Table 1.
Patients with GSD IV who underwent liver transplantation.
| References | Case | Sex | Age at LT (yr.) | Indications for LT | Complications | Follow-up/survival (yr.) | Liver function post-LT | Cardio-/myo-/neuropathy |
|---|---|---|---|---|---|---|---|---|
| Aksu et al. (3) | 1 | F | 23 | HF LC |
None | 0.5 | NA | cardiomyopathy |
| Choi et al. (24) | 2 | M | 0.8 | LF | None | 1 | normal | None |
| Willot et al. (25) | 3 | M | 0.9 | LF | Deatha | 6.3 | abnormal | cardiomyopathy |
| 4 | M | 0.9 | LF | Deathb | 1 | normal | Cardiomyopathy myopathy |
|
| Sokal et al. (26) | 5 | M | 1.2 | LF | Deathb | 0.9 | normal | Cardiomyopathy |
| Ban et al. (27) | 6 | F | 2.3 | LF | None | 0.5 | normal | NA |
| Selby et al. (23, 28) | 7 | M | 2.6 | LF | NA | 13.2 | normal | None |
| 8 | M | 0.9 | LF | Deathc | [7 days] | |||
| 9 | M | 3.0 | LF | Deathd | 0.1 | |||
| 10 | M | 3.8 | LT | NA | 11.9 | normal | None | |
| 11 | M | 1.8 | LF | Deathe | 5.4 | normal | ||
| 12 | M | 1.7 | LF | NA | 8.8 | normal | None | |
| 13 | M | 2.8 | LF | NA | 8.7 | normal | None | |
| Dhawan et al. (29) | 14 | M | 12 | LF | Non-function of graft | None | ||
| Re-transplant (2 days after 1st LT)f | NA | 3.9 | ||||||
| Alshak et al. (30)Rosenthal et al. (31) | 15 | M | 0.9 | LF HA |
Rejection Deathb |
2.5 | normal | cardiomyopathy |
| Bruno et al. (7) | 16 | M | 3 | LF | mild hypotonia Arthrogryposis | 3 | normal | myopathy |
| Magoulas et al. (2) | 17 | F | 0.8 | LF | Sepsis Deathg |
0.3 | normal | cardiomyopathy |
| Morioka et al. (32) | 18 | M | 3.7 | LF | Rejection | |||
| Re-transplant (6.2 months after 1st LT) | Sepsis Deathh |
0.6 | ||||||
| 19 | M | NA | NA | NA | NA | NA | NA | |
| 20 | F | NA | NA | NA | NA | NA | NA | |
| Matern et al. (33) | 21 | M | 1.3 | LF | None | 13.5 | NA | None |
| 22 | M | 3.7 | LF | None | 5.5 | NA | None | |
| 23 | M | 5.5 | LF | PVT | 9.9 | NA | None | |
| Troisi et al. (34) | 24 | F | 2 | NA | None | 0.8 | normal | None |
LF, liver failure; HA, hepatic adenomas; HF, heart failure; LC, liver cirrhosis; PVT, portal vein thrombosis; LT, liver transplantation; HAT, hepatic artery thrombosis; M, male; F, female; NA, not available.
a
Death due to terminal heart failure and Gram-positive bacterial sepsis.
b
Death due to heart failure.
c
Death 7 days post-LT due to sepsis following bowel perforation.
d
Death at 1 month post-LT following hepatic artery thrombosis.
e
Death due to meningococcal sepsis.
f
Due to non-functioning 1st donor liver.
g
Death 0.3 years post LT due to respiratory and circulatory failure secondary to sepsis.
h
Death 1 month after the second LT due to sepsis.