Table 1.
Indication and follow-up of GSDIa and GSDIb liver transplantation
| |
GSDIa (n = 58) |
GSDIb (n = 22) |
|---|---|---|
| Year of transplantation | 1982 – 2012 | 1991 – 2012 |
|
Indications |
|
|
| Hepatic adenomas or liver abnormalities (mostly focal nodular hyperplasia) |
29 [6-22] |
|
| Poor metabolic control |
27 [6,8-10,14-16,19,21,23-27] |
21 [13,28-34] |
| Growth retardation |
13 [10,14-16,23,24,27] |
3 [32,33,35] |
| Recurrent infections |
|
10 [28,33,34] |
| Renal failure |
5 [36] |
|
| -Of which also kidney transplant |
3 [17,26,37] |
|
| Bleeding complications (anemia) |
1 [27] |
|
| Pancreatitis |
1 [24] |
|
| Anal and oral ulcera |
|
1 [30] |
|
Immunosuppressive regime |
|
|
| Cyclosporine |
12 [6,7,9,10,12,14,15,17],[21,26] |
1 [28] |
| Cyclosporine + azathioprine |
17 [16,23,25,38,39] |
- |
| Tacrolimus |
9 [16,18,21,36] |
16 [30,31,33-35] |
| Not reported |
22 |
6 |
|
Outcome and complications GSDIa |
Short-term (≤1 year) |
Long-term (>1 year) |
| Normalization liver function |
58 (all) |
- |
| Catch-up growth |
- |
13 [10,13,14,23,25,39] |
| Sexual maturation |
- |
2 [25] |
| Re-OLT |
2 [16,23] |
1 [20] |
| Death |
1 (pancreatitis and sepsis) [25] |
3 (rejection related liver failure, suicide, metastatic hepatocellular carcinoma) [19], PC |
| Renal failure |
6 [21] |
4 [20] |
| -Of which already pre-transplant |
1 [39] |
|
| -Requiring dialysis |
1 [12] (temporary) |
2 [27], PC (permanent) |
| -Gouty arthritis (due to renal failure?) |
- |
1 [14] |
| Metastatic hepatocellular carcinoma |
1PC |
|
| Transplantation associated |
|
|
| -Hepatic artery thrombosis |
2 [16,23] |
- |
| -Portal vein thrombosis |
- |
2 [6,16] |
| -Hepatic vein obstruction |
2 [25] |
- |
| -Prolonged drainage |
2 [25,39] |
- |
| -Rejection |
5 [14,20,21,38,39] |
3 [6,9] |
| -Never functioning liver after transplantation |
2 [23,24] |
|
| Therapy (immune suppression) associated |
|
|
| -Hypertension |
4 [25,39] |
- |
| -Insulin-dependent diabetes (reversible) |
3 [8,9,21] |
- |
| -Infections (CMV, hepatitis) |
7 [21,25,39] |
1 [14] |
|
Outcome and complications GSDIb |
Short-term (≤1 year) |
Long-term (>1 year) |
| Normalization liver function |
23 (all) |
|
| Catch-up growth |
|
2 [32] |
| Death |
1 (systemic candidiasis) [40] |
|
| Neutropenia |
- |
|
| -Improved |
- |
14 [31,34,35] |
| -Persisted |
|
7 [13,29,30,32,33] |
| Thrombocytopenia |
|
1 [28] |
| Transplantation associated |
|
|
| -Infection |
6 [34] |
- |
| -Anemia |
1 [33] |
- |
| -Rejection |
- |
1 [13] |
| Therapy (immune suppression) associated |
|
|
| -Infection |
1 [30] |
1 [26] |
| -Tacrolimus encephalopathy | 1 [35] |