Table 2.
Patients with regression of HCA
| Age | Sex | GSD Type | DNA analysis | Age at HCA discovery (years) | Age at Time of HCA regression (years) | # of HCAs | Size of Largest HCA (cm) | Size of HCA after regression | Duration of follow-up since beginning of regression (months) | Duration it took for HCAs to completely resolve (months) | Duration of follow-up since complete resolution (months) | Total duration of follow-up (months) | Average triglyceride level prior to HCA regression in mg/dL | Average triglyceride level since beginning of HCA regression in mg/dL | |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
| 1 | 18 | M | GSDIa | R83C/130X | 5 | 9 | 14 | 1.5 | Complete resolution | 86 | 65 | 23 | 131 | 345 ± 68 | 211 ± 59 |
| 2 | 31 | F | GSDIa | R83C/R295C | 27 | 28 | 4 | 1.9 | Complete resolution | 29 | 7 | 24 | 152 | 957 ± 276 | 444 ± 140 |
| 3 | 34 | F | GSDIa | Q347X | 21 | 30 | 2 | 1.9 | Complete resolution | 60 | 19 | 41 | 81 | 591 ± 131 | 207 ± 22 |
| 4 | 32 | F | GSDIa | R83C | 12 | 23 | 5 | 2.0 | Complete resolution | 123 | 23 | 100 | 147 | 961 ± 403 | 294 ± 84 |
| 5 | 23 | M | GSDIa | W77R/G118S | 15 | 17 | 4 | 3.1 | Complete resolution | 57 | 36 | 21 | 57 | 814 ± 243 | 718 ± 169 |
| 6 | 35 | M | GSDIa | R83C/R295C | 22 | 30 | 2 | 3.3 | Complete resolution | 64 | 34 | 40 | 73 | 835 ± 184 | 312 ± 66 |
| 7 | 31 | F | GSDIa | Q347X | 19 | 25 | >10 | 2.6 | 1.5 cm (most small lesions resolved) | 41 | NA | NA | 60 | 314 ± 84 | 187 ± 14 |
| 8 | 34 | F | GSDIa | R83C | 29 | 30 | 5 | 13.2 | 9.1 cm | 49 | NA | NA | 49 | 1,205 ± 852 | 343 ± 110 |
| Average values | 63.6 | 30.7 | 41.5 | 93.8 | 753 ± 293 | 340 ± 164 | |||||||||