Table 5 Disease characteristics and metabolic parameters for patients diagnosed < 12 years of age versus patients diagnosed ≥ 12 years of age
Characteristic | Patients diagnosed < 12 years of age | Patients diagnosed ≥ 12 years of age | P-value |
|---|---|---|---|
Age at diagnosis, years | |||
n(available) | 35 | 8 | Â |
Mean (± SD) | 1.5 (± 2.3) | 20.9 (± 10.0) | |
Median (range) | 1 (at birth–11) | 17 (12–37) | |
Sex, n/n(available) (%) | 35 | 8 | Â |
Female | 30 (86) | 7 (87) | |
Male | 5 (14) | 1 (13) | |
Physical features, n/n(available) (%) | |||
Acanthosis nigricans | 14/31 (45) | 6/8 (75) | 0.24 |
Acromegaloid features | 20/31 (65) | 5/8 (62) | 1.00 |
Organ system abnormalities, n/n(available) (%) | |||
At least one abnormality | 27/35 (77) | 8/8 (100) | 0.32 |
Bones and/or joints | 3/35 (9) | 5/8 (62) | 0.003 |
Cardiovascular system | 15/35 (43) | 1/8 (13) | 0.22 |
Liver | 23/35 (66) | 7/8 (87) | 0.40 |
Hepatomegaly | 22/35 (63) | 5/8 (62) | 1.00 |
Pancreas | 1/34 (3) | 3/8 (38) | 0.02 |
Renal system | 6/35 (17) | 4/8 (50) | 0.07 |
Spleen | 14/35 (40) | 0/8 (0) | 0.04 |
Splenomegaly | 14/35 (40) | 0/8 (0) | 0.04 |
Medication history, n/n(available) (%) | |||
Anticoagulants | 0/35 (0) | 1/8 (13) | 0.19 |
Antidiabetic medication | 2/35 (6) | 7/8 (87) |  < 0.001 |
Cardiovascular medication | 5/35 (14) | 2/8 (25) | 0.60 |
Lipid-lowering medication/treatment | 2/35 (11) | 6/8 (75) | 0.001 |
Metabolic parameters | |||
FPG (mmol/L) | |||
 n(available) | 17 | 6 | 0.01 |
 Mean (± SD) | 6.7 (± 3.3) | 15.2 (± 5.8) | |
 Median (range) | 5.0 (4.0–14.0) | 17.9 (4.6–19.3) | |
HbA1c (%) | |||
 n(available) | 21 | 7 |  < 0.001 |
 Mean (± SD) | 6.0 (± 1.9) | 11.2 (± 3.1) | |
 Median (range) | 5.4 (4.5–11.5) | 12.0 (5.7–14.3) | |
  > 5.7%, n/n(available) | 7/21 (33) | 6/7 (86) | 0.03 |
  > 6.5%, n/n(available) (%) | 5/21 (24) | 6/7 (86) | 0.007 |
  > 8.0%, n/n(available) (%) | 3/21 (14) | 6/7 (86) | 0.001 |
Total cholesterol (mmol/L) | |||
 n(available) | 23 | 6 | 0.02 |
 Mean (± SD) | 4.4 (± 1.1) | 7.1 (± 3.2) | |
 Median (range) | 4.1 (2.1– 7.7) | 6.6 (3.9–13.2) | |
Triglycerides (mmol/L) | |||
 n(available) | 26 | 7 | 0.003 |
 Mean (± SD) | 3.9 (± 3.9) | 14.5 (± 10.7) | |
 Median (range) | 2.4 (0.9–18.4) | 12.4 (2.1–31.0) | |
 > 1.69 mmol/L, n/n(available) (%) | 16/26 (62) | 7/7 (100) | 0.07 |
 > 2.26 mmol/L, n/n(available) (%) | 14/26 (54) | 6/7 (86) | 0.20 |
 > 5.65 mmol/L, n/n(available) (%) | 5/26 (19) | 5/7 (71) | 0.02 |
ALT (IU/L) | |||
 n(available) | 28 | 7 | 0.29 |
 Mean (± SD) | 68.0 (± 55.0) | 56.0 (± 64.0) | |
 Median (range) | 48.0 (14.0–232.0) | 45.0 (14.0–198.0) | |
 > 35 IU/L, n/n(available) (%) | 18/28 (64) | 4/7 (57) | 1.00 |
 > 55 IU/L, n/n(available) (%) | 12/28 (43) | 1/7 (14) | 0.22 |
AST (IU/L) | |||
 n(available) | 28 | 6 | 0.01 |
 Mean (± SD) | 58.0 (± 33.0) | 30.0 (± 19.0) | |
 Median (range) | 51.0 (19.0–187.0) | 22.0 (15.0–63.0) | |
 > 35 IU/L, n/n(available) (%) | 23/28 (82) | 2/6 (33) | 0.03 |
 > 48 IU/L, n/n(available) (%) | 15/28 (54) | 1/6 (17) | 0.18 |
GGT (IU/L) | |||
 n(available) | 14 | 2 | 0.87 |
 Mean (± SD) | 69.0 (± 47.0) | 47.0 (± 4.0) | |
 Median (range) | 59.0 (13.0–175.0) | 47.0 (44.0–50.0) | |
 Creatine kinase (IU/L) | |||
 n(available) | 17 | 3 | 0.02 |
 Mean (± SD) | 1145 (± 1591) | 51 (± 31) | |
 Median (range) | 229 (60–6000) | 61 (16–76) | |