Table 3 Comparison of disease characteristics and metabolic parameters by CGL subtype
| Â | CGL1 subtype | CGL2 subtype | CGL4 subtype | P-value |
|---|---|---|---|---|
Age at diagnosis, years | ||||
n(available) | 14 | 18 | 10 | Â |
Mean (± SD) | 9.7 (± 10.6) | 3.3 (± 8.4) | 0.8 (± 0.8) | |
Median (range) | 7 (0.1–37) | 0.8 (0.1–36) | 0.5 (at birth–2) | |
Sex, n/n(available) (%) | ||||
n(available) | 14 | 18 | 10 | Â |
Female | 13 (93%) | 16 (89%) | 7 (70%) | |
Male | 1 (7%) | 2 (11%) | 3 (30%) | |
Physical features, n/n(available) (%) | ||||
Acanthosis nigricans | 9/14 (64) | 10/14 (71) | 0/10 (0) | 0.001 |
Acromegaloid features | 9/14 (64) | 10/14 (71) | 6/10 (60) | 0.91 |
Organ system abnormalities, n/n(available) (%) | ||||
Bones/joints | 4/14 (29) | 2/18 (11) | 2/10 (20) | 0.48 |
Cardiovascular system | 0/14 (0) | 10/18 (56) | 6/10 (60) | 0.001 |
Liver | 11/14 (79) | 13/18 (72) | 5/10 (50) | 0.39 |
Hepatomegaly | 8/14 (57) | 13/18 (72) | 5/10 (50) | 0.51 |
Pancreas | 2/14 (14) | 1/18 (6) | 0/10 (0) | 0.59 |
Renal system | 5/14 (36) | 5/18 (28) | 0/10 (0) | 0.09 |
Spleen | 3/14 (21) | 6/18 (33) | 5/10 (50) | 0.34 |
Splenomegaly | 3/14 (21) | 6/18 (33) | 5/10 (50) | 0.34 |
Medication history, n/n(available) (%) | ||||
Anticoagulants | 0/14 (0) | 1/18 (6) | 0/10 (0) | 1.00 |
Anti-diabetic medication | 6/14 (43) | 2/18 (11) | 0/10 (0) | 0.02 |
Cardiovascular medication | 1/14 (7) | 4/18 (22) | 2/10 (20) | 0.57 |
Lipid-lowering medication/treatment | 6/14 (43) | 3/18 (17) | 1/10 (10) | 0.16 |
Metabolic parameters | ||||
FPG (mmol/L) | Â | Â | Â | Â |
 n(available) | 12 | 9 | 1 | 0.62* |
 Mean (± SD) | 9.7 (± 6.3) | 7.4 (± 3.8) | 4.0 (± 0.0) | |
 Median (range) | 6.1 (4.2–19.3) | 5.4 (4.0–14.0) | 4.0 (4.0–4.0) | |
HbA1c (%) | ||||
 n(available) | 11 | 12 | 4 | 0.41 |
 Mean (SD) | 8.2 (± 3.8) | 6.9 (± 2.6) | 5.3 (± 0.4) | |
 Median (range) | 5.8 (4.6–14.3) | 5.8 (4.5–12.0) | 5.3 (4.8–5.7) | |
  > 5.7%, n/n(available) (%) | 6/11 (55) | 6/12 (50) | 0/4 (0) | 0.19 |
  > 6.5%, n/n(available) (%) | 5/11 (45) | 5/12 (42) | 0/4 (0) | 0.37 |
  > 8.0%, n/n(available) (%) | 5/11 (45) | 3/12 (25) | 0/4 (0) | 0.23 |
Total cholesterol (mmol/L)* | ||||
 n(available) | 12 | 13 | 3 | 0.04 |
 Mean (± SD) | 5.7 (± 2.7) | 4.5 (± 1.2) | 3.2 (± 0.9) | |
 Median (range) | 5.2 (3.8–13.2) | 4.3 (3.0–7.7) | 3.6 (2.1–3.8) | |
Triglycerides (mmol/L) | ||||
 n(available) | 12 | 16 | 4 | 0.39 |
 Mean (± SD) | 8.5 (± 10.6) | 5.2 (± 4.4) | 2.0 (± 0.6) | |
 Median (range) | 2.5 (0.9–31.0) | 3.9 (1.4–18.4) | 1.8 (1.6–2.9) | |
 > 1.69 mmol/L, n/n(available) (%) | 7/12 (58) | 13/16 (81) | 2/4 (50) | 0.28 |
 > 2.26 mmol/L, n/n(available) (%) | 6/12 (50) | 12/16 (75) | 1/4 (25) | 0.14 |
 > 5.65 mmol/L, n/n(available) (%) | 4/12 (33) | 5/16 (31) | 0/4 (0) | 0.65 |
ALT (IU/L) | ||||
 n(available) | 12 | 14 | 8 | 0.09 |
 Mean (± SD) | 48.0 (± 53.0) | 89.0 (± 69.0) | 54.0 (± 19.0) | |
 Median (range) | 34.0 (14.0–198.0) | 57.0 (20.0–232.0) | 59.0 (30.0–78.0) | |
 > 35 IU/L, n/n(available) (%) | 6/12 (50) | 10/14 (71) | 5/8 (62) | 0.61 |
  > 55 IU/L, n/n(available) (%) | 2/12 (17) | 7/14 (50) | 4/8 (50) | 0.14 |
AST (IU/L) | ||||
 n(available) | 10 | 15 | 8 | 0.02 |
 Mean (± SD) | 34.0 (± 20.0) | 65.0 (± 41.0) | 58.0 (± 14.0) | |
 Median (range) | 28.0 (15.0–76.0) | 49.0 (26.0–187.0) | 60.0 (36.0–78.0) | |
 > 35 IU/L, n/n(available) (%) | 4/10 (40) | 12/15 (80) | 8/8 (100) | 0.01 |
 > 48 IU/L, n/n(available) (%) | 2/10 (20) | 8/15 (53) | 6/8 (75) | 0.08 |
GGT (IU/L) | ||||
 n(available) | 5 | 8 | 2 |  |
 Mean (± SD) | 59 (± 66) | 58 (± 26) | 121 (0) | 0.16 |
 Median (range) | 44 (13–175) | 55 (31–96) | 121 (121–121) | |
Creatine kinase | ||||
 n(available) | 5 | 7 | 8 | 0.002 |
 Mean (± SD) | 76 (± 53) | 131 (± 78) | 2291 (± 1716) | |
 Median (range) | 63 (16–160) | 82 (60–249) | 1810 (101–6000) | |