Table 4 Disease characteristics and metabolic parameters in patients diagnosed with CGL at or before 1 year of age
|  | Patients diagnosed ≤ 1 year of age |
|---|---|
Sex, n/n(available) (%) | |
Female | 19/22 (86) |
Male | 3/22 (14) |
Physical features, n/n(available) (%) | |
Acanthosis nigricans | 6/18 (33) |
Acromegaloid features | 8/18 (44) |
Hirsutism* | 4/19 (21) |
Organ system abnormalities, n/n(available) (%) | |
Bones/joints | 1/22 (5) |
Cardiovascular system | 9/22 (41) |
Liver | 15/22 (68) |
 Hepatomegaly | 15/22 (68) |
Pancreas | 1/22 (5) |
Renal system | 4/22 (18) |
Spleen | 8/22 (36) |
 Splenomegaly | 8/22 (36) |
Medication history, n/n(available) (%) | |
Anticoagulants | 0/22 (0) |
Antidiabetic medication | 0/22 (0) |
Cardiovascular medication | 2/22 (9) |
Lipid-lowering medication/treatment | 2/22 (9) |
Metabolic parameters | |
FPG (mmol/L) | |
 n(available) | 11 |
 Mean (± SD) | 7.0 (± 3.5) |
 Median (range) | 5.4 (4.0–14.0) |
HbA1c (%) | |
 n(available) | 13 |
 Mean (± SD) | 6.1 (± 1.9) |
 Median (range) | 5.6 (4.5–11.5) |
  > 5.7%, n/n(available) (%) | 5/13 (38) |
  > 6.5%, n/n(available) (%) | 3/13 (23) |
  > 8.0%, n/n(available) (%) | 2/13 (15) |
Total cholesterol (mmol/L) | |
 n(available) | 14 |
 Mean (± SD) | 4.6 (± 1.4) |
 Median (range) | (2.1–7.7) |
  > 5.18 mmol/L, n/n(available) (%) | 4/14 (29%) |
Triglycerides (mmol/L) | |
 n(available) | 17 |
 Mean (± SD) | 4.1 (± 4.5) |
 Median (range) | 2.4 (0.9–18.4) |
 > 1.69 mmol/L, n/n(available) (%) | 10/17 (59) |
 > 2.26 mmol/L, n/n(available) (%) | 9/17 (53) |
 > 5.65 mmol/L, n/n(available) (%) | 4/17 (24) |
ALT (IU/L) | |
 n(available) | 16 |
 Mean (± SD) | 70 (± 51) |
 Median (range) | 49 (28–173) |
 > 35 IU/L, n/n(available) (%) | 12/16 (75) |
 > 55 IU/L, n/n(available) (%) | 7/16 (44) |
AST (IU/L) | |
 n(available) | 18 |
 Mean (± SD) | 63 (± 36) |
 Median (range) | 54 (29–187) |
 > 35 IU/L, n/n(available) (%) | 16/18 (89) |
 > 48 IU/L, n/n(available) (%) | 10/18 (56) |
GGT (IU/L) | |
 n(available) | 10 |
 Mean (± SD) | 77 (± 52) |
 Median (range) | 66 (18–175) |
Creatine kinase (IU/L) | |
 n(available) | 11 |
 Mean (± SD) | 1256 (± 1793) |
 Median (range) | 160 (60–6000) |