Table 2.
Demographic, clinical, and biochemical characteristics of the analyzed GSD III patients
| Patient no. | Age (years) | Sex | Subtype | Ethnic origin | GDE residual activity (%) | Liver complications | Cardiologic complications | Skeletal muscle complications | Most recent CK value (U/L) |
|---|---|---|---|---|---|---|---|---|---|
| 1 | 8 | M | IIIa | Caribbean | 0 | Hepatomegaly | None | Proximal myopathy | 1083 |
| 2 | 25 | F | IIIa | Caribbean | 0 | Hepatomegaly | None | None | 2232 |
| 3 | 26 | M | IIIa | Caribbean | 0 | Hepatomegaly | Septal hypertrophy | None | 893 |
| 4 | 40 | F | IIIa | Caribbean | 0 | Hepatomegaly | None | None | 3729 |
| 5 | 15 | F | IIIa | Mediterranean | 0 | Hepatomegaly | None | None | 662 |
| 6 | 20 | F | IIIa | Mediterranean | 0 | Hepatomegaly | None | None | 1342 |
| 7 | 32 | F | IIIa | Mediterranean | 0 | Hepatomegaly | Severe symptomatic left ventricular and septal hypertrophy | Exercise intolerance, distal myopathy | 1898 |
| 8 | 3 | M | Too young | Caucasian | 1 | Hepatomegaly | None | None | 133 |
| 9 | 30 | F | IIIa | Caucasian | 0 | None | None | Exercise intolerance, distal myopathy | 2257 |
| 10 | 30 | M | IIIb | Caucasian | 0 | Hepatomegaly | None | None | 68 |
| 11 | 41 | F | IIIa | Caucasian | 0 | Hepatomegaly | None | Exercise intolerance | 392 |
| 12 | 41 | F | IIIb | Caucasian | 0 | None, the patient is post-OLTc, pre-OLTc liver cirrhosis and hepatocellular carcinoma was present |
None | None | 70 |
| 13 | 3 | M | Too young | Caucasian | 0 | Hepatomegaly | None | None | 128 |
| 14 | 1 | M | Too young | Caucasian | 0 | Hepatomegaly | None | None | 466 |
M Male, F Female; OLT Orthotopic Liver Transplantation