0.3 | 0.5 | 0.28 | 0.43 | 0.38 |
Fasting glucose (mmol/l) | 3.61 | 3.45 | 3.8 | 4.83 | 4.77 |
Fasting insulin (μU/ml) | 12.7 | 13.1 | 12.9 | 10.01 | 11.3 |
HOMA-IR | 2.04 | 2.01 | 2.17 | 2.17 | 2.4 |
C peptide levels (ng/ml) | 2.0 | 2.6 | 2.8 | 2.0 | 2.7 |
Fasting triglycerides (mg/dl) | 132 | 146 | 130 | 121 | 106 |
Fasting cholesterol (mg/dl) | 194 | 187 | 103 | 200 | 166 |
aHepatitis screen include: Hepatitis B surface antigen, Hepatitis B core antibody and Hepatitis C antibody
bAutoimmune screening include: Anti-nuclear antibody, Anti-smooth muscle antibody and anti-KLM antibody